Editor and Author: Dr.

Marc Anders
MD (Germany), FRCA (UK), FCICM (Australia), Facharzt Anesthsiology and Intensiv Care (Germany)

WITH MANY THANKS TO ALL WHO CONTRIBUTED TO THIS GUIDE LINE !

Dr. Andreas Schibler, Dr. Bret McVinish, Dr. Chris James, Dr. Clare Nourse, Emma Haisz,
Dr. Jamin Mulvey, Dr. Jason Yates, Dr. Jim Morwood, Dr. Kevin Plumpton, Dr. Mark
Hayden, Dr. Nalini Selveindran, Rachel Teis, Quyen Tu, Dr. Rajshree Trivedi
for K
And special thanks to
Bennett Sheridan - for all the excellent diagrams
Rapsou Rapciewicz and Stephanie Chesneau for the wonderful drawings
Christian Stocker - for the hard discussions on the TGA chapter and the
excellent Single Ventricle chapter
Peter Damen for the iPhone app and webpage design
Jubal John for the webpage hosting

1st ed May 2011

2nd ed October 2012
3rd ed March 2013
4th ed March 2014
5th ed December 2014
This is only a guideline and does not substitute local clinical experience, clinical
evaluation and / or judgement of each individual patient!
Any comments / suggestions much appreciated,
email to marcanders@web.de.

TABLE OF CONTENTS
THE BASICS
Cardiac Admission
Patient Discharge from PICU
Initial postoperative care and problems
Definitions
Formulae
Cardiac Cycle
Frank Starling Mechanisms
Infection
Empiric Antibiotics for general infection
Central Venous Catheters
ANESTHESIA
Analgesia and Sedation
Muscle Relaxation
Tolerance and Withdrawal
Weaning Opiods and Sedation
Intubation in PICU
Inotropes and Vasopressors
Inotropes and Vasodilators
PICU
Fluids
Maintenance Fluids
Nutrition
Feeding Guideline
Blood products

TABLE OF CONTENTS <CONT>

Anticoagulation & Thrombolysis
Chest Drains
Chylothorax
Genetic Syndromes and Cardiac Defects
Low Cardiac Output Syndrome
Open Chest
Arrhythmias
Pacing
Prolonged QT Syndrome
Pulmonary Hypertension
Nitric Oxide
Renal Failure
Renal Failure Haemofiltration & Dialysis
T3 in cardiac surgery

CARDIAC DEFECTS
Anomalous Left Coronary Artery
Atrial Septal Defect
Atrioventricular Septal Defect
Blalock-TaussigShunt
Coarctation of the Aorta
Double Outlet Right Ventricle
Ebsteins Anomaly
Fontan Circulation
Glenn Shunt
Heterotaxy

TABLE OF CONTENTS <CONT>

Hypoplastic Left Heart Syndrome
Interrupted Aortic Arch
Left Ventricular Outflow obstruction
Patent Ductus Arteriosus
Pulmonary Atresia with IVS
Single Ventricle Physiology
TAPVD
Transposition of great arteries
Tetralogy of Fallot
Tricuspid Atresia
Truncus arteriosus
Ventricle Septal Defect
HEART FAILURE & ASSIST
Cardiomyopathy
Myocarditis
ECMO
ECMO Antibiotics
Heart Failure and VAD
Berlin Heart VAD EXCOR
Heart Transplantation
ABO incompatible HTX
Resuscitation Drug Chart
Resuscitation Quick Chart
Neonatal Resuscitaion Guideline USA
Neonatal Resuscitaion Guideline UK
APLS Guideline: Cardiac Arrest Australia

TABLE OF CONTENTS <CONT>

PALS Guideline Cardiac Arrest AHA

THE BASICS: CARDIAC ADMISSION

General principles: Anticipate the type of patient and potential problems ! Understand
lesion/post correction basic physiology and common postoperative issues ! - Ask if needed !
Admission: Expected admissions should be discussed in the morning ward round. Have a
quick read through the PICU Cardiac Guideline or discuss with fellow or consultant about the
lesion, surgery, expected problems and management. Theatre staff should call with an
expected time of arrival at least 20min in advance. The theatre technician should also write a
cardiac patient transfer report with details of procedure, lines, infusions etc. All patients
returning from cardiac theatre need to get a cardiac surgical admission, even if theyve been
in PICU pre-op. If they return to theatre for a second or more procedures they will get
cardiac surgical admissions each time.
Have cardiac handover sheet ready, write up blood form, CXR form.
Basic prescription for cardiac patients prior to arrival on the unit:
Venous Heparin flushes including flushes for intracardiac lines: Heparin infusion @
10U/kg/hr for all babies less than 5kg with CVL
Arterial Heparin flush infusion & bolus
Sedation infusion & boluses (usually morphine and midazolam separately)
Inotropes as per transfer note
Fluid boluses 5 10ml/kg NaCl 0.9% or Albumin 5%
For ECMO patients prescribe a PRN order for blood, platelets, FFP or Cryo. Also chart the
ECLS Heparin flushes, dialysate order. Blood (packed cells and CMV ve and irradiated for
all infants less than 4month or if patient is +ve for 22 q11 or any other suspected
immunodeficiency)
Antibiotics: Cephazolin 25mg/kg q8h for 3 doses plus Mupirocin 2% local application
around nares q12h for 3 days plus Nystatin 100.000U PO q8h as long as on antibiotics in
neonates. In open chest continue antiobiotics until chest closure.
Paracetamol: < 10kg 7.5mg/kg IV q6h, > 10kg 15mg/kg IV q6h, change to PO/NG as soon
as able, up to 15mg/kg q4h.
Fluid allowance :
all non-bypass cases get 50% maintenance if > 10kg and < 10kg 2ml/kg/hr
for bypass cases > 10kg 30% maintenance and < 10kg 1ml/kg/hr; these fluids are
upgraded with a general rule of increase of 1mL/kg/hr each day but check first i.e.
Day 1 - 30% maintenance or 1ml/kg/hr
Day 2 - 50% maintenance or 2ml/kg/hr
Day 3 - 75% maintenance or 3ml/kg/hr
Day 4 - full maintenance
Cardiac pre op (if patients are going to OT from PICU):
Ensure 4 units packed cells, 2 units platelet, 2 units FFP, 2 units Cryoprecipitate are ready
for all pre op cardiac patients if in PICU prior need to liaise with Blood Bank
Crossmatch is on a separate form and MUST ensure that the sample goes in a EDTA top
tube with an HANDWRITTEN label (blood bank will reject samples with signed labels)
With neonates make sure pre-op Chromosomes & GN FISH for 22q11 has been sent
especially in outflow obstructive lesions, prior any blood transfusion
These patients will get a routine medical admission and then cardiac surgical if they have
surgery or a procedure in cathlab.
Prescribe Mupirocin 2% local application around nares q12h, ideally for 2 days prior to
cardiac procedure.
Examine the patient just prior to scheduled departure time and hand over to anesthetist.
Transfer of cardiac patient to PICU:
Phase 1: Pre handover

 approximately 30 min before arrival on PICU, Cardiac Patient Transfer Report completed
by anesthetic technician. PICU informed of ETA by phone.
Phase 2: Equipment and Technology handover
On arrival on PICU ventilation, monitoring and support pumps are transferred across prior
to handover. Discussion should be kept to a minimum at this point.
The anesthetist will supervise and direct this transfer with assistance from the anesthetic
technician, OR nurse and PICU team.
Safety Check: once transfer has occurred the delivery team checks that the patient is
stable and ventilation is occurring and checks that the PICU team is ready for handover.
At this point the OR nurse may leave if they wish. All activity with the exception of
observing the patient and monitoring ceases.
Phase 3: Information handover
The anesthetist and then the surgeon hands over the patient (preferably without
interruption unless a member of the team identifies an urgent issue of stability in which case
handover should pause while it is dealt with). The information will be presented in a standard
order consistent with the Cardiac Surgical admission sheet.
Safety Check: The PICU team listens and takes notes, they use the Cardiac Surgical
Admission sheet to ensure that they have all necessary information and ask any relevant
questions.
Phase 4: Discussion and Plan
The team discusses the case, anticipate problems and agree on a documented plan.
The ICU team now assumes responsibility for the patient and commence routine
observations and management.
Notes:
The chief operating surgeon, anesthetist and admitting intensivist should all be present at
the handover.
In the event of an arrest during handover - the anesthetist will run things until the PICU
consultant feels they have enough information to do so, or to take over. The person in
charge should say something along the lines of "I will run this" so it's clear for all. The
handover sheet "Cardiac Surgical Admission" - may be filled out by the anesthetist in theatre
if there is adequate time.
Important details to remember:
Operation performed and the duration of CPB and aortic cross clamping (latter being time
of cardioplegia)?
Check if there are any concerns regarding anatomy or repair (e.g. coronaries, size of
shunt, etc.) or pulmonary hypertension?
Note pressures (RAP/CVP/PA, LAP and MAP), cerebral saturations, cardiac rhythm and
oxygen saturations in theatre.
Note intra-operative problems and any complications encountered during induction,
arrhythmia, pacemaker use, coming off CPB problems, bleeding and blood products given,
and medications given.
Ask what blood products are available (unused from theatre).
Discuss with consultant about plan/desired parameters and expected problems and their
management.
Initial Assessment:
Quick assessment focusing on cardiovascular stability and adequate ventilation (vital signs
and chest movement) as patient arrives from theatre.
Review current support drugs inotropes and/or vasodilators
Review with nurses that the patient is safely connected to ICU support and monitoring
equipment. Check ventilator settings
Review anesthetic, CPB charts and operation or any intra-operative TOE notes. Look for
difficult airway, anesthetic medications given, heart function, and residual lesions
Detailed physical examination

 Request: ABG / VBG / FBC / U&E / Calcium, Mg, PO4 / Coagulation profile, TEG / CXR
Document clinical findings and plan in the cardiac surgical admission
Review as soon as possible CXR (check position of ETT, temperature probe, lines, drains,
any pneumothorax/effusion), and first set of blood results ventilation settings may need
adjustment according to ABG
Check baseline ECG
Ongoing Management:
Adequate Opiate Analgesia. Titrate other sedatives as required
IV infusion of Muscle relaxant if cardiovascular unstable
Support the circulation - see lesion specific modules
Treat @ Arrhythmia
Bleeding > 3ml/kg/hr investigate (@ Chest Drains). If > 10ml/kg/hour or a sudden stop in
drainage needs urgent surgical & cardiology review. A quick bedside scan with the ultrasound machine in the unit maybe useful.
Fluid resuscitate, send FBC and coagulation profile. Consider TEG & replace factors as
indicated (most likely to need cryoprecipitate and platelets)
Watch for @ Tamponade ! If suspected quick bedside scan with the ultra-sound machine
in the unit maybe useful
Packed cells 4 ml/kg raises Hb by 1g/dl
Follow unit antibiotic policy
Investigations: FBC, U&E, LFT, Ca /Mg / PO4. Coagulation for post-op on first few days or
when lines/drains coming out. CRP if previous high value or if suspicion of sepsis
If intracardiac lines going to be removed the next day then needs coagulation profile (plus
crossmatch with blood available in theatre fridge important to check that the blood is
physically in the theatre fridge, not back in Blood Bank, the bedside nurse will usually do
this, just confirm)
Cardiac handover:
Keep it short/simple & sweet. Please SPEAK UP!
Name, age and day post-op
Lesion & procedure done
Cardiovascular HR, rhythm, BP, +/- pacing required, trends
Blood products if any given
Ventilations: Adequate gas exchange or mention issues if any
Gases include lactates and mixed venous, trends
Fluids balance and urine output / PD for last 24 hrs
Drain losses type eg. blood, serous, serosanguineous.
Trend of last 6 - 8hrs
Mention bloods if something grossly abnormal
If CXR done & seen mention briefly.
ECMO - flow, sweep, inlet and outlet pressures, ACTs, blood products given, bleeding,
circuit (i.e. clots), arterial line trace (pulsatile or non pulsatile)
The seven vital causes to remember of postoperative Tachycardia:
CNS: fever, pain, inappropriate sedation and analgesia, seizures
CVS: low cardiac output
CVS: Tachyarrhythmia
Respiratory: hypoxia, hypercarbia
Pulmonary Hypertension
Drugs: catecholamines
Residual defects

THE BASICS: DISCHARGE FROM PICU

Patients in the PICU will be evaluated and considered for discharge based on the reversal of
the disease process or resolution of the physiological instability that prompted admission to
the unit, and it is determined that the need for complex intervention exceeding ward
capabilities is no longer needed.
Transfer and / or discharge will be based on the following criteria:
Neurologically stable
stable hemodynamic parameters
intravenous inotropic support, vasodilators, and anti-arrhythmic drugs are no longer
required (minimum for 4hours post ceasing)
haemodynamic monitoring catheters removed or capped and well secured. Once the line
has been capped it is NOT to be flushed or re-used.
cardiac dysrhythmias are controlled
Pacing wires are generally removed after 24hours if there is no evidence of arrhythmia
stable respiratory status (patient extubated with stable arterial blood gases) and airway
patency (minimum 4hours post extubation)
minimal oxygen requirements that do not exceed patient care unit guidelines
Approval for and timing of discharges must be by the Consultant or Fellow !
PICU registrar discharge checklist:
enter a brief discharge note in the patients record before discharge, summarizing final
diagnosis, main PICU therapies, current problems, current medications, and current
management plan
complete the computer discharge summary
all notes and discharge summary need to be printed out and signed by registrar / fellow
complete ward drug chart, double check with pharmacist during duty hours for completion
and correctness
Notify the cardiologist, surgeon and ward registrar, if not already advised
Generic PICU to Ward shared Handover to be filled in and signed: Handover given to ward
physician, acute pain service informed or ward pain medication written up, plan of care and
outstanding issues, blood results reviewed and / or actioned, timing and frequency on
medical review, names of registrar / fellow, completed the discharge summary, name of the
ward physician, name of the pain team
immediately prior to discharge examine the patient, collate investigations including that
days x-ray and blood tests, review IV orders and drugs and check the medications and fluids
for the ward are written
The patient's relatives must be aware of the discharge plan and notified when their child is
moved

THE BASICS: INITIAL POSTOPERATIVE CARE AND PROBLEMS

Abdominal distension
Causes: air in stomach or bowel (usually from mask ventilation at induction of anesthesia)
or tension pneumothorax; fluid in bowel or in peritoneal cavity (usually capillary leak, high RA
pressure or PD fluid; rarely fluid overload or peritoneal hematoma). Exclude NEC, especially
in neonates with parallel circulations or long cross-clamp time.
Investigation and management: examine chest and abdomen: percussion; examine fluid
drainage from PD catheter and chest drains (amount and redness - measure Hb); aspirate
NG tube; repeat CXR (compare with previous, air in stomach or bowel, pneumothorax);
monitor abdominal girth; check coagulation and platelets (correct if abnormal and bleeding);
abdominal ultrasound if suspect retroperitoneal hemorrhage (femoral line). If capillary leak is
the problem, lasts up to 2days (longer in neonates, after long operations, in sepsis and when
the cardiac output is low); during this time, fluid restriction doesn't prevent edema or ascites,
but only leads to hypovolaemia. Adjust ventilation if necessary to compensate for reduced
abdominal compliance.
Atelectasis
Causes: tracheal intubation, thick secretions, inadequate humidification (check humidifier
tank and tubing temperatures), inadequate tracheal suction, airway compression or collapse
(eg malacia), paralysed hemidiaphragm while spontaneous ventilation
Signs: decreasing SaO2, rising PaCO2, may be reduced ipsilateral chest movement,
usually involves RUL and LLL.
Management: hand-ventilation, instill normal saline (0.25 - 0.5ml) into trachea before
suction, physiotherapy, culture tracheal aspirate, X-ray screen diaphragm if clinical suspicion
of paralysis, bronchogram if other signs suggest malacia (hyperinflation, wheezing,
prolonged expiration).
Atrial pressure increasing
Examine patient; check BP, heart rate, RAP, LAP. AV valve regurgitation. Look for V
wave in atrial trace (AV regurgitation or malposition of atrial catheter through AV valve).
Over-filling. In aortic or pulmonary stenosis, non-compliant ventricles cause atrial pressures
to rise with small increases in volume. Treat by careful diuretics and / or GTN (filling
pressures !). Tamponade. Associated with tachycardia and falling BP and cardiac output.
Notify surgeons and immediate Echo, but do not delay chest opening if situation serious. In
infants, myocardial edema without pericardial fluid can cause tamponade that may be
immediately relieved by opening the chest. Deteriorating myocardial performance.
Arrhythmia. Pneumothorax.
Management: re-calibrate transducers. Examine chest and abdomen; check blood gas,
electrolytes and lactate; hand-ventilate and suction ETT; check ECG rhythm; obtain a CXR;
notify surgeons if tamponade suspected; trial of vasodilators, diuretics and inotropes.
Cardiac Tamponade
Signs: rising HR; increasing lactate and metabolic acidosis; falling BP with low pulse
pressure and narrowing of systolic and diastolic BP; both atrial pressures rise (especially
RA); chest drainage may increase (if due to increased bleeding) or (usually) decrease
(drainage blocked); heart sounds may be muffled; QRS complexes may be smaller. Milk
drains (are they on adequate suction, are the drain reservoirs full). If there is the slightest
suspicion of tamponade, notify the surgeon ! (do not delay for investigations).
Management: obtain CXR (may show increase in heart size; more globular heart shape;
increased distance from pacing wires or LA/PA lines to heart border); obtain Echo; check

blood gases and clotting (PT, PTT, fibrinogen, platelets); milk the chest drains; stop
vasodilators; give blood or saline 10 ml/kg; maintain the coronary perfusion pressure using
inotropes; consider clotting factors or platelets; if ACT >100 sec, give protamine 0.5 mg/kg
and re-check ACT; consider aspirating LA or PA lines to check position of their tips (? in
pericardial cavity); may need urgent chest opening.to prevent further detoriation.
Convulsions
In a paralysed child, a seizure may consist only of increases in HR, BP, PA or atrial
pressures or spontaneous variations in pupil size. Review history: pre-op; intra-op and postop events; check blood glucose, gases and electrolytes (including Ca++ and Mg++); cease
muscle relaxants; check autonomic response to IV midazolam bolus; neurological
examination when muscle power returns; consider consulting neurologist (is this a fit?
prognosis? follow-up ?); monitor EEG during autonomic changes to confirm seizure present;
consider CT scan; load with phenobarbitone up to 30mg/kg IV in 5 - 10mg/kg increments
(beware hypotension); continue phenobarbitone if fits continue; avoid IV phenytoin
(myocardial depressant) after cardiac surgery. Consider Keppra 10mg/kg IV.
Fever
All children become febrile after open heart surgery, and most become febrile after any
thoracotomy. The fever appears as soon as the child re-warms after the operation, and lasts
24 48 hours. During this time, the child can still become septic, but the diagnosis of sepsis
depends on other signs. A secondary increase in temperature (after the normal post-op fever
has settled) means sepsis until proven otherwise (CRP, PCT, WCC, ITR). High postoperative fever may be associated with marked tachycardia, and an increase in VO2 (11%
increase in VO2 per 1oC increase in temperature). Regular Paracetamol (single dose
30mg/kg post-op) to keep core temperature < 37.5oC. If the temperature is > 39oC despite
paracetamol and the child is still paralysed, consider using cool peritoneal dialysis (1.5%
solution at room temperature in 30minutes cycles, each of 10ml/kg) or surface cooling to
normothermia, using a cooling blanket.
Haemorrhage
Causes: thrombocytopenia; poor platelet function; dilution or consumption of clotting
factors; residual heparin (usually in the first 4hours post-op); surgical problem.
Signs: losses from chest drains remain bright red and increase in amount or fail to
decrease normally; tamponade; hypoventilation and / or poor unilateral chest movement;
increasing abdominal distension.
Investigation and Management: notify surgeon early; measure Hb of chest drain fluid;
repeat CXR if suspect tamponade or pneumothorax; check ACT, TEG, coagulation and
platelets; give protamine 0.5mg/kg IV and repeat ACT; give platelets 10ml/kg; give FFP
10ml/kg if ACT, PT or APTT remain prolonged despite protamine; give cryoprecipitate if
fibrinogen low; consider giving aprotinin if major bleeding persists despite the above; urgent
Echo if tamponade is suspected.
If Aspirin stopped within 4days of surgery, give DDAVP if post-op bleeding is a problem.
Hypertension
Common after repair of coarctation beyond the newborn period and after heart transplant.
Other causes are pain, awareness, fits, full bladder, hypercarbia, vasoconstriction.
Examine chest, abdomen, pupils and fontanelle. Check blood gases and glucose. Give a
morphine bolus and reassess. Give a midazolam bolus and reassess. Start infusion of
sodium-nitroprusside (SNP): start with 0.1mcg/kg/min and increase gradually to 2 3mcg/kg/min if required (beware of cyanide toxicity and methaemoglobinaemia, especially

rising lactate). In a child > 1year of age, if HR > 100 and still hypertensive, give an IV beta
blocker (esmolol) cave: negative inotropic effect - or alpha blocker (phentolamine). Convert
to bolus drugs when stable (atenolol, phenoxybenzamine, captopril). Avoid giving a calcium
channel blocker plus a beta blocker.
Hypotension
Causes: hypovolaemia; low cardiac output; excessive peripheral vasodilatation in the face
of inadequate or limited cardiac output; has the child received a bolus of vasodilator
(intermittently blocked CVC, sudden increase in flow of other fluid through the same line as
the vasodilator); anaphylaxis; low-resistance pathway from the aorta (eg central shunt,
MAPCAs, AV fistula).
Exclude all the causes of inadequate cardiac output. If hypotension is profound, raise
the legs. Give a fluid bolus 10ml/kg and repeat if necessary (monitor RAP / LAP, may need >
10mmHg in the early postoperative Phase). If MAP < 25mmHg in a neonate or < 40mmHg in
an older child, start external cardiac massage. Notify the cardiac surgeon. Give adrenaline
bolus: 0.1ml/kg of 1:10.000; repeat if necessary and start an adrenaline infusion. If there is
aorto-pulmonary runoff and a high saturation, reduce the FiO2 to 0.21, increase PaCO2 to
45 - 55mmHg and Hb to 140.
Hypoventilation
Cardinal sign: rising PaCO2.
Causes: drugs, brain injury in theatre or post-operative, tracheal secretions, atelectasis,
pneumothorax, pulmonary oedema or chest wall oedema, large leak around ETT or in
ventilator circuit, changed ventilator settings, gas in stomach or recently started PD, muscle
relaxant ceased (reduced chest wall compliance)
Signs: tachycardia and sweating, falling saturation and rising PaCO2, rising PA pressure,
BP may rise (hypercarbia) or fall (impaired myocardial performance)
Management: examine chest and abdomen, blood gas, hand-ventilate, listen to chest,
suction ETT yourself; obtain CXR, check ETT and ventilator circuit for leaks, check ventilator
settings, increase ventilation or change ventilation mode if necessary, aspirate NG tube,
drain ascites, hand ventilate and suction with saline for atelectasis.
Hypoxaemia
Falling PaO2 or falling saturation. Causes: any of the causes of hypoventilation; right-to-left
shunt: intracardiac or intrapulmonary; parenchymal lung disease; pulmonary oedema;
atelectasis; pneumonia; intrapulmonary haemorrhage.
Investigation: Is it real? If SpO2 falling, rapidly check the oximeter pulse wave, try the
probe on yourself, change probe site. Take a blood gas sample immediately (noting the
oximeter reading at the time) and monitor the patient closely for signs of cyanosis,
hypotension and low cardiac output. Examine the chest. Manually ventilate and suction the
trachea yourself. CXR. Investigate hypoventilation if PaCO2 raised. Take blood from LA and
arterial lines and measure saturation to look for intracardiac right-to-left shunt. Bubblecontrast echocardiograph to locate intracardiac R-to-L shunt.
Pulmonary Hypertension
Usually occurs on a background of high pulmonary blood flow or left heart obstruction. Acute
rises in PA pressure usually occur in response to hypoxia, hypercarbia, acidosis or handling
but may also occur with transfusion of platelets or FFP or infusion of Protamine. It can also
occur without stimulus or warning. High risk patients:
keep well sedated & paralysed for first 4-8 hours. Fentanyl 1 2mcg/kg pre suction and
handling

 minimize handling
aim for PaCO2 30-35, PaO2 >120 mmHg, pH >7.4
dobutamine plus milrinone is a good combination for systemic cardiac output and
pulmonary vasodilation
start NO (10ppm) if increasing PA pressure causes tachycardia, hypotension, desaturation
and signs of poor cardiac output or if mean PA pressure > half mean systemic BP
In patients without a PA line, pulmonary hypertension may be indicated by acute
desaturation, decreased lung compliance, wheeze and hypotension. @ Pulmonary
Hypertension)
ETT Suction
Tracheal stimulation can cause severe increases in PA pressure. When suction is
considered necessary, pre-medicate with fentanyl (1 - 2mcg/kg) to ablate airway
responsiveness. Suction the ETT cautiously and quickly.
Sepsis
Increase in temperature (@ Fever and @ Infection); decrease in cardiac output; increase in
pulmonary artery pressure; warm skin, bounding pulses and reduced aortic diastolic
pressure; oliguria; decline in conscious state; increasing lactate and metabolic acidosis;
unexplained increase or decrease in blood glucose; increased CRP or PCT; decreased
platelet count.
Investigation: examine the child for evidence that sepsis is present and for a septic focus:
wound, lungs, cannulation sites (including signs of caval thrombosis), meningitis,
endocarditis (new murmurs, skin infarcts, fundi, splenomegaly, urinalysis), ears, paranasal
sinuses (especially with prolonged nasal intubation), bones, joints, urinary tract. Repeat FBE
and CRP. Blood cultures: percutaneous, arterial line and central venous cannula. Do not
culture arterial line tip. Consider formal non-bronchoscopic bronchoalveolar lavage if there
are lung opacities on chest x-ray. Culture drain fluid. Culture any pus and send a pus smear
on a microscope slide for Gram stain. Culture urine from suprapubic aspirate or catheter (not
from a bag specimen). Think of fungal sepsis: examine skin, mouth, larynx, fundi. Arrange
ultrasound examination of kidneys.
Management: consider antibiotics (choice depends on probable organism: flucloxacillin (or
vancomycin) plus gentamicin usually appropriate when the organism is unknown; monitor
drug levels carefully; add oral nystatin). Review culture results and CRP daily. Cease
antibiotics after 48 hours if culture results remain negative and clinical evidence of sepsis
gone. Otherwise, continue antibiotics for 5days (longer for severe and intractable infections
such as mediastinitis and endocarditis).
Sweating
Causes: pain, awareness, high PaCO2 (inadequate alveolar ventilation), hypovolaemia,
low cardiac output, hypoglycaemia, heart failure, drug withdrawal. Examine child (hydration,
vein status, response to voice, passive movement and tracheal suction; other signs of
sympathetic stimulation (such as pupils); review chart (change in pressures, HR, respiratory
rate, temperature and ventilation); hand-ventilate and suction trachea; check gas and
glucose; trial of fluid bolus 5 - 10ml/kg; trial of morphine bolus 50mcg/kg, repeat if necessary;
try IV midazolam.
Tachycardia
An important sign that something is wrong. You must identify the cause: arrhythmia, low
cardiac output, pulmonary hypertensive crisis, hypoventilation or hypoxaemia,

hypoglycaemia, central (fits, fever, pain or full bladder), drugs (pancuronium or inotropes),
anatomy (eg small LV).
Examine the child: chest, abdomen, pupils, fontanelle. Check the heart pressures, temp,
urine output, ECG, atrial electrogram. Check blood gases and electrolytes and glucose.
Echo.
Tachypnea
If the respiratory rate rises progressively, a cause must be found.
Causes: pain or other distress; restrictive lung disease (pulmonary oedema, atelectasis,
pulmonary haemorrhage, pneumonia); pneumothorax or pleural effusion; fever; sepsis;
metabolic acidosis; pulmonary hypertension; neuromuscular weakness (residual relaxants or
other cause).
Investigation: examine (chest, abdomen, pupils, muscle power, autonomic signs of
distress, response to voice, passive limb movement and tracheal suction); review chart (PA
and LA pressure, BP, temperature, urine output); blood gas; hand-ventilate and personally
suction trachea; repeat CXR; consider trial bolus of morphine or midazolam; CRP; platelet
count; culture blood, urine, tracheal aspirate and drain fluid; observe pattern of ventilation
(shallow tachypnoea versus hyperpnoea; coordination with the ventilator). Increase
ventilation (mandatory rate or support pressure) if muscle weakness present.
Ventilator dependence
A high pCO2 may be appropriate if there is metabolic alkalosis caused by hypochloraemia
from diuretic use. Respiratory depression. Drugs or encephalopathy. Irregular, shallow
breaths; high PaCO2; sleepy; may be other evidence of encephalopathy (eg fits); often
prolonged or high-dose morphine or midazolam infusion; wait (days) for sedatives to be
excreted; neurological examination; check fontanelle; cerebral ultrasound (insensitive) CT
scan (wait several days). Phrenic nerve palsy. Unilateral or (rarely) bilateral; often transient
(weeks); no ipsilateral inspiratory movement of abdomen. Diagnosis: ultrasound and / or Xray image intensifier (screening) both give false negatives. Plication should be considered
early in a small infant with unilateral palsy who has failed extubation, and after a week of
failed attempts in an older child (especially in palliative repair).
Neuromuscular weakness. Residual muscle relaxants; previous period of poor cardiac
output; impaired liver or kidney function; edema or ascites fluid store relaxant drugs;
prolonged or high dose relaxants (especially if doses given before child moves). Diagnosis:
train of four. Management: wait until movement returns (can lift legs off bed) before giving
neostigmine-atropine; don't rely on neostigmine-atropine to reverse a profoundly paralysed
child. ICU myopathy (prolonged IPPV + relaxants steroids sepsis; severely ill with normal
train of 4); EMG and consult neurologists if suspected; pressure support ventilation + good
nutrition + wait (avoid steroids and muscle relaxants)
Pleural effusion. If drainage required (after discussion), send fluid for culture, cell count,
triglycerides. Triglyceride >1.1 mmol/L (if fed) and cells > 1000/microL with lymphocytes >
80% suggests chylothorax; Echo and Ultrasound (exclude SVC obstruction), change to
Monogen, or stop feeds and give TPN (77% respond at a mean of 12 days, 45 days if MCT
given); if no response by 14 days, consider trial of octreotide 5 mcg/kg/hr IV @ Chylothorax
Tracheobronchomalacia. Wheeze, prolonged expiration, and active use of expiratory
muscles; gas trapping clinically and on CXR; bronchogram and / or bronchoscopy; use high
CPAP (10 - 15 cmH2O); wean CPAP using deep sedation (morphine chloral diazepam
chlorpromazine); anticipate days to weeks of repeated attempts to wean.
Residual cardiac abnormality. Left-to-right shunt; obstruction in left heart or pulmonary veins;
left-sided AV valve dysfunction; hypoplastic LV; PA stenosis or distortion in BCPS or Fontan
patients. Cardiac catheter re-operation.
[1] Pediatr Cardiol. 2013 Feb;34(2):341-7. McDonald ET AL: Impact of 22q11.2 deletion on the postoperative course of children
after cardiac surgery.

THE BASICS: DEFINITIONS

Aortic cross-clamp time. Duration of clamping of the aorta during bypass. Independent risk
factor for postoperative mortality.
CCF. Congestive Cardiac Failure.
Cardio-pulmonary bypass (CPB). All blood returning to the right atrium is pumped to a
device that adds O2 and removes CO2, and the blood is then returned to aorta.
Circulatory arrest time (arrest time). Duration of total circulatory arrest
(Cox) Maze Procdure. Surgical procedure with left atrial, right atrial and atrioseptal
incisions to prevent atrial flutter / fibtrillation.
DHCA. Deep Hypothermic Cardiac Arrest.
Extracorporeal membrane oxygenation (ECMO). A form of extracorporal life support, but
without the means of returning blood lost into the thorax back to the circuit.
LVEDP. Left Ventricular enddiastolic pressure.
LVH. Left Ventricular Hypertrophy.
MR or MI. Mitral Regurgitation / Insufficiency.
MS. Mitral Stenosis
PBF. Pulmonary Blood Flow.
Qp. Pulmonary Blood Flow.
Qs. Systemic Blood Flow.
Qp : Qs. Ratio of pulmonary to systemic blood flow. (normal physiology 1 : 1)
RVH. Right ventricular Hypertrophy.
RVOTO. Right-ventricular outflow obstruction.
SmvO2. Mixed Venous Saturation. Indication for oxygen consumption and CO (SaO2
SmvO2 < 30%)
TR or TI. Tricuspid Regurgitation / Insufficiency
TS. Tricuspid Stenosis.
Ventricular assist device (VAD). Form of extracorporal life support, where a blood pump
with axial, laminar or pulsatile flow to augment the function of the left ventricle (LVAD), right
ventricle (RVAD), or both (BiVAD - using two pumps).

THE BASICS: FORMULAE

Alveolar Gas Equation = (Patm PH2O) x FiO2 paCO2 / RQ
(Patm 760mmHg atmospheric pressure at sea level, PH2O 47mmHg water vapour
pressure, FiO2 inspired oxygen concentration, RQ respiratory quotient = CO2 eliminated
/ O2 consumed)
Body Surface Index (BSA) =
= ( [Height(cm) x Weight(kg) ] / 3600 )
Mean Arterial Blood Pressure (MAP) = (SBP + 2 x DBP) / 3
Transpulmonary Gradient (TPG) =
= mPAP PCWP or in Glenn / Fontan: SVC (CVP) - LAP
Cardiac output (CO) = SV x HR. normal: 2.1 3.5 l/min/m2
Cardiac index (CI) = CO / BSA. normal 3.0 5.5 l/min/m2
Systemic vascular resistance index (SVRI) =
= 80 x (MAP - CVP) / CI. normal 800 - 1600 dyne*sec/cm5/m2. SVRI / 80 = normal 15 - 30
Wood unit / m2
Pulmonary vascular resistance index (PVRI) =
= 80 x (MPAP - LAP) / CI. normal 80 - 240 dyne*sec/cm5/m2. PVRI / 80 = normal 1 - 3
Wood unit / m2
Stroke Volume (SV) = CO / HR. normal 1 1.5 ml/kg
Ejection Fraction (EF) = (EDV ESV) / EDV. normal 55 75 %
Fractional Shortening (FS) = (LVEDD LVESD) / LVEDD. normal 28 45 %
Modified Bernoulli Equation: p1-p2=4 x v2.
relates the pressure drop (or gradient) across an obstruction
Flow resistance. Poiseuilles Law: R = 8 x x L / x r4
( = viscosity, L = length, r = radius). laminar flow only
Right ventricular Pressure (RVP) = 4 x (TR Vmax)2 + RAP
Pulmonary to systemic blood (Qp : Qs) =
= (SaO2 - SmvO2) / (SpvO2 - SpaO2). normal 1 : 1
in parallel circulation Qp : Qs ~ 25 : (95 SaO2)
Oxygen Delivery (DO2) = CI x Hb (g/l) x SaO2 x 1.36 x SaO2 / 100
Oxygen consumption (VO2) =
= CI x Hb (g/l) x 1.36 x ((SaO2 - SmvO2) / 100).
normal: infant 160-180, child 100-130, adult 120-150 ml/min/m2
QT interval. Bazett's formula: QTc = QT (sec) / SqrRt of previous RR interval (sec). normal
approximately < 0.44 sec

THE BASICS: CARDIAC CYCLE

NORMAL VALUES
Heart Rate
Threshold
(bpm)
Term Newborn
120 180
up to 1yr
100 180
up to 2yrs
80 - 130
up to 7yrs
70 - 110
up to 16yrs
50 100

MAP
(mmHg)
45
55
60
65
65

[1] Pediatr Crit Care Med 2009 Vol. 10, No. 3: Bronicki et al: Cardiopulmonary Interaction.

THE BASICS: FRANK STARLING MECHANISMS

Increased Preload

Increased Preload
(A B)
increased LV Volume
increased Stroke Volume
Increasing Preload above diastolic
compliance
Failure (F)

Improved diastolic compliance

Improved diastolic compliance

(A B)
increased LV Volume
increased Stroke Volume

Increased Inotropy

Increased Inotropy
(A B)
increased Stroke Volume

Decreased Afterload

Decreased Afterload
(A B)
increased Stroke Volume

THE BASICS: INFECTION

Surgical Site Infection (superficial / deep / organ):
Prevalence 5 - 10%
within 10 14days post surgery
most common: Staphylococcus aureus
risk factors: neonate, HLHS, hospitalization prior surgery, TPN, emergency procedures,
long CPB
Blood Stream Infection:
Prevalence 5 - 10%
within 10 14days post surgery
most common: gramnegative organism (Pseudomonas, Enterbacter)
risk factors: surgical complexity, open sternum, low body weight, longer duration of central
line, prolonged ICU stay
Pulmonary Infection:
Prevalence 10%
risk factors: prolonged mechanical ventilation, surgical complexity, low cardiac output
syndrome, failed extubation
Current recommendation for antimicrobial Prophylaxis in Cardiac Surgery: Cefazolin up to
72hrs (prolonged use may increase antimicrobial resistance). In the setting of either a
presumed or known Staphylococcal colonization, the institution presence of a high incidence
of MRSA, patients susceptible to colonization, or an operation for a patient having prostethic
valve or vascular graft insertion, it would be reasonable to combine the beta-lactam with a
glycopeptide (Vancomycin) for prophylaxis.
Special considerations in immunodeficient syndromes (DiGeorge Syndrome, @
Chylothorax)
See also @ Sepsis and @ Fever
[1] Am J Infect Control 2010 Nov;38(9):706-710: Sohn et al: Risk factors and risk adjustement for surgical site infections in
pediatric cardiothoracic surgery patients
[2] Pediatr Cardiol 2010 May;31(4): 483-9: Abou Elella et al: Impact of bloodstream infection on the outcome of children
undergoing congenital heart surgery
[3] Am J Health Syst Pharm 2008 Nov 1;65(21): 2008, 2010: Survey of congenital heart surgeons preferences for antimicrobial
prophylaxis for pediatric cardiac surgery patients
[4] Ann Thorac Surg 2007 Apr; 83(4): 1569-76: Engelman et al: The Society of thoracic surgeons practice guideline series:
Antibiotic prophylaxis in cardiac surgery, Part II: Antibiotic Choice

THE BASICS: EMPIRIC ANTIBIOTIC GUIDELINES FOR GENERAL INFECTION

Age

if meningitis excluded

if meningitis is NOT
excluded
Amoxicillin 50mg/kg q6hr

< 3 months

Amoxicillin 50mg/kg q6hr

Gentamycin 7.5mg/kg q24hr

Gentamycin 7.5mg/kg q24hr

Cefotaxime 50mg/kg q6hr
consider especially in infants
Aciclovir 20mg/kg q8hr

> 3 months

any age group,

if immunocompromised

Cefotaxime 50mg/kg q6hr

Cefotaxime 50mg/kg q6hr

Flucloxacillin 50mg/kg q4-6hr

Flucloxacillin 50mg/kg q4-6hr

if Staphylococcus aureus
suspected
Amoxicillin 50mg/kg q6hr
Cefotaxime 50mg/kg q6hr
Vancomycin 15mg/kg q6hr
Clindamycin 15mg/kg q8hr
Cefotaxime 50mg/kg q6hr
Vancomycin 15mg/kg q6hr
Clindamycin 15mg/kg q8hr

Meropenem 20mg/kg IV q8hr

Vancomycin 15mg/kg q6hr
Gentamycin 7.5mg/kg q24hr

if Meningitis is NOT excluded, and suspicion of severe Meningitis (gram stain), replace Flucloxacillin by
Vancomycin 15mg/kg q6hr to cover for Penicillin resistant Pneumococcus Meningitis

THE BASICS: CENTRAL VENOUS CATHETERS

Use of central venous catheters in the acute care setting is an integral approach to deliver
fluids, blood products, nutrients, medications, obtaining blood specimens, maintaining
emergency vascular access, and for haemodynamic monitoring.
Risk factors: mechanical complications (malposition, occlusion, dislodgement, tamponade),
infection, pneumothorax, thrombosis
Insertion:
Ask nurse to complete the checklist and to stop you if you are about to breach the rules !
maximal sterile barriers for insertion
use chlorhexidine lollipops the use of liquid in pot is absolutely forbidden !
dedicated equipment cart easily accessed
use of a procedural pause stop the line if barrier precautions are breached
use of chlorhexidine impregnated patch at insertion site
appropriate dressings used over insertion site
radiographical confirmation of catheter tip position
always transduce pressure waveform (with heparin !!)
details of insertion documented in patient record
Maintenance:
commence Heparin 10U/kg/hr in patients < 5kg
daily review of lines with prompt removal of unnecessary lines
use of closed needless mechanical valve on each lumen
[1] http://www.cdc.gov/mmwr/preview/mmwrhtml/rr5110a1.htm
[2] The Pediatric Infectious Diseases Journal, 2010; Sept 29(9): 812 -815: Prasad et al: Risk Factors for Catheter-associated
Bloodstream Infections in a Pediatric Cardiac Intensive Care Unit.

ANESTHESIA: ANALGESIA AND SEDATION

Basic Pharmacology for PICU
Routes of administration and systemic absorption of drugs:
Rate of systemic absorption determines onset, intensity and duration of action. Drug
solubility and blood flow to the site of absorption are the most important factors:
Oral/enteral:
most convenient & economic route of administration
complicated by nausea/emesis & irregularities in absorption
principle site of absorption is the small intestine
GI mucosa and the liver contribute to extraction and metabolism of drugs
Oral/nasal transmucosal: drains to the SVC and / or VIJ and bypasses 1st pass hepatic
metabolism
Rectal:
highly unpredictable and irritant to rectal mucosa
absorption is slow due to the small available surface area
distal rectal administration will bypass 1st pass hepatic metabolism
proximal rectal administration will not bypass 1st pass hepatic metabolism
Parenteral:
includes subcutaneous, intramuscular and intravenous routes
absorption is more reliable and complete
IV administration avoids factors that limit systemic absorption by other routes and is a
more comfortable way to administer irritant drugs
Neuraxial:
epidural route is used to provide analgesia and anesthesia
significant systemic absorption may occur through the epidural venous plexus especially
with lipid soluble drugs and continuous infusions (eg. fentanyl)
intrathecal or spinal administration rarely causes unwanted systemic effects
Distribution of drugs after systemic absorption:
Highly perfused tissues (heart, lungs, brain, kidneys and liver) receive a disproportionate
amount of drug and initially sequester it from the plasma. Once plasma concentration falls
following a bolus dose, drug will redistribute back into the plasma.
Following a bolus dose, plasma concentration first falls rapidly during the distribution phase
and then more gradually during the elimination phase.
Remember that increasing an infusion of a drug to increase its desired effect should
be preceded by a repeat bolus / load otherwise its effect will take ~5 half times !
Repeated large doses and / or prolonged infusions will saturate inactive tissues which will
then act as reservoirs and prolong the duration of action of drugs.
Pharmacokinetic variables:
Volume of distribution (Vd):
apparent volume a drug is injected into (calculated from dose and initial plasma
concentration before any clearance)
determinant of elimination half time (t)
depicts the distribution characteristics of a drug in the body
used to determine loading doses
mainly influenced by physicochemical characteristics of the drug
Metabolism:
hepatic microsomal enzymes are responsible for most drug metabolism
hepatic extraction may be perfusion dependent (affected by hepatic blood flow) or capacity
dependent (affected by ionisation and protein binding)

 lungs (eg. catecholamines), kidneys (eg. morphine) and the GIT have considerable drug
metabolising ability
Plasma cholinesterase and non-specific esterases are important in drugs containing ester
bonds (eg. Esmolol, Succinylcholin)
Hoffman elimination is spontaneous non-enzymatic breakdown (eg. cisatracurium)
Clearance (Cl):
volume of plasma cleared of drug per unit time
determinant of elimination half time (t)
metabolism, excretion and non-organ clearance (eg. ester hydrolysis) all contribute to
clearance
may be 1st order (proportional to plasma concentration) or zero-order (constant amount of
drug cleared independent of plasma concentration)
Half times:
time necessary for the plasma concentration of a drug to decrease by 50% (t ~ Vd/Cl)
can be during distribution ( t) or elimination (t)
plasma concentration does not always correlate with the clinical effect of the drug
elimination half time determines the dosing interval to achieve steady state (~5 half times)
context sensitive half time (CSHT) is the time necessary for the plasma drug concentration
to decrease by 50% after ceasing a continuous infusion of a specific duration (context =
duration of infusion)
Effect site equilibration time (ESET):
delay between IV administration and onset of clinical effect reflects the delay in delivery of
the drug to its site of action and subsequent dynamic response
mainly determined by physicochemical properties of the drug
important in determining dosing intervals when titrating to effect
Physicochemical properties of drugs:
Ionization: most drugs are present as both ionized and non-ionized molecules / proportion
is determined by the pK of the drug and the pH of the surrounding fluid / only non-ionised
drug is free to diffuse across membranes, be metabolized or be excreted
Protein binding: a variable amount of drug may be bound to various plasma proteins which
affects distribution / clinically significant protein binding is > 90% / acidic and neutral drugs
generally bind to albumin and alkaline drugs generally bind to alpha1-acid glycoprotein / only
unbound drug is free cross membranes, be metabolized or excreted
Molecular size: small molecules diffuse much more readily than large ones.
Lipid solubility: ability to physically diffuse through cell membranes (does not necessarily
correlate with rapid onset of action).
Isomerism: mixtures often contain either inactive isomers or isomers that have different and
/ or adverse clinical effects (racemic and non-enantiopure preparations can be considered
mixtures of different drugs).
Individual variability in dynamic response:
The response (therapeutic and adverse effects) to many drugs varies widely among
patients.
There is up to a five-fold range of plasma concentrations required to achieve the same
pharmacologic effect in different individual patients.
There is up to a two-fold range of plasma concentrations required to achieve the same
pharmacologic effect in the same patient using the same dosing regime.
Absorption and bioavailability as well as variations in cardiac, renal and hepatic function
contribute to inter- and intra-individual variability.
Enzyme activity (eg. induction/inhibition) and genetic factors (eg. fast / slow acetylators)
also play a role.

Effects of age and disease:

Renal disease will affect drugs excreted by the kidneys to an extent proportional to the
degree to which the drug depends on renal excretion.
Hepatic disease alters plasma protein levels (decreased binding), increases Vd (ascites),
reduces metabolism and may alter bioavailability (decreased 1st pass metabolism and / or
porto-caval collaterals).
Neonates and Infants:
proportionally more water, larger intravascular volume and larger highly perfused organs
immature blood-brain barrier makes them more sensitive to drugs acting in the CNS
immature and inefficient hepatic metabolizing capacity and lower plasma protein levels
GFR < 10% of adult values will affect clearance

Sedatives and Analgesics

Intravenous anesthetic agents (see Table):
classified as barbiturates (Thiopentone) and non-barbiturates (Propofol and Ketamine)
Thiopentone use is largely limited to induction in status epilepticus and for treatment of
raised ICP; it has no analgesic properties and is in fact anti-analgesic at sedative doses.
Propofol is suitable for induction (bolus) and maintenance of sedation / anesthesia
(infusion); it is suitable for discrete painful procedures but has only minimal analgesic
properties at sedative doses and so must be combined with a suitable analgesic.
Propofol is a direct myocardial depressant and so should be used in caution in patients in
(or at risk of) low cardiac-output syndrome (LCOS). It obtunds the normal baroreceptor reflex
and so causes a decrease in blood pressure and heart rate.
Ketamine is a dissociative anesthetic that is also a potent analgesic; it is suitable for
discrete painful procedures but increases respiratory secretions and is complicated by
psychadelic phenomena. Midazolam is suitable to treat or obviate Ketamine's emergence
phenomena but will prolong recovery time.
combined Ketamine and Propofol in a ratio ranging from 1:1 to 1:4 (Ketofol) is becoming a
popular awake-sedative to facilitate medical procedures.
Narcotics (see Table):
Morphine, Fentanyl and Methadone are effective analgesics and sedatives; Oxycodone is
also a popular analgesic but is less sedating.
Levels of sedation, analgesia and respiratory depression do not correlate (patients may be
well sedated and have respiratory depression but not have adequate analgesia).
Morphine is not a suitable narcotic for discrete painful procedures due to its long and
unpredictable effect site equilibration time (Fentanyl is more appropriate).
Fentanyl is often used epidurally and results in significant systemic absorption of drug and
resulting side effects.
Sufentanil, Alfentanil and Remifentanil are phenylpiperidine narcotics used to provide the
analgesic component of general anesthesia. They are very infrequently used in PICU.
All have predictable effects which include respiratory depression, cough suppression,
sedation, meiosis, biliary spasm, constipation, nausea and vomiting, urinary retention and
cutaneous flushing (especially about the face).
Benzodiazepines (see Table):
Midazolam and Diazepam are effective sedative agents commonly used in PICU.
They are direct myocardial depressants via blockade of voltage-gated calcium channels
(use carefully in patients with LCOS).
Midazolam is also used to acutely treat seizures in bolus doses and in infusions (up to
1mg/kg/hour).
They are less likely to produce withdrawal syndromes than barbiturates and narcotics (but
no analgesic effect).
Alpha2 agonists (see Table):
Clonidine and Dexmedetomidine are sedative / anaesthetic agents employed as sedatives
in PICU; they also treat symptoms of drug withdrawal.
Their main advantage is lack of respiratory depression which allows quicker weaning of
mechanical ventilation.
They obtund central (brain and spinal cord) sympathetic outflow resulting in negative
inotropy and chronotropy and so should not be combined with direct myocardial depressants
(Benzodiazepines, Propofol etc.) in patients at risk of (or in established) LCOS.
They cannot be bloused as this can lead to transient alpha1-agonism and severe
hypertension.

Local anaesthetics:
Local anaesthetics block voltage gated sodium channels and so prevent conduction along
central and peripheral nerve pathways.
Lignocaine is commonly locally infiltrated for short painful procedures (eg. suturing,
insertion of chest drains etc).
Bupivacaine and Ropivacaine are generally used for regional blocks and neuraxial
infusions.
Levobupivacaine (S-bupivacaine) and Ropivacaine are enantiopure preparations.
Cardiotoxicity is less.
0.5% solutions contain 5mg/mL; 1% solutions contain 10mg/mL; 2% solutions contain
20mg/mL etc. (1% = 10mg/ml)
Onset of effect is related to the pKa of the drug; potency is related to lipid solubility; and
duration of action is related to protein binding.
Systemic absorption of local anaesthetics depends on site of infiltration: intercostal >
subarachnoid > epidural > brachial plexus > femoral > subcutaneous.
Vasoconstrictors (Adrenaline) slow systemic absorption and increase the maximum safe
dose
EMLA is a mixture of 2.5% Lignocaine and 2.5% Prilocaine used for topical anaesthesia
prior to cannulation / incision; Prilocaine can induce Methaemoglobinaemia and application
to mucous membranes will result in rapid systemic absorption of drug.
CNS toxicity manifests first as excitatory phenomena (circumoral tingling, tinnitus,
dizziness and tremors / seizures) followed by CNS depression (unconsciousness, apnoea
and coma).
CVS toxicity manifests as systemic hypotension, myocardial depression, ventricular
arrhythmias and cardiovascular collapse.
Treatment of local anaesthetic toxicity is by supportive therapy (airway management,
treatment of seizures with Benzodiazepines, fluids +/- inotropes / vascoconstrictors) and
administration of 20% lipid emulsion (Intralipid) if in cardiac arrest: 1.5mL/kg over 1 minute
followed by an infusion of 0.25-0.5ml/kg/min; repeat bolus doses every 5 minutes during
CPR.
Non-steroidal anti-inflammatory drugs (NSAIDs):
Classified as specific (COX-2 eg. Parecoxib) or non-specific (COX-1 and COX-2 eg.
Ibuprofen).
Adverse GI effects are due to decreased mucosal blood flow and decreased secretion of
mucus and bicarbonate.
Platelet thromboxane A2 is produced from prostaglandins and so NSAIDs impair platelet
aggregation.
Prostaglandins are vasodilators involved in physiologic control of vasomotor tone
(especially in the kidneys) and their inhibition leads to unopposed vasoconstriction.
Inhibition of prostaglandin synthesis leads to shunting of arachnidonic acid to lypoxygenase
which is a bronchoconstrictor.
Specific COX-2 inhibitors are considered to lack effects on platelets and the GIT but will
still affect vasomotor tone.
Their use in PICU needs careful consideration due to their wide range of potential side
effects.
Paracetamol is generally considered a (central) COX-3 inhibitor; it also acts peripherally by
inhibiting bradykinin-chemoreceptor associated pain impulse generation.

Other (see Table):

Chloral hydrate is a prodrug that produces the halogenated alcohol Chloroethanol; its
mechanism is poorly understood but probably acts in a similar way to the volatile
halogenated gases via central GABA-A receptors.
First-generation antihistamines (eg. Promethazine, Cyclizine etc.) are also effective
sedatives by virtue of their anticholinergic properties; they are generally only used when
specific antihistaminergic and / or anticholinergic properties are desired (eg. antisialogogue
for secretions, anti-tussive).

Table: intravenous anesthetic agents

Type/class

Thiopentone
Barbiturate

Mechanism

GABAA & glycine agonist

Propofol
Isopropylphenol
GABAA & glycine direct
agonist and central
nicotinic antagonist
(Possible 5HT3 blockade)

Ketamine
Phencyclidine
NMDA non-competitive
antagonist & blocks central
catecholamine reuptake

Oral
bioavailability
Oral dose

25%

n/a

n/a

IV Bolus

2-7mg/kg

1.5-2.5mg/kg

5mg/kg
0.25-0.5mg/kg (analgesia)
1-5mg/kg (GA)

IV Infusion

1-5mg/kg/hour

1-4mg/kg/hour (sedation)
5-15mg/kg/hour (GA)

10-40mcg/kg/hour

Onset time IV
ESET
pKa
UNionised
fraction
Protein
binding
Vd
Clearance
t -dist
t -elim

< 30seconds
30 seconds
7.6

< 30seconds
< 30 seconds
11

30-60seconds
60seconds
7.5

60%

> 99%

45%

80%

99%

25%

2.5L/kg
3mL/min/kg
8minutes
12hours

3L/kg
15mL/min/kg
12minutes
2-3hours

Excretion

Urine

4L/kg
50mL/min/kg
4 minutes
30-60minutes
-Hepatic (CYP2C9 & 2B6)
& extrahepatic (site/s
unknown)
Inactive metabolites
Urine

Hepatic failure

No effect

No effect

Decreased clearance

Renal failure

Active metabolites will

accumulate

No effect

No effect

Metabolism

Hepatic (may become zeroorder with prolonged infusion)

Some active metabolites

Pros

Rapid onset
Anticonvulsant
Can produce isoelectric EEG
(maximal decreased cerebral
metabolic O2 demand)

Cons

Resp depression / apnoea

Antanalgesic
Can produce paradoxical
excitement
Will accumulate with prolonged
infusion (long CSHT)
Tolerance & withdrawal are a
problem

Other points

BP (SVR)
HR (reflex)
Wont obtund airway reflexes
Racaemic formulation

Rapid onset & titratability

Bronchodilator
Will obtund airway reflexes
Anticonvulsant
Antiemetic & antipruritic
properties at low doses
Can produce isoelectric
EEG
Mild analgesic properties
Stable CSHT (< 40mins
even after > 8 hrs infusion)
Resp depression / apnoea
Myocardial depressant
Can cause a refractory
bradycardia (need agonist)
Rarely causes propofolinfusion syndrome
Formulation contains
soybean oil & egg lecithin
BP
(SVR & CO)
HR (obtunded
baroreceptor reflex)

Hepatic
Active metabolites
Urine

Intense analgesia at low dose

Favourable haemodynamic
profile due to increased central
sympathetic outflow
Bronchodilator
Prevents & treats opioid
tolerance
No respiratory depression /
apnoea
Myocardial depressant
Emergence delirium
(especially in older patients
consider midazolam)
Increased secretions (consider
glycopyrrolate)
BrainZ/BIS inaccurate
EEG dissociation between
thalamus & cortex
Wont obtund airway reflexes
Typical induction agent in
asthma & sepsis

Table: Benzodiazepines
Midazolam
BDZ
GABAA receptor indirectagonist
40%
0.5mg/kg up to 20mg
0.05-0.2mg/kg
up to 5mg/dose
10-100mcg/kg/hour

Diazepam
BDZ
GABAA receptor
indirect-agonist
95%
0.05-0.2mg/kg
0.05-0.4mg/kg
up to 10mg/dose
n/a

1-2mins

1-2mins

1-2mins

5mins
6.2

5mins
3.3

5-10mins
1.8

90%

>99%

>99%

95%

95%

50%

1.5L/kg
10mL/min/kg
5mins
1-4 hours
Hepatic (CYP3A4)
Active metabolites

1.5L/kg
1mL/min/kg
5mins
24-36 hours
Hepatic (CYP3A4/5)
Active metabolites

Excretion

Urine

Urine

0.5L/kg
20mL/min/kg
5mins
60mins
Hepatic
No active metabolites
90% urine
10% bile

Hepatic
failure

Decreased clearance

Decreased clearance

Decreased clearance

Renal failure

Active metabolite may

accumulate

Active metabolites will

accumulate

No effect

Pros

Sedation, amnesia &

anxiolysis
Anticonvulsant
Decreases cerebral
metabolic O2 demand

Effective orally
Sedation, amnesia &
anxiolysis
Anticonvulsant
Decreases cerebral
metabolic O2 demand

Allows specific reversal of BDZ component

of resp depression and / or polypharmacy
overdose
Rarely causes acute anxiety &/or agitation

Cons

Myocardial depressant
Resp depression
Can cause paradoxical
excitement

Myocardial depressant
Resp depression
Can cause paradoxical
excitement
Painful on injection

Can precipitate seizures in epileptics on

maintenance BDZs

Other points

BP
(SVR & CO)
[HR (reflex)]

BP
(SVR & CO)
[HR (reflex)]

Is probably a partial agonist

Type/class
Mechanism
Oral bioavail
Oral dose
IV Bolus
IV Infusion
Onset time
IV
ESET
pKa
%
UNionised
Protein
binding
Vd
Clearance
t -dist
t -elim
Metabolism

Flumazenil
BDZ
BDZ receptor competitive antagonist
25%
n/a
8-15mcg/kg
up to 200mcg/dose
2-10mcg/kg/hour

Table: Narcotics
Morphine

Fentanyl

Methadone

Naloxone

Type/class

Phenanthrene
opiate

Phenylpiperidine
opioid

Diphenyl-propylamine
opioid

Phenanthrene opioid

Mechanism

Non-specific OR
agonist

MOR agonist with

some mild activity at
KORs

MOR agonist (Lisomer) & NMDA

antagonist (D-isomer)

Non-specific OR
competitive antagonist

Oral
Bioavail.

30%

n/a

75%

<1%

Oral dose

0.2-0.5mg/kg q4-6h

n/a

0.1-0.2mg/kg q6-24h

n/a

IV bolus
dose

0.05-0.2mg/kg

1-10mcg/kg

0.1mg/kg

10mcg/kg

IV infusion

5-100 mcg/kg/hr

1-10 mcg/kg/hr

n/a

10 mcg/kg/hr

Onset time
IV

15-30mins

1-2mins

10-20mins

1-2mins

ESET

30-90mins

3-6mins

10-20mins

5-10mins

pKa

8.0

8.4

9.2

7.9

25%

10%

1%

30%

35%

85%

90%

50%

Vd

3L/kg

4L/kg

3.5L/kg

0.2L/kg

Clearance

25
mL/min/kg

10-20 mL/min/kg

1-3 mL/min/kg

30 mL/min/kg

t -dist

2-3mins

1-2mins

1-2 mins

t -elim

2-4hours

2-4hours

18-36 hours

45-60mins

Hepatic & renal

10% to active M6G
90% urine
10% bile
May precipitate
encephalopathy
Morphine & M6G
will accumulate
No myocardial
depression
Sedation &
euphoria
Antitussive
Respiratory
depression
Histamine release
Nausea & vomiting
Pruritis
Urinary retention
Constipation

Hepatic (CYP3A4)
No active metabolites
90% bile
10% urine

Hepatic (CYP3A4)
No active metabolites
50% urine
50% bile

Hepatic
No active metabolite

No effect

reduced clearance

reduced clearance

No effect

No effect

No effect

No myocardial
depression
Sedation & euphoria
Antitussive
No histamine release
Respiratory
depression
Nausea & vomiting
Pruritis
Urinary retention
Constipation
Prolonged CSHT

Effective enterally
Helpful in withdrawal
syndromes
Suitable for chronic
pain (NMDA actions)
Respiratory
depression
Nausea & vomiting
Constipation
Histamine release
possible but rare
Prolongs QT interval

Acts rapidly & is

titratable
Antiinflammatory
properties

Meiosis
HR & BP (SVR)

Meiosis
HR & BP (SVR)

Meiosis
HR & BP (SVR)

%
UNionised
Protein
binding

Metabolism
Excretion
Hepatic
failure
Renal
failure

Pros

Cons

Other points

Urine

Can precipitate acute

withdraw
Rarely may cause
pulmonary oedema &
Arrhythmia
Usually needs repeat
dosing
1mcg/kg effective for
narcotic pruritis
BP may rise or fall

Table: Alpha2 agonists

Clonidine

Dexmedetomidine

Type/class

Synthetic imidazoline

Synthetic imidazoline

Mechanism

2 adrenoceptor partial agonist

2 adrenoceptor agonist

Oral bioavail

>99%

15%

Oral dose

1-5mcg/kg up to 300mcg

n/a

IV bolus dose

1-5mcg/kg

1-2mcg/kg

IV infusion
dose

0.5-2mcg/kg/hour

0.2-0.7mcg/kg/hour (sedation)
5-10mcg/kg/hour (GA)

Onset time IV

10-30minutes

10minutes

20-30minutes

10-20minutes

pKa

8.0

7.1

UNionised %

20%

50%

ESET

Protein
binding
Vd

20%

95%

2L/kg

1.5L/kg

Clearance

5mL/min/kg

10mL/min/kg

t -dist
t -elim

30minutes
12-18hours

10 minutes
2-3hours

Metabolism

50% hepatic
50% excreted unchanged

Hepatic
No active metabolites

Excretion

Urine (50% unchanged)

Urine

Hepatic
failure

No effect

Decreased clearance

Renal failure

Active drug will accumulate

No effect

Effective sedative
No respiratory depression
Spinal-mediated analgesia (very effective
neuraxially)
Known to be useful in opioid & alcohol
withdrawal syndromes
Raises the shivering threshold
Prolongs regional block by local anaesthetics
Rapid IV administration will agonise 1 receptors
(BP)
Negative inotropy & chronotropy
Dry mouth
Rebound hypertension can occur (worse if
patient is on a TCA or -blocker)
Long half time
HR & BP
CO
Dry mouth may be used therapeutically if
secretions are an issue

Effective sedative
No respiratory depression
Spinal-mediated analgesia
Useful for symptoms of opioid withdrawal
Raises shivering threshold
Prolongs regional block by local
anaesthetics
Short(er) half time

Pros

Cons

Other points

Rapid IV administration will agonise 1

receptors (BP)
Negative inotropy & chronotropy
Dry mouth
Cannot be used neuraxially due to glycine in
preparation
HR & BP
CO
Dry mouth may be used therapeutically if
secretions are an issue

Table: Others
Chloral hydrate

Promethazine

Type/class

Halogenated alcohol

Phenothiazine

Mechanism

Prodrug below data is for trichloroethanol

(active drug)
Probably a GABAA agonist

H1 receptor antagonist & anticholinergic

(antimuscurinic)

Oral bioavail

>99%

25%

Oral dose

10-100mg/kg

0.25-1.5mg/kg

IV bolus dose

n/a

0.25-1.5mg/kg

IV infusion
dose

n/a

n/a

Onset time IV

15minutes (oral)

30-60minutes

ESET

30-60minutes (oral)

1-3hours

pKa

12.7

9.1

UNionised %

>99%

<1%

50%

80%

Protein
binding
Vd

1L/kg

7L/kg

Clearance

not known

15mL/min/kg

t -dist
t -elim

n/a
4-8hours
Hepatic
Metabolites of trichloro-ethanol are inactive
Urine

1-2hours
12hours
Hepatic (CYP2D6)
Inactive metabolites
Urine

Decreased clearance

Decreased clearance

No effect

No effect

Metabolism
Excretion
Hepatic
failure
Renal failure

Pros

Cons

Other points

Effective sedative & anxiolytic

Rapid onset following enteral administration
Mild anticonvulsant
Relatively wide therapeutic index
Minimal interference with REM-sleep
Respiratory depression in high doses
Irritant to GI mucosa
Arrhythmias in high doses
Trigger for porphyria
Patients can develop tolerance & withdrawal
BP (SVR)
HR (reflex)
Actual half time of chloral hydrate is minutes
(metabolised by esterases)

Effective antihistamine & antiemetic at low

doses
Effective sedative/hypnotic at high doses
Antitussive
Effective in motion sickness
Useful in allergic pruritis but not opioid induced
pruritis
Respiratory depression is rare
Anticholinergic effects (dry mouth, blurred
vision, urinary retention etc)
Central anticholinergic syndrome in overdose
Prolonged QT-interval & AV-block
Paradoxical excitement may occur
[HR & BP]
Antidopaminergic properties
Local anaesthetic properties

ANESTHESIA: MUSCLE RELAXATION

Definition: Muscle relaxants block transmission at the neuromuscular junction (NMJ) by
interfering with nicotinic cholinergic receptors (AChRs). They are large polar molecules with
small volumes of distribution that are not orally bioavailable and do not cross the placenta or
blood-brain barrier. They have no analgesic, anaesthetic or amnestic properties and so
should never be given without appropriate sedative / anaesthetic drugs.
The clinical indications for muscle relaxation are:
to facilitate intubation of the trachea
to improve surgical and / or procedural working conditions
to facilitate intra-hospital and inter-hospital transfers
to prevent shivering in patients being therapeutically cooled
to facilitate mechanical ventilation including using mechanical ventilation to manipulate
PaCO2 and acid-base status
to improve post-operative stability (especially in high-risk cardiac surgery and laryngotracheal surgery with or without complex / abnormal airway anatomy)
Drugs are classified as depolarising (mimic the actions of ACh) and non-depolarising
(interfere with the actions of ACh).
Suxamethonium is the only depolarising neuromuscular blocking drug still in clinical use.
Non-depolarising neuromuscular blocking drugs are classified as long-acting (pancuronium),
intermediate-acting (rocuronium, vecuronium, atracurium & cisatracurium) and short-acting
(mivacurium).
Drug selection is influenced by desired speed of onset, duration of action and the possibility
of drug induced side effects (see table of drugs).
Among suxamethoniums myriad of adverse effects it is also a known trigger for malignant
hyperthermia (genetically abberant muscle sarcoplasmic reticulum calcium channels) the
treatment is active cooling and Dantrolene 1mg/kg up to 10mg/kg.
Patients with genetically abdnormal pseudocholinesterase will have prolonged
neuromuscular blockade with suxamethonium (choline apnoea) they need supportive care
until it is cleared (severe cases require dialysis to clear the drug) and an assessment of their
pseudocholinesterase function (dibucaine number).
Suxamethonium and rocuronium (see Table: Muscle relaxans rapid onset) are the only
drugs capable of producing intubating conditions in 60 - 90 seconds and so are the only
drugs used for rapid sequence induction (RSI). Suxamethonium has a brief duration of
action where as an RSI-dose of rocuronium will have a prolonged duration of action.
The duration of action of non-depolarising neuromuscular blocking drugs is prolonged by
hypokalaemia, hypocalcaemia, hypoproteinaemia, hypermagnesaemia, dehydration,
acidosis and hypercapnoea.
Potency of neuromuscular blocking drugs is described by the effective dose (ED) necessary
to depress single-twitch depression by 95% in the adductor pollicis muscle ED95
(Intubating doses are generally two times the ED95 dose; the RSI-dose for rocuronium is
four times its ED95); potency is Centrally located muscles (larynx, jaw and diaphragm)
develop neuromuscular blockade faster, experience less profound block and recover more
quickly than in more peripherally located muscles (adductor pollicis). Eyelash reflex and
orbicularis inversely related to onset time.

Monitoring of depth of neuromuscular blockade:

Nerve stimulators are used to monitor the depth of neuromuscular blockade.
There is a margin of safety regarding nAChRs at the NMJ and the generation of a myocyte
action potential such that >75% of nAChRs must be occupied by drug before clinically
significant (and detectable) blockade is apparent.
Neuromuscular blocking drugs must occupy at least 75% of nAChRs before there is clinically
significant and detectable blockade (this is the margin of safety with regard to nAChR
numbers and transmission at the NMJ).
The ulnar or radial nerves are commonly used with the negative electrode on the volar
surface of the wrist directly over the nerve to be stimulated and the positive electrode at least
3cm distal where it cannot interfere with the relevant muscle groups.
A current of 60mA (maximum 80mA) is applied for 0.1ms (maximum 0.3ms) per stimulation;
the patterns of stimulation used in PICU are the train-of-four (TOF) count, tetanic (>30Hz)
stimulation and post-tetanic count.
The TOF ratio is the ratio of the height of the first twitch (T1) to the fourth twitch (T4) this is
not easily interpretable if only using visual and tactile evaluation of the response. The TOF
count (absolute number of twitches) is easier to detect and interpret:
T4 begins to reduce in height at >70% receptor occupancy;
T1 starts to reduce in height at >80% occupancy;
T4 disappears at >90% occupancy;
T1 disappears at >95% occupancy.
Tetanic stimulation (usually 50Hz) is known to increase subsequent twitch height either by
mobilising ACh stores and / or increasing calcium influx into the nerve ending; When the
TOF count is zero (>95% blockade) then a tetanic stimulation and a post-tetanic (TOF) count
can help define deep neuromuscular blockade.
The effects of tetany last for up to 6 minutes and this must be taken into consideration if
repeat testing occurs.
If the TOF-count is zero and the post-tetanic count is also zero this signifies either very
deep neuromuscular blockade or a malfunctioning nerve stimulator (test it on yourself).
Reversal of neuromuscular blockade:
Antagonist-assisted reversal of neuromuscular blockade using anticholinesterases
(edrophonium, neostigmine or pyridostigmine) reflects their inhibition of acetylcholinesterase
(AChE) and the resulting increased ACh at the NMJ to compete for nAChR binding sites.
Neostigmine is generally used at a dose of 4 - 7mcg/kg and is more suitable for
reversing deeper levels of block.
Anticholinesterases produce typical and expected muscarinic side effects (mainly
bradycardia, bronchoconstriction, increased secretions & GI hyper-peristalsis) and so should
be given with an antimuscarinic anticholinergic drug such as Atropine (20mcg/kg) or
Glycopyrrolate (10mcg/kg).
Sugammadex is a cyclodextrin that encapsulates rocuronium and vecuronium and
effectively neutralises them; remaining drug diffuses away from the NMJ and its effects are
reversed.
It acts within 2 minutes and has no other effects (as yet). The complex is excreted in the
urine. The dose for routine reversal is 2 - 4mg/kg; the dose for emergent reversal in a cant
intubate-cant ventilate scenario is 16mg/kg.

Table: Muscle Relaxans rapid onset

Suxamethonium
Type/class

Dicholine ester

ED95

0.3mg/kg
1mg/kg (adults)
2mg/kg (children)
3mg/kg (neonates)
30-60seconds

Intubating
dose
Onset time
Recovery
time
Infusion
dose
VD
Protein
binding
Clearance
t-elim

Rocuronium
Aminosteroid
(intermediate acting)
0.3mg/kg
0.6mg/kg
1.2mg/kg (RSI)
30-90seconds

3-5minutes

20-35minutes

n/a

5-15mcg/kg/min

0.17L/kg

0.3L/kg

99%

30%

40mL/kg/min
3-5minutes

4mL/kg/min
80minutes

Metabolism

Plasma pseudocholinesterase

No significant metabolism

Excretion

Resulting choline is taken up into nerves

<5% unchanged in urine

Bile (50% unchanged)

Urine (30% unchanged)

No effect

t-elim up to 100 minutes

No effect

t-elim up to 100 minutes

Suitable for RSI due to shorter onset
time
No histamine release
Minimally affected by renal & hepatic
impairment
May be reversed with sugammadex

Hepatic
failure
Renal failure

Pros

Cons

Other points

Rapid onset & intense paralysis make it suitable for RSI

Raised intra-gastric, intra-ocular & intra-cranial pressures

Fasciculations that can lead to severe myalgia & even
rhabdomyolysis
Bradycardia (muscarinic) +/- brady-arrhythmias
Hyperkalaemia (more so with neuromuscular disease &
burns)
Malignant hyperthermia
Choline apnoea
Anaphylaxis
80% of an administered dose is hydrolysed before
reaching the NMJs
Repeat doses should always be accompanied by an
anticholinergic (consider routine anticholinergic
administration in infants)

Will accumulate with prolonged

infusions (ensure monitoring of depth
of blockade)

There are rare reports of anaphylaxis

It does cause a small increase in
intra-occular pressure

Table: Muscle Relaxans slow onset

Vecuronium
Aminosteroid
(intermediate acting)
0.05mg/kg

Pancuronium
Aminosteroid
(long acting)
0.06mg/kg

Cisatracurium
Benzylisoquinolinine
(intermediate acting)
0.05mg/kg

0.1mg/kg

0.1mg/kg

0.1mg/kg

3-5minutes

3-5minutes

3-5minutes

20-35minutes

60-90minutes

20-35minutes

0.5-2mcg/kg/min

n/a

1-10mcg/kg/min

0.27L/kg

0.26L/kg

0.2L/kg

60-90%

15-30%

unknown

5mL/kg/min
60minutes
Hepatic with some active
metabolites
Urine (25% unchanged)
Bile (25% unchanged)

2mL/kg/min
132minutes
Hepatic with some
active metabolites
Urine (80% unchanged)
Bile (10% unchanged)

5mL/kg/min
25minutes
Hoffman elimination (no active metabolites)

t-elim up to 3 hours

t-elim up to 6 hours

No change

t-elim up to 2 hours

t-elim up to 48 hours

No change

Pros

Commonly used
medication with
predictable onset &
duration of action
No histamine release
May be reversed with
sugammadex

Long acting
(decreased dosing
requirements)

Non-organ clearance makes it unaffected

by renal and/or hepatic impairment
Stable offset time after prolonged infusions
due to rapid Hoffman elimination
No histamine release

Cons

Will accumulate with

prolonged infusion
(ensure monitoring of
depth of blockade)

Type/class
ED95
Intubating
dose
Onset time
Recovery
time
Infusion
dose
VD
Protein
binding
Clearance
t-elim
Metabolism
Excretion
Hepatic
failure
Renal
failure

Other
points

Large doses may cause

a slight (10-15%) drop in
SVR and BP

Minimally metabolized
so sensitive to effects
on hepatic & renal
function
Risk of arrhythmias in
patients on digoxin
10-15% increase in HR
(mainly anticholinergic
effect)
Mild increase in BP
secondary to increased
HR (no inotropy)
Useful for obviating HR
effects of induction
doses of narcotics
It may decrease the PT
and APTT

Urine

Potent drug with prolonged onset time

One of 10 stereoisomers of atracurium

(atracurium is not used often anymore due
to histamine release and has a metabolite
that can cause convulsions).

ANESTHESIA: TOLERANCE AND WITHDRAWAL

Definition: Tolerance is the development of the need to increase the dose of a drug to
achieve the same effect previously achieved with a lower dose.
Duration of therapy is the major factor associated with onset of tolerance and physical
dependence and continuous infusions induce tolerance more rapidly than intermittent and
enteral therapy.
Tolerance begins within 48 hours of continuous infusion but typically takes 2 3 weeks of
regular intermittent use to develop to a clinically significant extent
Long-term pharmacodynamic tolerance can persist for months-years in some individuals.
The mechanisms are poorly understood but probably involve changes in receptor number
(down-regulation) and modulation of intracellular signalling pathways leading to receptor
desensitisation.
Genetics also play a role in both response to opioids and the development of tolerance and
physical dependence but its clinical importance is still being defined.
Physical dependence also develops to some degree after only 48 hours of continuous
infusion but requires 4 weeks of regular intermittent use to become established.
Discontinuing a drug after physical dependence is established will produce a typical
withdrawal abstinence syndrome.

ANESTHESIA: WEANING OPIODS AND SEDATION

Withdrawal from drugs (principally opioids) prolongs hospital admissions and causes
morbidity !
Gradual weaning of drug dosing aims to prevent the onset of withdrawal abstinence
syndromes:
regime one: 10% reduction in original dose per day weaning over 10days or
regime two: 20% reduction in original dose per day weaning over 5 days
regime three: 20% reduction in original dose every 2nd day weaning over 10days
All are equally effective and the shorter 5-day wean is not associated with any
increased withdrawal symptoms requiring reinstitution of drug therapy !
The choice of regime is typically arbitrary based on length of therapy and clinician choice.
DOSE CONVERSION of IV and ENTERAL
Dose escalation and / or opioid rotation are both effective ways to combat tolerance
(although there is an inevitable amount of cross tolerance) but not physical dependence.
Converting between opioids and route of administration involves documenting the total 24hour dose being administered and then using the conversion table and calculating a total
daily dose of the new drug via the new route.
Drug

IV equivalent

IV : morphine ratio

enteral equivalent

IV : enteral ratio

Morphine
Codeine
Oxycodone
Fentanyl
Methadone

10mg
100mg
10mg
100mcg
10mg

1:1
10 : 1
1:1
0.01 : 1
1:1

30 mg
200mg
20mg
n/a
20mg

1:3
1:2
1:2
n/a
1:2

DOSE CONVERSION for iv Midazolam INTO oral Diazepam

[Midazolam IV [rate in mcg/kg/min] x weight x 24 ] x 0.5 = Diazepam oral
[1] Brunton, L et al (2010), Goodman and Gillman's the Pharmacological Basis of Therapeutics 12th Edition, McGraw Hill
Medical, New York
[2] Macintyre, PE et al (2010), Acute Pain Management: Scientific Evidence 3rd Edition, Australian and New Zealand College of
Anaesthetists and Faculty of Pain Medicine, Melbourne
[3] Miller, RD et al (2009), Miller's Anesthesia 9th Edition, Churchill Livingstone Elsevier, Philadelphia
[4] Peck, TE & Hill, S (2008), Pharmacology for Anaesthesia and Intensive Care 3rd Edition, Cambridge University Press,
Cambridge
[5] Sasada, M & Smith, S (2003), Drugs in Anaesthesia and Intesive Care 3rd Edition, Oxford University Press, Oxford
[6] Stoelting, RK & Hillier, SE (2005), Pharmacology and Physiology in Anesthetic Practise 4th Edition, Lippincott, Williams and
Wilkins, Philadelphia
[7] Pediatrics 2010 May;125(5):e1208-25: Anand et al: Tolerance and withdrawal from prolonged opioid use in critically ill
children.

ANESTHESIA: INTUBATION IN PICU

Indication:
to secure the airway: severe airway obstruction / inadequate protective reflexes (coma or
prolonged seizures)
to facilitate ventilation: hypoxaemic and / or hypercarbic respiratory failure
Intubation should NOT be attempted by the inexperienced if more skilled personnel
are available ! Two doctors always present if possible !
Assessment:
how urgent is the intubation ?
anatomical abnormality, which would predict difficult intubation ?
any evidence of airway obstruction ?
cardiovascular status any hypovolaemia / hypotension ?
is the patient fastened ?
Preperation equipment:
Intubation drugs
Volume replacement (10ml/kg NaCl 0.9%)
ETT (size = age / 4 + 4 for uncuffed ETT for cuffed ETT size = age / 4 + 3.5), one size
above and one size below calculated ETT
Styllete, gum elastic bougie
Laryngoscope with blade (check light bulb and battery)
Magills forceps
Face Mask
Guedel and nasopharyngeal airways
self inflating bag and anaesthetic circuit
suction equipment: Yankauers sucker and suction catheters
connector, cuff inflating syringe, tape
CO2 detector
Procedure:
monitor cardiovascular and respiratory status (ECG, SpO2, BP non-invasive / invasive)
explain to patient / parents
empty stomach if nasogastic tube is in situ
position patient: neutral position in neonates, young children sniffing position in older
children, adolescents
preoxygenation for minimum two minutes
consider atropine 20mcg/kg IV
give analgesic agent
give sedative agent
apply gentle pressure to the cricoid
check for bag and mask ventilation possible with appropriate visual inflation / deflation and
chest wall movement
give paralysis agent
continue bag and mask ventilation, while continuing to apply gentle cricoid pressure,
except in circumstances where bag and mask ventilation is contraindicated (see rapid
sequence induction)
intubate orally, release cricoid pressure
check ETT position: chest wall rise, auscultation and CO2 detector (@ Resuscitation drug
chart)
once patient stabilized and appropriate ventilation, consider to change to a nasal ETT
once ETT position confirmed, tape ETT
insert nasogastric tube, empty stomach
CXR to confirm position of ETT and nasogastic tube

 consider ongoing Analgesia and Sedation

document event
Intubation Drugs:
@ Analgesia and Sedation in PICU
Analgesia
cardiovascular stable, no airway
obstruction > 1 year

cardiovascular stable, with airway

obstruction > 1 year

cardiovascular stable, no airway

obstruction < 1year

cardiovascular stable, with airway

obstruction < 1 year

cardiovascular unstable, any age

Rapid Sequence Induction

patients with raised ICP

anticipated difficult airway

Fentanyl
1 2mcg/kg
or
Morphine
100mcg/kg
Fentanyl
1mcg/kg
or
Morphine
100mcg/kg
Fentanyl
1 2mcg/kg
or
Morphine
100mcg/kg

Sedation

Paralysis

Propofol
1 2.5mg/kg

Vecuronium 0.1mg/kg

Ketamine
1 2mg/kg

Vecuronium 0.1mg/kg

Midazolam
50 -100mcg/kg

Vecuronium 0.1mg/kg

Always seek senior assistance !

Consider induction with volatile anaesthetic !
Fentanyl
1 2mcg/kg
or
Morphine
100mcg/kg
Fentanyl
1 2mcg/kg
or
Morphine
100mcg/kg
Fentanyl
1 2mcg/kg
or
Morphine
100mcg/kg

Vecuronium
0.1mg/kg

Midazolam
50 -100mcg/kg

Rocuronium
1mg/kg

Thiopentone
2 7mg/kg

Rocuronium
1mg/kg

Always seek senior assistance !

Consider induction with volatile anaesthetic !

Unexpected difficult intubation:

call for help !
restart bag and mask ventilation with gentle cricoid pressure
optimize patient position
consider bougie or stylete
consider different laryngoscope blade
Cannot ventilate Cannot Intubate:
call for help !
consider reposition of head
jaw thrust
insert Guedel / nasopharyngeal airway
use both hands to hold mask
release cricoid pressure
consider laryngeal mask (LMA)

ANESTHESIA: INOTROPES AND VASOPRESSORS

Definition:
Inotropes: sympathomimetic agent which act on the sympathetic (or adrenergic) nervous
system (-receptors) resulting in positive inotropic (increase in contractility), chronotropic
(increase in heart rate), dromotropic (increase in conduction of impulse) and lusitropic effect
(improved diastolic relaxation)
Vasopressors: sympathomimetic agent which act on the sympathetic (or noradrenergic)
nervous system (-receptors) resulting in vasoconstrictor effect.
The ideal vasoactive support agent: effect on cardiac output / effect on SVR / effect on
myocardial oxygen consumption / no tachyphylaxis does not exist !
a) Sympathomimetics: endogenous catecholamines:
Adrenaline (1 >> 2 and 1 > 2 agonist) via cAMP
Dose
mcg/kg/min

Clinical effect

HR, SV, CO
() SVR
0.05 0.10
+++
HR, SV, CO
HR, SV, SVR
0.10 0.20
+++
+++
+++
() CO
Side effects: increasing myocardial oxygen requirement, Tacharrhythmias, worsening diastolic function,
Tachyphylaxis, Hyperglycaemia, Lactate increase
- 0.05

++

++

Noradrenaline (1 > 2 and 1 >> 2 agonist) via cAMP ?

Dose
mcg/kg/min

Clinical effect

SVR, HR
() CO
SVR, HR, SV
0.10 0.20
++++
+++
+++
CO
Side effects: increasing myocardial oxygen requirement, can cause decrease in CO, Tachyphylaxis,
Hyperglycaemia
- 0.10

+++

++

+++

Dopamine (D1 and D2, higher doses: 1 >> 2 and 1 > 2 agonist) via cAMP. Precursor
of norepinephrine
Dose
mcg/kg/min
0.5 2
25

Clinical effect

increased splanchnic perfusion

HR, SV, CO
HR, SV, SVR
5 10
++
++
() CO
SVR
> 10
+++
CO
Side effects: increasing myocardial oxygen requirement, can cause decrease in CO, Tacharrhythmias,
Tachyphylaxis, Hyperglycaemia, immunsuppressive effect, inhibition of thyrotropin releasing hormone
++

b) Sympathomimetics: synthetic catecholamines

Dobutamine (1 >> 2)via cAMP
Dose
mcg/kg/min

Clinical effect

HR, SV, CO
() SVR
> 10
+++
HR, SV, CO
Side effects: increasing myocardial oxygen requirement, Tacharrhythmias, worsening diastolic function,
Tachyphylaxis, Hyperglycaemia
2.5 10

++

++

Isoprenaline ()via cAMP

Dose
1
2
1
2
Clinical effect
mcg/kg/min
0.01 - 1
+++
+
HR, SV, CO
Side effects: increasing myocardial oxygen requirement, Tacharrhythmias, worsening diastolic function,
Tachyphylaxis, Hyperglycaemia

c) Sympathomimetics: synthetic noncatecholamines

Phenylephrine (1 >> 2 agonist) resuscitation in Fallot spells
Dose
mcg/kg/min

Clinical effect

SVR
HR (reflex), CO
Side effects: increasing myocardial oxygen requirement, can cause decrease in CO, Tachyphylaxis,
Hyperglycaemia
0.1 5

+++

++

+++

d) Phosphodiestarase Inhibitors
Milrinone via cAMP
Dose
mcg/kg/min
Load: 50mcg/kg
0.2 1

Clinical effect

CO (positive inotropic and lusitropic effect)

PVR, (SVR)
Side effects: arrhythmia, hypotension (ensure appropriate volume load)

e) Myofilament calcium sensitizers

Levosimendan via increasing sensitivity to calcium
Dose
mcg/kg/min
Load: 1.25mcg/kg
over 10min
Infusion: 0.2

Clinical effect

CO (positive inotropic and lusitropic effect)

Side effects: arrhythmia, hypotension in the first hours

f) vasoregulatory agents
Vasopressin (V1 arterial and V2 tubular agonist)
Dose
V1 & V2
Clinical effect
IU/kg/hr
0.01 0.06
+++
SVR
Side effects: increasing myocardial oxygen req irement, can cause decrease in splanchnic perfusion
[1] Am Heart J 2002 Jan; 143(1) : 15-21: Hoffman TM et al: Prophylactic intravenous use of milrinone after cardiac operation in
pediatrics (PRIMACORP) study.
[2] Lancet 2002, 306: 196-202: Follath F et al: Efficacy and Safety of intravenous levosimendan compared with dobutamine in
severe low-output heart failure (the LIDO study); a randomised double-blind trial.
[3] Curr Opin Crit Care. 2010 Oct;16(5):432-41: Parissis et al: Inotropes in cardiac patients: update 2011
[4] Curr Opin Anaesthesiol. 2009 Aug;22(4):496-501: Salmenper et al: Levosimendan in perioperative and critical care
patients.
[5] Pediatr Cardiol. 2013, Jan34(1):1-29: Severin et al: The pediatric cardiology pharmacopoeia: 2013 update
[6] Pediatr Crit Care Med. 2006 Sep;7(5):445-8: Namachivayam P et al: Early experience with Levosimendan in children with
ventricular dysfunction.
[7] N Engl J Med. 2008 Feb 28;358(9):877-87: Russel et al: Vasopressin versus norepinephrine infusion in patients with septic
shock

ANESTHESIA: INOTROPES AND VASODILATORS

Vasodilators: decreasing the pressures against which the heart works (systemic and
pulmonary afterload) decreases the work of the heart hence myocardial O2 demand. Usual
indications for vasodilator therapy are: systemic vasodilation (LV afterload reduction),
pulmonary vasodilatation (RV afterload reduction), systemic hypertension, improving
coronary blood flow. Beware that infants, in response to low CO, increase afterload to
maintain BP. The use of vasodilators leads to increase in vascular capacitance and may
require volume replacement. Avoid or use judiciously with lesions where there is obstruction
to blood flow or fixed stroke volume.
Sodium-nitroprusside (SNP) via release of endogenous NO
Dose
mcg/kg/min

Clinical effect

Direct smooth muscle cell relaxation

arterial > venous vasodilation
Side effects: severe hypotension (titrate slowly), worsening V/Q mismatch, Cyanide and Thiocyanate
intoxication, Methaemoglobinemia, tachyphylaxis
0.2- 6

Glyceryl-trinitrate (GTN) via release of endogenous NO

Dose
mcg/kg/min

Clinical effect
Direct smooth muscle cell relaxation
venous > arterial vasodilation
improved coronary perfusion
Side effects: severe hypotension (titrate slowly)

1 - 10

Phenoxybenzamine via irreversible alpha-blockage

Dose
mcg/kg/min
Load: 1 mg/kg over 1hr
TDS or BD:
0.5mg/kg
Side effects: severe hypotension

Clinical effect
Vasodilation

Hydralazine via direct vasodilation by decreasing intracellular Ca++

Dose
mcg/kg/min

Clinical effect

10 - 50

Vasodilation
Side effects: reactive Tachycardia

Prostacyclin = PGI2 (Epoprostenol) via increase in NO

Dose
Clinical effect
ng/kg/min
2 20 (40)
Pulmonary vasodilation, treatment of PHT
Side effects: systemic hypotension, haemorrhagic diasthesis due to Platelet aggregation inhibition

Prostaglandine = Alprostadil = PGE1 via release of endogenous NO

Dose
ng/kg/min

Clinical effect

Pulmonary Vasodilation
Maintaining PDA patency
Side effects: systemic hypotension, fever, hypoventilation and apnea, antiplatelet function
5 - 100

Inhaled Nitric Oxide (iNO) @ NO and @ PHT

Sildenafil @ NO and @ PHT

Clonidine via presynaptic alpha 2 adrenergic action

Dose
mcg/kg/hr

Clinical effect

Vasodilation
Sedation
Analgesia
Side effects: systemic hypotension, avoid in Porphyria, may decrease CO
0.5 - 2

Dexmedetomidine via presynaptic alpha 2 adrenergic action

Dose
mcg/kg/hr
0.2 - 1

Clinical effect
Vasodilation
Bradycardia (can be used therapeutically)
Sedation
Analgesia
Side effects: systemic hypotension, decreases CO, avoid in LCOS

Captopril (ACE-I) via angiotensin converting enzyme inhibition

Dose
mcg/kg

Clinical effect

Vasodilation
Improve in CO
TDS or QID, increase dose by 0.1mg/kg until clinical effect achieved
Side effects: systemic hypotension, renal dysfunction
Test dose: 0.1

PICU: ARRHYTHMIA
prevalence of postoperative arrhythmia: 15 48%
at risk: young age, low body weight, long CPB time, complex surgery, presence of residual
defects
Prevalence of postoperative arrhythmia is up to 50%
Haemodynamic impairment in > 50%
Aggressive treatment in > 50% required
Most common is sinus bradycardia with / without junctional escape > premature complexes
> supraventricular tachycardia > AV block > JET
Mechanisms: Re-entry: on / off, inducible, overdriveable, cardiovertable; automatic /
ectopic: warm up, not inducible, not overdriveable, not cardiovertable
Prevention and unspecific treatment: strict maintenance of normothermia, avoid triggering
drugs, avoid volume overload, avoid acidosis, Mg++ >1.0, Ca++ >1.0, K+ 4.5 5mmol/L
Bradyarrhythmia
Sinusbradycardia: increased vagal tone, elevated ICP, drugs (Digoxin, -blocker,
Amiodarone, Dexmedetomidine,), respiratory (hypoxia), metabolic (Hypoglycaemia, Hyper
/ hypocalcaemia, Hypomagnesiaemia), post-surgical (Fontan Circulation, Mustard / Senning)
correction of underlying cause, Atropine 0.02mg/kg, Isoprenaline 0.1 2mcg/kg/min
infusion, Pacing: AAI, DDD, DDI @ Pacing
AV Block: congenital, increased vagal tone, drugs, respiratory, metabolic, post-surgical
(VSD, AVSD, ccTGA, TGA, Fontan Circulation) correction of underlying cause, Pacing:
VVI, DDD, DDI @ Pacing
Tachyarrhythmia
Sinustachycardia: six causes: central (pain, awake, fever, seizure), cardiovascular
(hypovolaemia, LCOS @ LCOS, PHT @ Pulmonary Hypertension), respiratory (hypoxia,
hypercarbia), heart failure correction of underlying cause, sedation, fluid bolus, general
prevention and treatment
Intraatrial Reentry Tachycardia (IART atypical atrial flutter): causes: post-surgical
(Fontan Circulation, Mustard / Senning, ccTGA, TOF, Ebsteins anomaly, VSD, ASD, TGA)
diagnostic: Adenosine (100mcg/kg iv, increasing up to 300mcg/kg iv), treatment:
overdrive pacing if rate low enough (@ Pacing), Cardioversion (1 J/kg), Amiodarone (loading
25mcg/kg/hr for 4 hours in Guardrail (set VTBI) followed by 5 15mcg/kg/min infusion for
rate control or Digoxin (20mcg/kg iv in infants, 30 - 40mcg/kg iv in children). Titrate for effect.
AV reciprocating tachycardia (WPW if preexcitation on baseline ECG). Treatment:
Adenosine (100mcg/kg iv, increasing to 300mcg/kg iv). Consider Overdrive pacing.
Consider Cardioversion 1J/kg. If recurrent/ongoing consider beta-blocker or Digoxin or
Amiodarone if concerned in regards to function.
Atrial Ectopic Tachycardia (AET chaotic atrial tachycardia) difficult to control
pharmacologically: -blocker: Esmolol (bolus up to 500mcg/kg iv followed by 100
1000mcg/kg/min infusion) or Propranolol (bolus 10 100mcg/kg slowly iv), Digoxin,
Procainamide, Flecainide (3 6mg/kg/day), Sotalol (2 6mg/kd/day), Amiodarone,
overdrive-pacing if rate low enough, catheter ablation. Consider sedation if compromised
cardiac output.
Atrial Fibrillation: preexcitation-syndromes, post-surgical (ASD, Fontan Circulation, AS)
Amiodarone, overdrive pacing, Cardioversion (1J/kg). Consider anticoagulation if
persistent > 48hrs
Junctional Ectopic Tachycardia (JET): 180 250bpm: congenital, post-surgical (ASD,
VSD, AVSD, TOF, Fontan Circulation) decrease adrenergic drugs if feasible, electrolyte
correction (Mg++and K+), sedation and paralysis, correct acidosis / alkalosis, correct
hypovolemia, correct hypoxemia / hypo or hypercarbia, overdrive pacing,

pharmacologically: Amiodarone, surface cooling to 35C to slow heart rate (and allow AV
sequential pacing)
Premature Ventricular Contraction (PVC): < 1 / min acceptable, otherwise treatment
of underlying cause. Beta-Blocker if clinically indicated.
Ventricular Tachycardia: respiratory, metabolic (inborn errors of metabolism), drugs
(Class I, Class III, Digitalis toxicity), anatomical (myocarditis), post-surgical, idiopathic in
unstable patient: immediate Cardioversion (1 J/kg 4 J/kg) and CPR, correction of
underlying cause, Amiodarone (loading over 20min: 5mg/kg iv) or Procainamide (loading
over 30min: 10 mg/kg iv), catheter ablation, ICD
Torsade de Pointes (polymorph VT): causes: TCA intoxication, long QT Syndrome,
dyselectrolytaemia, see also VT, MgSO4 (0.2mmol/kg), consider Beta-Blocker or pacing if
recurrent.
Ventricular Fibrillation: immediate DC and CPR Resuscitation
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Arrhythmia
[2] Am J Emerg Med. 2008 Mar;26(3):348-58: O'Connor et al: The pediatric electrocardiogram part II: Dysrhythmias
[3] Anaesth Intensive Care. 2009 Sep;37(5):705-19: Skippen et al: Diagnosis of postoperative arrhythmias following paediatric
cardiac surgery
[4] Nat Clin Pract Cardiovasc Med. 2008 Aug;5(8):469-76. Snyder: Postoperative ventricular tachycardia in patients with
congenital heart disease: diagnosis and management
[5] Pacing Clin Electrophysiol. 2008 Feb;31 Suppl 1:S2-6: Roos-Hesselink et al: Significance of postoperative arrhythmias in
congenital heart disease
[6] Circulation. 2007 Jun 26;115(25):3224-34: Walsh: Interventional electrophysiology in patients with congenital heart disease
[7] Circulation. 2007 Jan 30;115(4):534-45: Walsh et al: Arrhythmias in adult patients with congenital heart disease
[8] Z Kardiol. 2004 May;93(5):371-80: Haas et al: Postoperative junctional ectopic tachycardia (JET)
[9] Circ Arrhythm Electrophysiol. 2010 Apr 1;3(2):134-40: Chang et al: Amiodarone versus procainamide for the acute treatment
of recurrent supraventricular tachycardia in pediatric patients
[10] Pediatr Emerg Care. 2007 Mar;23(3):176-85; Manole MD: Emergency department management of the pediatric patient
with supraventricular tachycardia

PICU: FLUIDS
Anion Gap = Na+ + K+ - (Cl- + HCO3-). normal 8 12 mEq/l.
Total body water (TBW) = intracellular fluid (ICF) plus extracellular fluid (ECF). [Weight x
600 ml in adults (500 ml in female), Weight x 650 ml in paeds, Weight x 700 ml in neonates]
ECF = intravascular fluid (plasma and lymph in the vessels) plus interstitial fluid (between
cells)
Osmolality = 2 x Na+ + K+ + Glucose (mmol/l) + Urea (mmol/l).
Osmotic Gap = measured Osmolality calculated Osmolality
Na+ deficit [mmol/l] = (Na+Target Na+Current) x TBW / 1000
Cl- deficit [mmol] = (Cl-Target Cl-Current) x 0.2 x Weight
Water deficit = 4 ml x Weight x (Na+Target Na+Current)
Maximum change in Osmolality in hyper- or hypoosmolaric: 1 mosmol/l per hour.
Cave central pontine Myelinolysis !
Body water and Blood volume composition with age
Adult bodies are 60% water (20% ECF, 40% ICF). Blood volume 70 ml/kg. Term neonate
bodies are 75% water (40% ECF, 35% ICF), and term neonates usually lose 5-10% of their
weight in the first week of life, almost all of which is water loss. Blood volume 80 ml/kg.
Preterm neonates have more water (at 23 weeks' gestation, 90% water composed of 60%
ECF and 30% ICF), and they may lose 10-15% of their weight in the first week of life. Small
for gestational age (SGA) preterm infants may have a higher proportional body water content
(90% for SGA infants vs 84% for appropriate for gestational age [AGA] infants at 25-30
weeks gestation).
10 kg:
4 ml/kg/hr

Maintenance Fluid [ml/hr] for active Children > 2 days

11 kg & < 20 kg
40 ml/hr +
2 ml/kg/hr

21 kg
60 ml/hr +
1 ml/kg/hr

Day 1

Day 2
Day 3
Maintenance Fluid [ml/hr] active Neonates 3 days
2 ml/kg/hr
3 ml/kg/hr
4 ml/kg/hr
+
Na Requirements for active Neonates 3 days
1 3 mmol/kg/d
3 5 mmolk/kg/d
2 4 mmol/kg/d
+
K Requirements for active Neonates 3 days
1 2 mmol/kg/d
2 3 mmol/kg/d
1 2 mmol/kg/d
Normal maintenance fluid:
NaCl 0.9 % or NaCl 0.9 % in 5 % Dextrose or NaCl 0.9 % in 2.5 % Dextrose or Ringer Lactate or Hartmann
Solution
[1] Pediatrics, 1957, May;19(5):823-32: Holliday at all: The maintenance need for water in parenteral fluid therapy.
[2] Kidney Int., 2005, Jan;67(1):380-8: Friedman: Pediatric hydration therapy: historical review and a new approach.
[3] Pediatr Nephrol. 2005, Dec;20(12):1687-700: Moritz ML at all: Preventing neurological complications from dysnatremias in
children.
[4] Arch Dis Child, 2006, 91(3):226-32: Neville at all: Isotonic is better than hypotonic saline for intravenous rehydration of
children with gastroenteritis: a prospective randomised study.
[5] N Engl J Med 2011;364:2483-95: Maitland et al: Mortality after Fluid Bolus in African Children with Severe Infection

PICU: MAINTENANCE FLUIDS

Maintenance Fluid [ml/hr] requirements for 10 kg
Wt / kg

Active
Inactive
Fever
Hypothermia
30 % post OP Cardiac
Day 1
50 % post OP Cardiac
Day 2

12
6

20
28
36
10
14
18
o
add 10 % for every 1 Celsius
o
deduct 12 % for every 1 Celsius

10
40
20

12

13

10

14

18

20

add 4 % for every 1 % burnt (day 1)

add 2 % for every 1 % brunt (day 2)

Burns

Maintenance Fluid [ml/hr] requirements for 11 kg & < 20 kg

Wt / kg

11

13

15

17

20

Active
Inactive
Fever
Hypothermia
30 % post OP Cardiac
Day 1
50 % post OP Cardiac
Day 2

42
21

46
50
54
23
25
27
o
add 10 % for every 1 Celsius
o
deduct 12 % for every 1 Celsius

60
30

14

15

17

18

20

21

23

25

27

30

add 4 % for every 1 % burnt (day 1)

add 2 % for every 1 % brunt (day 2)

Burns

Maintenance Fluid [ml/hr] requirements for 21 kg

Wt / kg

21

30

50

60

Active
Inactive
Fever
Hypothermia
30 % post OP Cardiac
Day 1
50 % post OP Cardiac
Day 2

61
30

70
80
90
35
40
45
o
add 10 % for every 1 Celsius
o
deduct 12 % for every 1 Celsius

100
50

20

23

27

30

33

30

35

40

45

50

Burns

40

add 4 % for every 1 % burnt (day 1)

add 2 % for every 1 % burnt (day 2)

PICU: NUTRITION
Facts:
1. Nutritional support for children in PICU is important to prevent deficiency and remain in a
positive or neutral neutrogen balance
2. Overfeeding is associated with adverse effects
3. There is no clear evidence supporting PN over TPN
4. If the bowel works, use it !
5. Always slowly introduce lipids !
5. Cautiously introduce enteral feeding where there is low CO !
premature
0 < age < 1
1 < age < 7
7 < age < 12
12 18

Energy requirements in healthy children

110 120 kcal/kg/d
90 100 kcal/kg/d
75 90 kcal/kg/d
60 75 kcal/kg/d
30 60 kcal/kg/d

Day

Dextrose

1
2
3
4
5
6

48
48
5 10
5 12
6 15
7 16

Age
< 1 mo
< 6 mo
<1y
<7y
< 12 y
> 12 y

Feeding the healthy neonate

Protein
g / kg /d
1 ( - 3)
1 ( - 3)
1 ( - 3)
2 ( - 3)
2.5 ( - 3)
2.5 ( - 3)

Feeding the healthy child

Dextrose
Protein
g / kg /d
7 16
2.7
7 16
1.5
7 16
1 1.5
5 15
0.9
5 15
0.9
5 10
0.9

Lipids
1
2
3
3
3
3

Lipids
13
13
13
12
12
1-2

1 Kcal = 1 Calorie = 1000 cal = 4184 joules

1 g Dextrose = 3.8 Kcal (1 g hydrolysed Dextrose = 3.2 Kcal !)
1 g Protein = 4 Kcal
1 g Lipid = 9 Kcal
[1] Intensive Care Med, 2004, 30(9), 1807-13: van der Kuip M: Nutritional support in 111 pediatric intensive care units: a
European survey
[2] Cochrane Database Syst Rev. 2009, 15;(2):Joffe et all: Nutritional support for critically ill children
[3] Ger Med Sci, 2009 Nov 18;7, Doc15: Fusch et all: Neonatology/Paediatrics - Guidelines on Parenteral Nutrition, Chapter 13
[4] J Parenter Enteral Nutr. 2009 May-Jun;33(3):260-76: A.S.P.E.N. Clinical Guidelines: Mehta et al: nutrition support of the
critically ill child
[5] J Parenter Enteral Nutr. 2010 May-Jun;34(3):247-53: Jaksic et al: A.S.P.E.N. Clinical guidelines: nutrition support of
neonates supported with extracorporeal membrane oxygenation JPEN

Table: Nutrition in EBM / Formula

kCal
Prot (g)
/ml
/ 100ml
INFANT FEEDS 0 12 MONTHS or < 8kg
Human Milk/ EBM
0.69
1.0
Human Milk + 2 blue scoops Karicare
0.83
1.3
Karicare Gold Plus 1 strength
0.83
1.7
Infatrini FS
1.0
2.6
Pepti-junior Gold FS (partially hydrolysed)
0.67
1.8
Pepti-junior Gold 1 strength
0.84
2.25
Neocate LCP FS (extensively hydrolysed)
0.70
1.9
Neocate LCP 1 strength
0.87
2.4
Monogen FS (MCT based feed
0.75
2.0
chylothorax)
Monogen 1 Strength
0.93
2.5
Kindergen FS (renal)
1.0
1.5
Kindergen 1 Strength
1.25
1.9
Neocate advance FS (>12months)
1.0
2.9
PAEDIATRIC FORMULAE 1-6 YEARS
* Nutrini Multi-fibre
1.0
2.8
(0.8g fibre per 100ml)
Nutrini
1.0
2.8
Nutrini Energy
1.5
4.1
ADULT FORMULAE >12 YEARS or 45kg
* Nutrison multi-fibre (1.5g per 100ml)
1.0
4.0
Nutrison Standard
1.0
4.0
Nutrison Energy
1.5
6.0
Nutrison Energy multi-fibre (1.5g per
1.5
6.0
100ml)
ADDITIVES (per 100ml) per gram or ml
Duocal 1x Blue Scoop (1.2g)
5.9
0
Polyjoule 1x Blue Scoop (1.2g)
4.6
0
Protifar 1x Blue Scoop (0.85g)
3.1
0.75
Per ml MCT oil
8.4
Karicare Gold 1x Blue Scoop
6.9
0.15
Formula / Additive

Fat (g)
/ 100ml

CHO (g)
/ 100ml

4.3
4.9
4.4
5.4
3.6
4.5
3.4
4.2

7.2
8.8
9.1
10.3
6.9
8.6
7.9
9.9

2.1

12.0

2.6
5.3
6.6
5.1

15
11.8
14.8
10.5

4.4

12.3

4.4
6.7

12.3
18.5

3.9
3.9
5.8

12.3
12.3
18.5

5.8

18.5

0.27
0
0.014
0.95
0.37

0.87
1.1
0.013
0.72

PICU: NUTRITION / FEEDING GUIDELINE

1. Decide on enteral or parenteral nutrition
Absolute contraindications for enteral feeding:
Escalating vasoactive/inotropic support
Haemodynamic instability with ongoing volume resuscitation
Suspected or confirmed NEC
Mechanical bowel obstruction
Significant GI bleed
Ischemic bowel
Complex GI surgery (discuss with surgeons)
Central cooling
Relative contraindications for enteral feeding:
< 24hrs after cardiac arrest
Duct dependent lesion
2. Is the patient able to feed orally ?
Yes encourage enteral feeds on demand
No continue Question 3
3. Is the patient able to feed enterally ?
No consider PN proceed to Step 9
Yes continue Question 4
4. Is there ANY risk factor of aspiration ?
Previous history of aspiration
Altered intestinal motility
Delayed gastric emptying
Witnessed regurgitation/aspiration
Significant gastroesophageal reflux
Altered mental status with depressed gag reflex
Persistent vomiting (or more episodes/day)
No consider commencing continuos NG feeds
Yes consider commencing continuos NJ feeds
4. Is the patient ready to advance to full enteral feeds ?
No Consider Trophic feeds: 0.5mL/kg/hr (max 20mL/hr). Discuss eligibility for
advancing feeds on daily rounds, and if unable after 5 days, consider starting PN
Yes Start feeds 1mL/kg/hr or 25mL/hr (max). If malnourished, NBM>1 weeks or
concerns for poor gut perfusion consider starting feeds at 0.5mL/kg/hr as at risk of
refeeding syndrome.
5. Measure gastric residual volume and assess for signs of intolerance after 4 hours
Signs and symptons of enteral feeding intolerance
Vomiting 2 or more episodes/24hrs
Abdominal discomfort
Abdominal distention 2 consecutive increases of abdominal girth in 24hrs
Diarrhoea 3 or more episodes of loose stool in 24hrs
Note: The absence of bowel sounds is NOT an indicator of feeding intolerance

6. Does the patient have any enteral feeding intolerance or more than > 5mL/kg
gastric residual volume ?
No advance feeds 4hrly by extra 1mL/kg/hr or 25mL/hr (max), if feeds stopped
for intolerance restart at tolerance rate continue with Step 6 until full enteral
nutrition reached
Yes hold feeds at current rate replace gastric residual volume up to 5mL/kg
(max. 150mL) unless contraindicated reassess after 1hour for feeding intolerance:
Does the patient have any enteral feeding intolerance or more than >
5mL/kg gastric residual volume ?
No advance feeds 4hrly by extra 1mL/kg/hr or 25mL/hr (max), if
feeds stopped for intolerance restart at tolerance rate continue
with Step 6 until full enteral nutrition reached
Yes stop feeds for 4hours then reassess
7. Has the patient met enteral nutrition goals by Day 2 ?
No

consider
Promotility agent (Metoclopramide, or Erythromycin)
Post pyloric feeds (if Gastric fed)
If PN is indicated

Yes proceed to Step 8

8. Enteral Nutrition Maintenance
Volume goal reached:
Review energy and protein adequacy on daily rounds
Consider increasing density of formula if required
Continue to monitor for signs of intolerance routine measurement of GRV not
indicated unless other signs and symptoms of intolerance are present
Monitor weight weekly (minimum)
Consider Indirect Calorimetry for resting energy expenditure
Avoid unnecessary and prolonged interruptions to EN
Monitor for signs of overfeeding (excessive weight gain, new ventilator dependence,
hyperglycemia, hypertriglyceridemia)
Monitor BSL
Interruptions to feed:
Always aim to meet the energy goal for the day, after periods of fasting you may
need to increase the feed rate to ensure that the child gets the full feed volume for
the day
Re-start feeds at the same rate as prior to interruption
9. Parenteral Nutrition
Indications for PN
When enteral nutrition is not possible
o > 2days in a neonate
o > 3days in infants
o > 5days in an older child
Weight loss of >10% with a poorly functioning GI tract
Anticipated need for PN support for (minimum) 3-5 days

Complications of PN therapy:
Hyper and Hypoglycaemia
Electrolyte and acid/base disturbance
Fluid imbalance
Hypertriglyceridaemia
Re-feeding syndrome
Line related complications
Liver disease
Monitoring:
Baseline : U&E, Ca, Mg, PO4, BSL, TG, VBG, LFT, FBC
Daily while grading up: U&E, Ca, Mg, PO4, BSL, TG
Ongoing: Weekly U&E, Ca, Mg, PO4, BSL, TG, VBG, LFT and Urine Na, K and Cl

Day

Dextrose

1
2
3
4
5
6

48
48
5 10
5 12
6 15
7 16

Age
< 1 mo
< 6 mo
<1y
<7y
< 12 y
> 12 y

Feeding the healthy neonate

Protein
g / kg /d
1 ( - 3)
1 ( - 3)
1 ( - 3)
2 ( - 3)
2.5 ( - 3)
2.5 ( - 3)

Feeding the healthy child

Dextrose
Protein
g / kg /d
7 16
2.7
7 16
1.5
7 16
1 1.5
5 15
0.9
5 15
0.9
5 10
0.9

Lipids
1
2
3
3
3
3

Lipids
13
13
13
12
12
1-2

10. Refeeding Syndrome

Patients at risk - after prolonged fasting/ poor intake (> 7days), malnutrition, recent
weight loss >5-10%BW, patients presenting with low K, PO4, Mg
Manifests as hypophosphataemia, hypomagnesaemia and hypokalaemia
Observe for arrhythmia and weakness
Monitor bloods baseline levels, then 6 hourly for 24-48 hours (K, PO4, Mg), and 12
hourly until stable. Replace as required.
Consider Dietitian Referral

PICU: BLOOD PRODUCTS

Pump Blood
Pump blood is the blood remaining in the bypass circuit on the completion of bypass. It is a
mixture of the patients own blood, other fluids and any bank blood used to prime the bypass
circuit.
Unfiltered pump blood has a low Hct and contains large amounts of heparin and
inflammatory cytokines. The use of CUF (continuous ultrafiltration) during bypass or MUF
(modified ultrafiltration) after bypass concentrates the pump blood and removes some
heparin and cytokines. If pump blood is used, additional Heparin reversal will be needed. It
should not be used if excessive bleeding or if the Hb is low.
Protamine sulphate is used to reverse the heparin in pump blood. The dose is 1 mg per 25ml
of pump blood. Recheck ACT after 10ml/kg pump blood. Rapid infusion may cause
pulmonary hypertension.
Packed Red Blood Cells (PRBC)
Each unit of pack cells contains ~ 300ml and has an Hct of 0.5 0.7 (Na+ ~ 20, K+ up to
20mmol/l, especially if irradiated)
Warning: Neonates should be transfused with blood which is as fresh as possible, and
sufficiently slowly to minimise any adverse effect from hyperkalaemia and citrate toxicity
(hypocalcaemia).
Used for treatment of anaemia and the management of active bleeding. Must be compatible
with recipients ABO and Rh groups and clinically significant red cell antibodies.
Transfusion of 4ml/Kg increases Hb by approximately 1g/dl. In rapid transfusion situations
alternate red cell units with colloid solutions eg. FFP.
Store only in a designated blood refrigerator (2 to 6C). Use within 4 hours of removing from
refrigerator and always use a leucocyte filter.
Request irradiated products if suspicion of immunodeficiency (eg Di George anomaly)
or in any neonate <1month undergoing cardiac surgery.
Donor blood exposes the patient to risk of infection and transfusion reaction. Pump blood,
however is blood to which the patient has already been exposed.
Platelets
Cardiopulmonary bypass frequently leads to both thrombocytopenia (dilutional) and more
importantly platelet dysfunction (early onset). Platelet transfusion should be considered for
excessive bleeding, irrespective of the absolute platelet count. Transfused platelets have a
storage (function) defect lasting 2 - 4hrs.
Platelet transfusion should not be used for routine volume expansion. Should be ABO
compatible to prevent haemolysis caused by donor anti-A and anti-B. Female infants and
children (all females <45 years) should receive RhD negative platelets. The dose is 10ml/kg
repeat platelet count and/or TEG.
Fresh Frozen Plasma
Plasma separated from one donation of blood. Contains normal
levels of stable clotting factors, albumin and immunoglobulin. Factor VIII levels are ~70%
normal while plasma proteins (immunoglobulins and clotting factors) are slightly diluted
. It should be ABO compatible to prevent haemolysis by donor anti-A or anti-B.
Should be used for microvascular bleeding following massive transfusion or cardiopulmonary
bypass, emergency reversal of warfarin effect (in addition to Vitamin K), bleeding resulting
from hepatic failure and proven coagulopathy (factor deficiency or DIC).
Loss of clotting factors may occur as a result of excessive loss of peritoneal, pleural fluid or
ascites (via PD catheter). If replaced with NaCl 0.9% alone this may lead to a dilutional
coagulopathy.
Dose is 10 20ml/kg iv. FFP should ideally administered slowly (<40ml/kg/hr) as rapid
administration can result in cardiovascular collapse by several mechanisms including

calcium chelation by citrate (check patient iCa if concerned).Infection risk similar to other
blood components. Transfusion should not be used for routine volume expansion.
Cryoprecipitate
The cold precipitated fraction derived from FFP. Contains factor VIII, fibrinogen, von
Willebrand factor and factor XIII.
Should be used for significant fibrinogen deficiency associated with clinical bleeding, DIC,
trauma or during invasive procedures. Suitable for haemophilia and von Willebrand disease
specific factors are unavailable. Dose is 5ml/kg iv. One bag is usually 20 30ml. Infection
risk is similar to other blood components.
Human Albumin Solutions
Albumex 4%

Albumex 20%

Protein 40 g/l

Protein 200 g/l

Na 140mmol/l

Na 48 - 100mmol/l

Volume expansion

Hypoproteinaemia

50, 250, 500ml bottle

10, 100ml bottle

5 - 10ml/kg aliquots

5ml/kg aliquots

Albumex 20% is hyperoncotic and in an ideal situation (ie. normal capillary permeability)
should expand circulating volume by a factor of 5.
[1] Cochrane Database Syst Rev. 2013 Feb 28;2. Perel et al: Colloids versus crystalloids for fluid resuscitation in critically ill
patients.
[2] SAFE Study Investigators, Finfer et al: Effect of baseline serum albumin concentration on outcome of resuscitation with
albumin or saline in patients in intensive care units: analysis of data from the saline versus albumin fluid evaluation (SAFE)
study.
[3] Pediatr Crit Care Med. 2007 Sep;8(5):459-64: Jatana et al: Deletion 22q11.2 syndrome--implications for the intensive care
physician.
[4] Peditar Crit Care Med 2011 Vol.12, No2: Isthaphanous: Red blood cell transfusion in critically ill children: A narrative review

PICU: ANTICOAGULATION & THROMBOLYSIS

Unfractionated Heparin (UFH)
Indications: Low dose heparin infusions are used in the maintenance of central venous
lines, arterial lines and the prevention and treatment of deep vein thromboses. This protocol
may need to be altered according to individual patient requirements.
Administration: Heparin can be administered by intravenous and subcutaneous routes.
This protocol applies to the intravenous route only. Heparin is compatible with 5% Dextrose,
0.9% NaCl and 0.45% NaCl.
Obtain patient weight and baseline FBC, aPTT, INR
For maintenance of central line in infants less than 5kg commence 10U/kg/hr
For Shunt prophylaxis in any patient commence 10U/kg/hr once no major postoperative
bleeding.
For antithrombotic treatment commence see Standard Therapeutic IV UFH Protocol
The need for monitoring will be individualised for each patient. In general it is
recommended an aPTT be obtained at 24hours, and some stable patients may require aPTT
only every 2 - 3 days.
Twice weekly FBC must be obtained to monitor for heparin induced thrombocytopaenia
(consider HIT ELISA screen)
Coagulation studies required for other reasons should not be obtained from a line
containing heparin.
Adverse Events: Bleeding whilst on low dose heparin is uncommon, but can occur. If
bleeding occurs, cease heparin infusion. Check FBC, clotting and TEG. Consider seeking
Haematology consult. Antidote: Protamine
Precautions: In patients with renal failure this low-dose heparin infusion may result in
therapeutic anticoagulation.
Age
Loading
Maintenance

Standard Therapeutic IV UFH Protocol

< 1year
> 1year
75U/kg
75U/kg
25U/kg/hr
20U/kg/hr

Adult
5000U
1500U/hr

Obtain venous blood sample for aPTT 4hours post completion of loading infusion (NOT
earlier). Adjust heparin infusion rate to maintain aPTT 60 85s the baseline aPTT or within
the range determined as optimal for that patient.
aPTT (sec)
< 50
50 59
60 85
86 95
96 120
>120

Normogram for adjusting UFH IV Dose

Bolus (U/kg)
Hold (min)
Rate Change (U/hr)
50
0
+ 20%
0
0
+ 10%
0
0
No change
0
0
- 10%
0
30
- 10%
0
60
-15%

repeat aPTT
4hrs
4hrs
24hrs
4hrs
4hrs
4hrs

Monitoring of Therapy: Heparin is usually monitored by aPTT. However, this may be

inaccurate in certain clinical circumstances. An alternative is an anti-Xa assay.
Heparin Antidote: If anticoagulation with heparin needs to be discontinued for clinical
reasons, termination of the heparin infusion will usually suffice. If an immediate effect is
required, consider administering protamine sulfate. Protamine is a medication that requires a
high level of caution when being prescribed and administered. Outside cardiac surgery and

ICU, consultant or fellow approval is required for the use of protamine - do not allow this to
lead to delayed administration in the case of bleeding. Contact the appropriate senior person
immediately.
Protamine sulfate neutralises heparin by virtue of its positive charge. Following IV
administration, neutralisation occurs within 5 minutes. The maximum dose of protamine
sulfate, regardless of the amount of heparin received is 50mg except for reversal of heparin
following cardiopulmonary bypass. Protamine sulfate is usually administered in a
concentration of 10mg/ml at a rate not to exceed 5mg/minute. If administered too quickly,
protamine sulfate may cause cardiovascular collapse (severe pulmonary hypertension).
Patients with known hypersensitivity reactions to fish, and those who have received
protamine-containing insulin or previous protamine therapy may be at risk of hypersensitivity
reactions to protamine sulfate.
Obtain blood for PT and aPTT 15min after the administration of protamine sulfate.
The dose of protamine sulfate is based on the amount of heparin received in the previous
2hrs as follows:
Time since last
Heparin dose
< 30min
30 60min
60 120min
> 120min

Protamine dose (mg) per 100U Heparin received

1mg
0.5 0.75mg
0.375 0.5mg
0.25 0.375mg

Low molecular weight Heparin (LMWH)

Indications: Low Molecular Weight Heparins are used for the prophylaxis or treatment of
deep vein thrombosis. The decision to use LMWH instead of standard heparin (or warfarin)
will depend upon the clinical scenario and individual patient factors such as risk of bleeding
or availability of venous access.
The following are guidelines only and may need to be adapted in individual circumstances.
Administration:
Obtain patient weight and baseline FBC, aPTT, PT.
Dose as follows, administering via subcutaneous route, either via an insuflon catheter, or
by rotating sites of subcutaneous injections.
Timing of commencement of therapy (especially post-procedural) should be individualised.
Duration of therapy is determined on an individualised basis, based up on indication for
treatment.
Age
Treatment
Prophylaxis
Treatment
Prophylaxis

LMWH (Enoxaparin) in infants and children

< 2mths
2mth 18yrs
1.5mg/kg/dose BD
1mg/kg/dose BD
0.75mg/kg/dose BD
0.5mg/kg/dose BD
LMWH (Dalteparin) in adults (100U = 1mg)
100U/kg/dose BD
2500 5000U OD

Anti-Xa level (U/ml)

Normogram for LMWH therapy

? Hold next dose
Dose change

< 0.35

No

+ 25%

0.35 0.49

No

+ 10%

0.5 1.0

No

No change

1.1 1.5

No

- 20%

1.6 2.0

3hrs

- 30%

> 2.0

Until Anti-Xa <

5U/ml

- 40%

? repeat Anti-Xa level

4hrs post next dose
4hrs post next am dose
Once per week / 4hrs post am
dose
4hrs post next am dose
Trough level pre next dose, then
4hrs post next am dose
Trough level pre next dose and if
not <0.5U/ml repeat BD

Adverse Events: The major adverse event related to treatment with LMWH is bleeding. If a
patient on LMWH develops a major bleed, withhold further doses and seek an urgent
Haematology consult. HIT is rare in LMWH treatment, but consider if rapid fall in platelet
count. Antidote: Protamine
Precautions: In patients with renal failure this low-dose heparin infusion may result in
therapeutic anticoagulation. It is recommended that prior to any surgery or spinal or epidural
procedure, 2 doses of LMWH be omitted. Haematology consult to advise on management
around such procedures is advised.
Heparin Antidote: If anticoagulation with LMWH needs to be discontinued for clinical
reasons, termination of the heparin infusion will usually suffice. If an immediate effect is
required, consider administering protamine sulfate. Protamine is a medication that requires a
high level of caution when being prescribed and administered. Outside cardiac surgery and
ICU, consultant or fellow approval is required for the use of protamine - do not allow this to
lead to delayed administration in the case of bleeding. Contact the appropriate senior person
immediately.
Protamine sulfate neutralises heparin by virtue of its positive charge. If protamine is given
within 8hrs of the LMWH then a maximum neutralizing dose is 1mg Protamine/1mg (or
100U)) of LMWH given in the last dose. If more than 8hours have passed since the dose of
LMWH was given, administer 0.5mg Protamine per 1mg (or 100U) of LMWH given.
Protamine is administered by slow IV infusion (over 10 mins) to avoid a hypotensive
reaction.
Aspirin
Aspirin is a medication only available for oral administration (in Australia). Tablets are
available in enteric and non-enteric coating. Dispersible tablets are also available. For
infants and small children it may be necessary to either crush tablets or use a dispersible
tablet and administer the aspirin in liquid form. These guidelines are for the use of aspirin for
its antiplatelet activity.
Indications: Aspirin is commonly used in patients with cardiac disease and those with a
history of arterial stroke. There are also certain indications for the use of Aspirin in
pregnancy. It is more commonly used in patients with or at risk of arterial thrombosis. There
is no data to support the use of aspirin in the treatment / prevention of venous
thromboembolism.
Administration and Maintenance:
Aspirin is commenced only when patients are permitted oral
Commence 3 - 5mg/kg/day to a maximum of 100mg
Continue aspirin therapy as clinically indicated. For primary and secondary prophylaxis at
least 3months therapy is recommended.

 Post Norwood Procedure or in Patients with Shunt. Prophylaxis is required until surgical
correction.
Therapeutic monitoring is not required !
Precautions: A significant association between Reyes Syndrome and the ingestion of
aspirin by children with influenza-like illness or chicken pox has been reported in the
literature. Parents should be educated regarding the risk of developing Reyes Syndrome
secondary to aspirin therapy. It should be clearly explained that aspirin therapy must be
stopped in the presence of fever and/or chicken pox or measles. Paracetamol is permitted in
this scenario. The concurrent use of non-steroidal anti-inflammatory medications and Aspirin
is not recommended.
Mechanism: irreversible platelet inactivation. Once therapeutic doses are taken, antiplatelet
effect remains for the lifespan of the platelet population which is 7 - 10days. Patients
scheduled to undergo surgical procedures should in general, stop aspirin 7 - 10days prior to
surgery. Perioperative Aspirin therapy may increase the risk of perioperative bleeding. The
timing of cessation of aspirin therapy is the decision of the primary physician.
Adverse Effects: Patients on aspirin therapy are at a slightly increased risk of bleeding and
bruising. Usually this is not significant. If a patient develops significant bleeding or bruising
whilst on aspirin, prompt referral to a haematologist is required.
Clopidogrel
Clopidogrel is a theinopyridine derivate, that produces its antiplatelet effect through an active
metabolite, which irreversibly modifies the ADP purinergic P2Y12 platelet receptor.
Indications: Clopidogrel is widely used in adult cardiac and cardiovascular ischemic disease
(MATCH Trial), however the use in children is based on single center experience or safety
trials (PICOLO Trial)
Administration and Maintenance:
Clopidogrel is commenced only when patients are permitted oral
Commence 0.2mg/kg/day
Continue aspirin therapy as clinically indicated. For primary and secondary prophylaxis at
least 3months therapy is recommended.
Post Norwood Procedure or in Patients with Shunt after surgical consultation. Prophylaxis
is required until surgical correction, however latest results show no reduction in morbidity or
mortality in systemic-pulmonary-artery shunts
Adverse Effects: Patients on Clopidogrel therapy are at a slightly increased risk of bleeding
and bruising. Usually this is not significant. If a patient develops significant bleeding or
bruising whilst on Clopidogrel, prompt referral to a haematologist is required.

THROMBOLYSIS with r-TPA (ALTEPLASE)

These guidelines are for systemic thrombolytic therapy. There is no data to support the use
of local thrombolytic therapy in infants and children except for line blockages.
Indications: Massive pulmonary embolism / Pulmonary embolism not responding to heparin
/ Arterial occlusions / Potential for acute, extensive DVT threatening organ or limb viability.
Contraindications: Active bleeding / Significant potential for serious local bleeding /
General surgery within the previous 2 days / Neurosurgery within the previous 3 weeks / AV
malformations / Recent severe trauma.
Preparation for infusion:
Obtain patient weight, FBE, INR/PT, aPTT and Fibrinogen. Platelet count must be >100.
Fibrinogen must be > 2.0. Administer FFP infusion 20ml/kg (plus Frusemide) if clinically
indicated, especially in neonates < 1month (low plasminogen levels). Consider appropriate
staffing requirements are in place to monitor infusion.
Ensure adequate venous access to: infuse thrombolytic therapy and obtain blood
specimens during infusion.
Establish heparin infusion of 10U/kg/hour to be administered continuously throughout
thrombolytic infusion. If possible heparin should be administered for 6 hours prior to lysis as
this may be advantageous for thrombolytic action.
Premedicate with paracetamol and/or promethazine due to potential for allergic reactions.
Administration:
FFP
Heparin
r-TPA

Loading
20ml/kg in neonates
< 1month
Preferably commence 10U/kg/hr,
6hrs prior r-TPA
No

Infusion

10U/kg/hr during r-TPA treatment

0.5mg/kg/hr
for 6hrs

Monitoring:
HR and BP hourly.
All puncture sites hourly during infusion and for 4hours post infusion.
check Fibrinogen at 3hours into infusion and at completion.
if any signs of bleeding and / or bruising occur cease infusion, check FBC, clotting and
TEG and seek urgent Haematology consult !
If treating a peripheral artery thrombosis, observe limb hourly for pulse, colour, temperature
and capillary return.
After r-TPA: cease lytic therapy at 6 hours and increase heparin to 20U/kg/hour aiming for
aPTT 60 85s (no bolus). Arrange clinical review (eg Doppler-Ultrasound) to determine
response or need for further thrombolysis.
Complications:
In 30 - 50% of patients a bleeding event will occur. This is usually in the form of oozing from
a wound or puncture site. Treatment with local pressure is usually sufficient. Major bleeding
(intracranial, retroperitoeal, external) can develop in up to 10% of patients. If bleeding
occurs, cease infusion and seek an urgent Haematology consult.
Precautions
No IM injections during thrombolytic therapy.
Minimize patient handling during infusion.
Avoid concurrent use of warfarin and antiplatelet agents.
Delay any invasive procedures such as urinary catheterization, re-siting venous/arterial
access, or perform such procedures pre-thrombolytic infusion.

Blocked CVL Line

Indications: CVLs that will not infuse properly or CVLs that will not allow for the withdrawal
of blood samples when this is an essential function of that line. (eg. Haemodialysis, oncology
patients).
Initial Management: Obtain CXR to confirm line placement and absence of kinking.
Ultrasound to rule out major vessel thrombosis.
Initial Action if Blood Related Blockage:
If unable to draw blood sample, unable to infuse blood or there is blood back-up in infusion
line;
Attempt to aspirate.
Flush with 0.9% N/saline.
If unsuccessful flush with strong heparin solution (100U/ml) to a maximum of 5ml.
Give r-tPA in each obstructed lumen: <10kg (0.5mg r-TPA each lumen) or >10kg (2.0mg rTPA each lumen) and leave for 2 4hrs. Try to withdraw thereafter and flush with NaCl
0.9%.
If able to flush line but no blood return, arrange diagnostic imaging as clinically indicated.
If unable to flush line obtain surgical consult or consider venography and / or ultrasound
[1] Chest. 2012 Feb;141(2 Suppl):e737S-801S. Monagle et al: Antithrombotic therapy in neonates and children: Antithrombotic
Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice
Guidelines.
[2] CIRCULATION 2008, 117:553-559: Li et al: Dosing of Clopidogrel for Platelet Inhibition in Infants and Young Children:
Primary Results of the Platelet Inhibition in Childen On cLOpridogrel (PICOLO) Trial
[3] N Engl J Med. 2013 Jun 20;368(25):2377-84. Wessel et al: Clopidogrel in infants with systemic-to-pulmonary-artery shunts.

PICU: CHEST DRAINS

If losses > 10ml/kg/hr in a postoperative patient, notify surgeon immediately !
In the first two hours, losses may be up to 5ml/kg/hr, thereafter it should be less than
2ml/kg/hr. If losses exceeds these levels, check ACT, aPTT, PT, Fibrinogen, Platelets and
TEG, and transfuse accordingly.
If significant losses continue, notify surgeons immediately !
Insertion of Chest Drains
If surgical patient offer procedure to surgeons first !
Preparation and Equipment:
1. THAL-Quick Chest Tube Set and Tray
2. choose appropriate size
3. CXR before procedure
4. identify insertion site via ultrasound (ensure distance from liver, kidneys, spleen or heart)
5. prepare chest tube insertion site with antiseptic solution and sterile drapes in standard
fashion (sterile gown, gloves, head and face mask)
6. consider local anaesthetic (ie Lignocaine; remember: 1% = 10mg/ml, maximum
Lignocaine dose 5mg/kg without adrenaline).
7. attach introducer needle to syringe and advance slowly and carefully needle over the
superior border of the rib into the pleural space. Fluid or air should be aspirated to verify
intrapleural position.
8. When the appropriate drainage site and depth has been identified, de-attach syringe and
slowly introduce the J-tip of the guidewire: the guidewire should pass through and into
the pleural space without any resistance !
9. remove the needle, but leave wire in situ.
10. while maintaining the wire position, dilate the tract by supplied dilator (hold dilator always
at the tip, next to the skin, rotate it carefully to prevent the wire from kinking)
11. remove dilator, the guidewire in situ, advance slowly the chest tube into the pleural
space (if any resistance, ensure the guidewire is still in situ, re-dilate skin / pleural opening, if
necessary)
12. remove guidewire, leaving the chest tube in situ
13. use sutures or steri-strips to secure the chest tube
14. attach 3-way tap and connect the tip of the chest tube via connector to a chest tube (use
minimal suction 10 cmH2O)
15. CXR to confirm position and success !
16. Observe ventilation pressures and FiO2 always before, during and after the
procedure !

Removal Of Chest Drains

If surgical patient not to be removed until documented order in notes by surgeon !
Preparation and Equipment:
1. keep patient fastened. Food / Formula 6 hours, breast milk 4 hours, clear fluids 2 hours.
2. continue monitoring ECG, SpO2, BP.
3. all emergency equipment available.
4. appropriate analgesia.
Age
Morphine
Ketamine

< 6 month
20mcg/kg

> 6 month
0.5mg/kg

if dysphoric response with Ketamine, consider

midazolam 0.1mg/kg, can be repeated once

5. remove drain in aseptic technique during exspiration.

6. repeat CXR 30min after drain removal to exclude pneumothorax
7. patient must be alert and rousable prior to ward discharge !

PICU: CHYLOTHORAX
Definition: accumulation of lymphatic fluid or chyle in the pleural space (triglyceride level >
1.1mmol/l or total cell count > 1000cells/ml with > 80% lymphocytes).
Congenital Chylothorax in lymphangiectasia, congenital heart disease, mediastinal
malignancy, chromosomal abnormalities, H-type TracheoesophagealFistula.
Acquired Chylothorax is usually due to surgery or trauma. Post cardiac surgery incidence
up to 4%, depending on type of surgery (Fontan Circulation, TOF repair, HTX, BCPS) or also
clot formation in the thoracic large veins.
Pathophysiological Chylothorax can develop because of disruption of the thoracic duct or
increased pressure within the intrathoracic lymphatic system due to increased central
venous pressure. Congenital due to abnormal lymphatic drainage.
Complications due to loss of fatty acids and lymphyocytes: hypovolemia, cellular and
humoral immunodeficiency, loss of ATIII, malnutrition due to loss of Lipids
Diagnosis: Triglyceride level > 1.1mmol/l or total cell count > 1000cells/ml with > 80%
lymphocytes in pleural effussion
Treatment:
1. conservative:
management of the underlying disease
dietary modification (low fat diet, medium chain triglyceride diet [monogen] )
TPN only (75% respond within 2 weeks)
Octreotide (decrease of splanchnic and hepatic blood flow reducing the flow of chyle,
side effects: glucosemetabolism disturbance, transient hyperthyroxinemia, abdominal
distension, NEC)
Immunoglobulin supplementation if needed (ie Di-George syndrome)
2. surgical: pleurodesis, surgical abrasion, ligation of the thoracic duct or pleuroperitoneal
shunt
[1] Cochrane Database Syst Rev., 2010, Sep 8: Das et all: Octreotide for the treatment of chylothorax in neonates.
[2] Ann Thorac Surg, 2005, 80, 1864 1871: Chan et all: Postoperative Chylothorax after cardiothoracic surgery in children
[3] Ann Thorac Surg. 2013 Sep;96(3):930-6. Yeh et al: Utility of a clinical practice guideline in treatment of chylothorax in the
postoperative congenital heart patient.
[4] J Thorac Cardiovasc Surg. 2014 Feb;147(2):678-686. Mery CM: Incidence and treatment of chylothorax after cardiac
surgery in children: Analysis of a large multi-institution database.

PICU: GENETIC SYNDROMES AND CARDIAC DEFECTS

Syndrome

Genetics

Presentation

clinical importance

Alagille
Syndrome

Autosomal dominant
with variable
expression

RVOT stenosis
proximal / distal

detailed cardiac assessment

JAG1 mutation
(common)

Chronic cholestasis
(paucity of
intrahepatic bile duct)

Notch2 mutation (rare)

Butterfly vertebrae
Broad forehead
Abnormalities of the
eyes

Beckwith
wiedemann

Chromosome 11p15
alterations

Ophthalmic assessment

(dysplastic kidneys)

Renal ultrasound and renal

function tests

Cardiomegaly, cardiac
defects

Risk for malignancy in early

childhood (Wilms Tumor)

Magroglossia
Abdominal wall
defects
Viseromegaly
Kidney abnormalities
Characteristic facial
features
Neonatal
hypoglycaemia

Chromosome 22 partial
tetrasomy

AP spinal X-ray

Pigmentary
retinopathy

Macrosomia

Cateye
Syndrome

liver function tests (the

conjugated
hyperbilirubinaemia of ALGS
is associated with high GGT
levels), serum cholesterol
and triglycerides, bile acids,
clotting studies, liver
ultrasound, scintiscan, and
biopsy

coloboma of the iris

anal atresia with
fistula
downslanting
palpebral fissures
preauricular tags
and/or pits
frequent occurrence

of heart and renal

malformations,

CHARGE
Syndrome

CHD7 gene mutation

normal or near-normal
mental development
Coloboma ocular
Heart defect

Association to Noonan
Syndrome or Costello
Syndrome

Atresia chonal
Growth deficit
genital hypoplasia
Ear abnormalities
Cleft Lip Palate
Renal abnormalities
trachea-oeseophageal
fistula
Cardiofacialcutaneous
Syndrome

most common:
mutations in BRAF
gene

PS, ASD or HCM

High forehead

Association to Noonan
Syndrome or Costello
Syndrome

Short nose
Widely spaced eyes
Ptosis
Skin abnormalities
Hypotonia
Failure to thrive
Di George
Syndrome
(Velocardiofacial
Syndrome)

22q11 deletion

TOF, PA, VSD, major

MAPCAs, Truncus
arteriosus
Thymus hypo-/aplasia
Cellular
Immunodeficiency
Parathyroid
Hypoplasia
Abnormal facies

Preferably MRI and/or CT

angio preop
analysis of
lymphocyte populations prior
to transfusion
administration
of irradiate blood products,
and aggressive treatment
of perioperative infections
with antimicrobial and
antifungal

prophylaxis
Calcium Subsitution
High mortality due to infection
Send chromosomes (for 22 q
del) & lymphocyte subsets
(before midday to C&W)
before any transfusions
Down
Syndrome

Trisomy 21
Translocation 21
Mosaic

AVSD, ASD, VSD,

TOF
Small eyes, nose,
mouth

No increased mortality in
Down Syndrome Patient
undergoing cardiac surgery
Postoperative Hypotonia

Excess nuchal / skin

edema
GI anomalies
Haematological
disorders
Hypothyroidism
Joint laxity
C1-C2 instability
EhlersDanlos
Syndrome

Cola 5A or Cola 3A
mutations

Valvular CHD
Dilation Aneurysma of
the aorta
Connective tissue
disease
Hypermobiltiy
Vascular: easy
brusing
Kypohoskoliosis

Goldenhar
Syndrome

Various CHD
Incomplete
development of ear,
nose, soft palate,
mandible
Microtia
Ocular dermoid cyst

Difficult intubation

Holt Oram
Syndrome

TBX5 gene

ASD or VSD
Arrhythmia
Upper limb
abnormalities

Kartagener
Syndrome

autosomal recessive

primary ciliar
dyskinesia
PHT
Situs inversus
CHD
Sinusitis
Bronchiectasis

Marfan
Syndrome

Fibrillin 1 gene
mutation on
Chromosome 15

Aortic dilatation with

risk of aortic
discection
Mitral valve prolapse
Arachnodactyly
Hyperextensibility

Noonan
Syndrome
and

PTPN 11 at
Chromosome 12q22
mutation

Hypertrophic CM
Pulmonary stenosis
Branch pulmonary
stenosis

Leopard
Syndrome

Short stature
Short webbed neck
Hemivertebrae
Bleeding diathesis
Trisomy 18

Conotruncal
abnormalities (VSD,
TOF, DORV, AVSD)
IUGR
Microcephaly
Prominent occiput
Overlapping fingers

High risk factor if presentation

as a neonate

Short sternum
Renal abnormalities
GI abnormalities
Turner
Syndrome

45X

Short stature

Aortic valve disease

46XY

Gonadal dysgenesis

Coarctation

CHD

Aortic Dissection

Renal malformation
Facial anomalies
Pterygium colli
Vertebral anomalies

VA(C)TERL
association

VSD or other defects

can develop Fanconi

Anaemia

Anal atresia +/- fistula

Tracheo-oesophageal
fistula
Radial dysplasia
Renal abnormalities
Williams
Syndrome

Chromosome 7q.11.23
deletion

Left and/or right heart

obstructive lesions
Coronary artery
disease

PS may improve over time

with conventional
management

Renal artery stenosis

Craniofacial
abnormalities
First joint laxity then
contractions
Abnormal Calcium
levels

[1] Eur J Cardiothorac Surg. 2012 Aug;42(2):235-40; discussion 240-1. Mainwaring et al: Surgical outcomes for patients with
pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome.
[2] Eur J Hum Genet. 2012 Mar;20(3):251-7. Turnpenny et al: Alagille syndrome: pathogenesis, diagnosis and management.
[3] Appl Clin Genet. 2014 Sep 16;7:169-75. Milani et al: Beckwith-Wiedemann and IMAGe syndromes: two very different
diseases caused by mutations on the same gene.
[4] Cardiol Young. 2014 Sep 12:1-6. Jhang et al: Clinical and molecular characterisation of Holt-Oram syndrome focusing on
cardiac manifestations.
[5] Eur J Cardiothorac Surg. 2009 Apr;35(4):606-14. Formigari et al: Genetic syndromes and congenital heart defects: how is
surgical management affected?
[6] Congenit Heart Dis. 2013 Jul-Aug;8(4):E119-26. Kobayashi et al: Tetralogy of Fallot with complete DiGeorge syndrome:
report of a case and a review of the literature.

[7] J Paediatr Child Health. 2014 Jul;50(7):504-11. Hsu et al: CHARGE syndrome: a review.
[8] Am J Med Genet A. 2012 Dec;158A(12):3087-100. Solomon et al: Clinical geneticists' views of VACTERL/VATER
association.
[9] Asian J Androl. 2014 Jan-Feb;16(1):101-6. Sha et al: Management of primary ciliary dyskinesia/Kartagener's syndrome in
infertile male patients and current progress in defining the underlying genetic mechanism.

PICU: LOW CARDIAC OUTPUT SYNDROME (LCOS)

Definition: oxygen delivery provided by CO does not meet oxygen demand or CI <
2.1l/min/m2 due to inflammatory response of CPB, myocardial ischemia from aortic
crossclamp, hypo-/hyperthermia, reperfusion injury and surgical treatment (ventriculotomy).
Lowest CO usually 6 18hrs post cross clamp removal, return to baseline after 24hrs;
approximately 25% of all children undergoing CHD have critical LCOS.
Monitoring Cardiac Output
arterial blood pressure, but MAP ~ CO x SVR !
cardiac filling pressures: CVP 9 12mmHg, MPAP < 1/3 MAP, LAP 9 15mmHg in
biventricular heart without shunt
peripheral perfusion (capillary refill time)
core peripheral temperature difference
urine output
base deficit
lactate trend
mixed venous saturation (aim SmvO2 > 70% in biventricular heart without shunt)
ECHO (EF, wall motion abnormality, valve dysfunction)
Thermodilution (Gold-Standard)
others: NIRS, esophageal Doppler, bioimpedance measurement, arterial pulse pressure

Management Strategies in LCOS

exclusion of residual defects which compromise CO ( ECHO)
pre-bypass strategies (Methylprednisolone 10mg/kg)
pre - surgical strategies (eg PFO/ASD in compromised RV function after biventricular
repair, Baffle fenestration in Fontan Circulation)
post-surgical strategies: delayed sternal closure or chest reopening
ensure appropriate analgesia and sedation
consider muscle paralysis (to reduce oxygen demand)
preload adjustement: monitor filling pressures in regards to underlying lesion: optimize Hb
(100 140 mg/dl in non-cyanotic, 140 160 mg/dl in cyanotic lesions), NaCl 0.9% bolus or
Albumin 4% bolus
pharmacological support: Inodilator therapy (Milrinone 0.5 1 mcg/kg/min) or
Phenoxybenzamine (0.5 mg/kg 8hrly) to reduce afterload; -adrenergic drugs (Dopamine 5
10mcg/kg/min, Dobutamine 5 10mcg/kg/min, Adrenaline 0.05 0.1mcg/kg/min), but can
increase diastolic dysfunction; short-term use of vasoconstrictors to maintain appropriate
perfusion pressure (Noradrenaline 0.05 0.1mcg/kg/min, Vasopressin 0.02 0.05U/kg/hr)
@ Inotropes
exclude rhythm abnormality (@ Arrhythmia and @ Pacing)
cardio-pulmonary interaction: aim for early extubation in Fontan circulation, TOF, Glenn
Shunt if feasible
Hypothermia: cooling to 34 35C to reduce oxygen consumption
Calcium-infusion (CaCl2 2 10mg/kg/hr or 0.01 0.07mmol/kg/hr), aim for Ca++ : 1.4
1.6mmol/l
Triiodothyronine substitution in selected cases @ T3 in cardiac surgery
Steroid replacement (Hydrocortisone 1 mg/kg every 6hrs)
consider PD
ECLS: @ ECMO or @ VAD
[1] Circulation. 1995 Oct 15;92(8):2226-35: Wernovsky et al: Postoperative course and hemodynamic profile after the arterial
switch operation in neonates and infants. A comparison of low-flow cardiopulmonary bypass and circulatory arrest
[2] Eur J Cardiothorac Surg. 1999 Apr;15(4):515-8: Dalrymple-Hay et al: Induced hypothermia as salvage treatment for
refractory cardiac failure following paediatric cardiac surgery
[3] Pediatr Crit Care Med. 2005 Nov;6(6):655-9: Suominen et al: Hemodynamic effects of rescue protocol hydrocortisone in
neonates with low cardiac output syndrome after cardiac surgery
[4] Am Heart J. 2002 Jan;143(1):15-21: Hoffmann, Wernovsky et al: Prophylactic intravenous use of milrinone after cardiac
operation in pediatrics (PRIMACORP) study. Prophylactic Intravenous Use of Milrinone After Cardiac Operation in Pediatrics
[5] J Cardiothorac Surg. 2010 Nov 17;5:112: Coskun et al: Extracorporeal life support in pediatric cardiac dysfunction
[6] Cardiology in the Young (2009), 19, 573579: Vojitovic et al: Haemodynamic changes due to delayed sternal closure in
newborns after surgery for congenital cardiac malformations
[7] Pediatr Crit Care Med. 2009 May;10(3):313-22: Bronicki et al: Cardiopulmonary interaction
[8] Curr Opin Cardiol. 2010 Mar;25(2):77-9: Absi et al: Noninvasive cardiac output monitoring in the pediatric cardiac Intensive
Care Unit
[9] Crit Care Med. 2001 Oct;29(10 Suppl):S220-30: Wessel et al: Managing low cardiac output syndrome after congenital heart
surgery
[10] Arch Dis Child. 2003 Jan;88(1):46-52: Tibby et al: Monitoring cardiac function in intensive care

PICU: NITRIC OXIDE (NO, INO)

Definition: Nitric oxide (NO) is produced by the endothelium by eNOS (endothelial nitric
oxide synthase). It works beside other chemical mediators, to regulate vascular tone. NO
induces soluble guanylate cyclase to increase cGMP reducing cellular calcium levels and
thus producing smooth muscle relaxation10 and pulmonary vasculature relaxation and a
decrease in PAP. Half-life is 15 30sec, when inhaled, at doses of 5 80ppm. When it
enters the bloodstream it binds to Hb producing nitrosylmethemoglobin (forming
methemoglobin and nitrate), rendering it ineffective at producing systemic vasodilation. It
oxidizes to form NO2 (nitrous dioxide) when exposed to oxygen, which can be toxic to the
alveolar or vascular cells so levels of NO2 > 2ppm should be avoided.
Indications:
conditions where pulmonary hypertension is present
hypoxic respiratory failure (e.g. meconium aspiration, ALI, ARDS)
Monitoring for effectiveness:
PaO2 rise of > 20% impoving V/Q mismatch9
reduction in PAP (but doesnt need to occur to produce PaO2 rise)
reduction of R L shunt
If no significant beneficial effect is shown NO should be immediately discontinued
prior to tolerance occurring. If benefit is shown this must be documented in the notes.
NO can only be commenced with PICU consultant approval !
Monitoring for side effects:
systemic hypotension (with improved pulmonary venous return to a functionally impaired
left ventricle)
daily measurements of methemoglobin levels (ABG results) avoiding levels > 2% avoid
NO > 22ppm
Setup;
Testing of the INOvent prior to use should be done by a person deemed competent by their
institution. Once the unit has been shown to be working properly the following should be
present6:
that the NO2 alarm should be set to an upper limit of 2ppm
that the anaesthetic bag is connected to the INOvent, whilst the patient is on iNO therapy.
that the O2 and NO settings are reading what has been prescribed.
exhaust gases from the breathing circuit must be scavenged to minimize environmental
pollution.
Higher doses of iNO up to 80ppm have been described, but usually no more than
20ppm is recommended, as higher doses showed increased side effects, but no
improvement in outcome !
Weaning:
Weaning can result in rebound PHT and dose reductions should be made cautiously with
some references recommending over 12 24hrs, while other recommending reductions to
be made every 2hrs.
wean iNO every 30min by 1ppm
when iNO is weaned to 2ppm increase FiO2 to 60%
give Sildenafil 0.4mg/kg orally
cease iNO 60min after Sildenafil dose given
wean FiO2 to previous settings

In children mean plasma levels 1 h after doses of Sildenafil 0.52.0 mg/kg are similar to the
maximum plasma concentrations reported in adults receiving doses within the therapeutic
range.
Sildenafil has also been used as a short term prophylactic therapy post-operatively in those
undergoing palliative or definitive surgery for congenital heart disease with varying
improvements in PAP and / or SpO2.
[1] Am J Respir Crit Care Med. 2006 Nov 1;174(9):1042-7: Namachivayam et al: Sildenafil prevents rebound pulmonary
hypertension after withdrawal of nitric oxide in children.
[2] Pediatr Cardiol. 2010 May;31(4):515-20. Epub 2010 Jan 7: Uhm et al: Postoperative use of oral sildenafil in pediatric
patients with congenital heart disease.
[3] Lancet. 1992 Oct 3;340(8823):819-20: Kinsella et al: Low-dose inhalation nitric oxide in persistent pulmonary hypertension
of the newborn.
[4] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S30-6: Barr et al: Inhaled nitric oxide and related therapies
[5] Anesth Analg. 2010 Sep;111(3):693-702: Liu et al: Special article: rescue therapies for acute hypoxemic respiratory failure
[6] Clin Chest Med. 2000 Sep;21(3):519-29: Payen: Inhaled nitric oxide and acute lung injury.
[7] JAMA. 1996 Oct 9;276(14):1189-92: Murad et al: The 1996 Albert Lasker Medical Research Awards. Signal transduction
using nitric oxide and cyclic guanosine monophosphate
[8] J Perinatol. 2004 May;24(5):290-4: Guthrie: Initial dosing of inhaled nitric oxide in infants with hypoxic respiratory failure
[9] N Engl J Med. 2000 Feb 17;342(7):469-74: Clark et al: Low-dose nitric oxide therapy for persistent pulmonary hypertension
of the newborn. Clinical Inhaled Nitric Oxide Research Group

PICU: OPEN CHEST

Facts:
Negative impact on haemodynamics and respiratory parameters after median sternotomy
first described in 1975, emphasizing on compression of the heart and produce a cardiac
tamponade.
Indications for delayed sterna closure and Open Chest:
large heart syndrome
haemodynamic instability after temporal sternal approximation
low cardiac output post CPB
ECMO or VAD cannulation
severe arrhythmia
severe bleeding complications
severe contamination requiring frequent reexploration
Potential complications due to delayed sterna closure with increased rate of infection,
longer PICU stay.
Effects of sternal closure:
increase in intrathoracic pressure
decrease in total lung compliance
decrease in systolic / mean BP
decrease in SV decrease in CO
decrease in cerebral oxygenation
Indication for chest closure: when the patient is ready !
haemodynamically stable (age appropriate MAP with minimal inotropic support, stable
CVP, LA, PAP, stable heart rate /and rhythym, appropriate CRT)
respiratory stable (acceptable ventilation settings, FiO2 requirements)
stable fluid status (oedema ?, fluid balance over last 12 / 24hrs)
Preperation for sterna closure in PICU:
sterile surgical field (gown, mask)
standard monitoring (ECG, invasive BP, CVP (LA, PAP,), Pacemaker, SaO2, etCO2,
Ventilation parameters)
standard setup: standby for inotropic support with Adrenaline or Dobutamine
(Noradrenaline infusion), Adrenaline 10mcg/kg Bolus, fluid resuscitation with NaCl 0.9%,
Human Albumin and PRBC (minimum 2 units crossmatched), good floating fluid line and
separate inotropic line, accessible outside the surgical field
Cephazolin 25 50mg/kg IV minimum 30min preceding the surgical procedure
Fentanyl 5mcg/kg IV Bolus and Midazolam 100mcg/kg IV Bolus plus Vecuronium 0.1mg/kg
IV Bolus to provide adaequate Anaesthesia and muscle relaxation
Observe during and post sternal closure: haemodynamic / respiratory / fluid and
metabolic stability
[1] J Thorac Cardiovasc Surg. 2010 Apr;139(4):894-900: Horvath et al: Cerebral and somatic oxygen saturation decrease after
delayed sternal closure in children after cardiac surgery
[2] Cardiology in the Young (2009), 19, 573579: Vojitovic et al: Haemodynamic changes due to delayed sternal closure in
newborns after surgery for congenital cardiac malformations
[3] J Thorac Cardiovasc Surg. 1997 May;113(5):886-93: Tabbutt et al: Delayed sternal closure after cardiac operations in a
pediatric population

PICU: PACING
NBG Code
I
Paced
0-none
A-Atrium
V-Ventricle
D-dual

II
Sensed
0-none
A-Atrium
V-Ventricle
D-dual

III
Mode
0none
T-triggered
I-inhibited
D-dual

IV
Modulation
0 -none
R-rate modulated

V
Multi-site
A-Atrium

Modes of Pacing
Description
AOO

asynchronous atrial

VOO

asynchronous
ventricular

Indication
Bradycardia w/ intact AV, poor atrial
sensing
Bradycardia w/ conduction problems
and poor ventricular sensing

AAI

demand atrial

Bradycardia w/ intact AV

VVI

demand ventricular

DOO
DVI
DDI
DDD

asynchronous AV
sequential
ventricular inhibited, AV
sequential
dual sensing, AV
sequential
AV universal

Bradycardia w/ conduction problems /

SSS / AF / Overdrive
Bradycardia, which benefits w/
sequential
Desire for dual chamber pacing with
poor atrial sensing

Limitation
vulnerable Phase AF
vulnerable Phase VF
not possible in Atrial
Tachycardia
no atrial seqeuential
mode
vulnerable Phase AF
or VF
risk of AF

all possible
all possible, except atrial
tachyarrhythmia

not in atrial tachycardia

Specific Indications:
AVRT: Consider overdrive pacing in AAI
AF: VVI
Overdrive pacing when JET rate controlled pacing 10% faster in AAI or DDD to regain
atrial kick with AV conduction
Pace termination of reentry tachycardia (either atrial or AVRT): pace AAI 10 - 20%
faster than atrial rate for short burst. If rapid reinitiation after successful capture try gradually
slowing pacing rate after reversion (risk of atrial fibrillation)
Atrial ECG: bipolar - attach atrial wire to right arm and left arm lead (atrial ECG prominent
in I), unipolar attach atrial wire to V1 and V2 (atria ECG prominent in V1 and V2)
Problems & Troubleshooting:
Daily pacemaker check: underlying rhythm, sensing and capture threshold (set threshold
twice as measured)
Failure to pace: causes and treatment: threshold (increase output), ischemia, electrolytedisturbance (correct), post DC, lead malfunction, medication (Flecainide, Sotalol,
Propafenone, Lignocaine, Procainamide), cross-talk inhibition (reduce sensitivity, reduce
output), oversensing (increase sensitivity), can also try to reverse polarity, or addition of
skinlead
Failure to capture: threshold (increase output), ischemia, electrolyte disturbance (correct
!), post DC, medication (Flecainide, Sotalol, Propafenone, Lignocaine, Procainamide) can
also try to reverse polarity
Failure to sense: causes and treatment: sensing threshold (decrease sensing threshold)
Pacemaked-mediated Tachycardia (change mode to DDI, adjust post ventricular atrial
refractory period)
Failure to track in DDD mode: adjust PVARP, AV interval and upper track rate

Checking and Testing the Pacemaker:

Patient non-pacing dependent
Start setup: atrial & ventricular leads connected to pacemaker (PM) cables, cables
unplugged from PM, PM off
Turn PM on, default settings appear (DDD, rate 80, atrial (A) output 10mA, ventricular (V)
output 10mA, A sensing threshold 0.5mV, V sensing threshold 2mV
Testing the sensing thresholds
o Set rate at least 20% below patients rate
o Turn A and V outputs to 0.1mA
o Turn A and V sensing to asynchronous
o Slowly increase V sensing by decreasing the number on scale, and observe the V red light
o Record measured V sensing threshold (?? red light blinking)
o Set V sensing to default 2mV
o Slowly increase A sensing by decreasing number on scale, and observe the A red light
o Record measured A sensing threshold (?? red light blinking)
o Set A sensing threshold to default 0.5mV
Testing the output thresholds
o Set rate at least 20% above patients rate
o Slowly increase A output by increasing the number on the scale, and observe ECG for
distinct rate change
o Record measured A capturing threshold
o Turn A output threshold back to 0.1mA
o Slowly increase V output by increasing the number on the scale, and observe ECG for
distinct rate and QRS shape change
o Record measured V capturing threshold
o Turn V output threshold back to 0.1mA
o Turn rate back down to at least 20% below patients rate
Final PM setting in backup mode
o Check V sensing on 2mV and V light blinking red
o Check A sensing on 0.5mV and A light blinking red
o Set pacing rate at acceptable backup rate below patients own rate
o Turn A output up to 2x measured A output threshold
o Turn V output up to 2x measured V output threshold
Patient pacing dependent by PICU, Cardiology or Cardiac Surgical Consultant only !
[1] Anaesthesia. 2007 Apr;62(4):364-73: Reade: Temporary epicardial pacing after cardiac surgery: a practical review. Part 2:
Selection of epicardial pacing modes and troubleshooting
[2] Pediatr Crit Care Med 2010 Vol. 11, No. 1: Skippen et al: Pacemaker therapy of postoperative arrhythmias after pediatric
cardiac surgery

PICU: PROLONGED QT SYNDROME

Definition: prolonged QTc interval, calculated by Bazetts formula: QTc = QT : sqr (previous
RR interval). normal < 440ms (460ms in women and children).
Congenital pQTS (Jervell and Lange-Nielsen syndrome, Romano-Ward syndrome,
idiopathic) or Acquired pQTS (metabolic: hypokalemia, hypomagnesemia, hypocalcacemia,
drugs: Quinidine, Procaine, Amiodarone, Sotalol, Erythromycin, Terfenadine, Haloperidol,
TCA, Risperidone, Methadone, Droperidol, Organophosphates, or myocardial ischemia, HIV,
Hypothermia)
Diagnosis: pQTS measured via Bazetts formula, Torsades de pointes, T-Wave alternans,
Syncope, family history
Treatment:
1. acute: haemodynamically unstable patient: DC 2J/kg, MgSO4 (0.2mmol/kg), Lignocaine
(1mg/kg over 2min), Isoprenaline infusion (0.05 1mcg/kg/min), overdrive pacing
2. chronic: -Blocker, pacemaker, ICD, sympathectomy
[1] American Heart Journal, 1957, 54, 59 - 68: Jervell et all: Congenital deaf-mutism, functional heart disease with prolongation
of the Q-Y interval and sudden death
[2] Circulation, 2000, 102, 782 - 784: Splawski et all: Spectrum of mutations in long QT syndrome genes: KVLQT1, HERG,
SCN5A, KCNE1, and KCNE2
[3] JAMA, 2003, 23, 2041 2044: Moss: Long QT Syndrome

PICU: PULMONARY HYPERTENSION (PHT)

Definition:
systolic PAP > 35mmHg or
mean PAP > 25mmHg
clinically if systolic PAP : systolic BP < 0.5
I.
I.1
I.2
I.3
I.4
I.5
II.
III.
IV.
V.

WHO Classification
Pulmonary arterial hypertension
Idiopathic
Familial
Associated with collagen vascular disease, portal hypertension, HIV, Drugs & Toxins, congenital
systemic-pulmonary shunts, others
Persistent PHT in the Newborn
Pulmonary veno-occlusive disease
Pulmonary Hypertension w/ left heart disease:
left sided atrial, valvular or ventricular disease (TAPVR, MS, AS, Coarctation)
PHT associated with disorders of the respiratory system: COPD, sleep apnea, central
hypoventilation Syndrome, high altitude, CLD
PHT due to chronic thrombotic or embolic events
Miscs: Sarcoidosis, Histiocytosis, others

Diagnosis:
PA pressure: most reliable, invasive line
LA pressure: Differentialdiagnosis: LV dysfunction !
Echocardiography: measurement of TR jet velocity (modified Bernoulli equation: RVSP =
4 * v2 + RAP), movement of the interventricular septum, identify anatomical problems
Cardiac Catheterisation: right heart catheter (mPAP > 25 mmHg or PVR > 3 Wood
units/m2) vasodilator therapy challenge to guide further therapy
Cardiac MRI: RV structure and function (limited in neonates)
High resolution chest CT with contrast: parenchymal lung disease, thromboembolism,
others
Physiology:
increase in RV afterload RV volume and pressure increase RV systolic dysfunction (
TR) and diastolic dysfunction ( RV diastolic HTN increased right to left shunt if exists
Hypoxia) reduced RV output reduced LV filling reduced CO and reduced coronary
artery perfusion pressure RV ischemia and ventricular interdependence RV systolic
dysfunction
Neonatal PHT:
Incidence 2 : 1000, most common due to MAS, RDS, Pneumonia, also idiopathic or in
congenital diaphragmatic hernia
Postoperative PHT:
preoperative predisposition: increased PVR, increased PBF, increased PVR and PBF,
increased pulmonary venous pressure, lesion related (TAPVD, AVSD, VSD, IAA, Truncus,
Shunt Ops)
cardiopulmonary Bypass: decreased NO production, ischemia-reperfusion injury,
attendant inflammatory response (thromboxane, microemboli, leucosequestration, HPV)
standard cardiovascular monitoring: early signs are tachycardia and hypotension;
Hypoxia occurs early only due to intracardiac shunts or as a late sign !
Prophylaxis for postoperative PHT:
maintain adequate analgesia and sedation (Fentanyl 1mcg/kg iv before painful stimuli),
consider paralysis, normothermia, normal pH, aim paCO2 30-35mmHg, paO2>75mmHg in

non-cyanotic lesion, prevent hyper- and hypoinflation, minimize intrathoracic pressures,

consider Milrinone infusion, consider iNO
Therapy for acute PHT crisis:
increase FiO2 to 1.0: O2 is the best pulmonary vasodilator
support cardiac output: @ Resuscitation, if required, Dopamine (5 10mcg/kg/min),
Dobutamine (5 10mcg/kg/min), Adrenaline (0.02 0.1mcg/kg/min), Milrinone (0.25 0.75mcg/kg/min) as a PDE3 Inhibitor increases cAMP PVR vasodilation
NO donator: increased cGMP vasodilation: commence NO 20 ppm (@ Nitric Oxide),
SNP (0.5 4mcg/kg/min), GTN (0.5 5mcg/kg/min)
Prostacyclin (= Prostaglandin I2 = Epoprostenol): increased cAMP vasodilation,
commence infusion (5 15ng/kg/min), increase slowly to prevent hypotension, or nebulized;
half-life: 3min., can increase PBF and promote pulmonary edema
Surfactant in neonates: promotes lung expansion, commence stat dose of Poractant
alpha (200mg/kg)
consider HFO: promotes lung expansion, avoid hyperinflation
consider ECMO
Therapy for chronic PHT:
Sildenafil: (PDE5 Inhibitor increased cGMP), test dose 0.1 mg/kg, then increase slowly
to maximum 2mg/kg q4hr. FDA recommends against the use in Sildenafil in chronic PHT in
children, as lower doses were not effective, higher doses increased mortality. The
implication of its use in PICU is unclear.
Prostacyclin infusion (5 50ng/kg/min)
Bosentan (1 mg/kg BD, increase to 2mg/kg BD after 4weeks)
Lung Transplantation
[1] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S79-84: Steinhorn: Neonatal pulmonary hypertension
[2] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S27-9: Taylor et al: Fundamentals of management of acute postoperative
pulmonary hypertension
[3] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S23-6: Mullen: Diagnostic strategies for acute presentation of pulmonary
hypertension in children: particular focus on use of echocardiography, cardiac catheterization, magnetic resonance imaging,
chest computed tomography, and lung biopsy
[4] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S15-22: Bronicki et al: Pathophysiology of right ventricular failure in pulmonary
hypertension
[5] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S30-6: Barr et al: Inhaled nitric oxide and related therapies
[6] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S41-5: Ivy: Prostacyclin in the intensive care setting
[7] Pharmacotherapy. 2010 Jul;30(7):728-40: Buckley et al: Inhaled epoprostenol for the treatment of pulmonary arterial
hypertension in critically ill adults
[8] Circulation. 2012 Jan 17;125(2):324-34. Barst et al: A randomized, double-blind, placebo-controlled, dose-ranging study of
oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension.
[9] Pulm Circ. 2014 Mar;4(1):10-24. Brunner et al: Perioperative pharmacological management of pulmonary hypertensive crisis
during congenital heart surgery.

PICU: RENAL FAILURE

Definition: Acute Kidney Injury (AKI): failure of the kidneys to regulate electrolyte, acidbase and fluid homeostasis adequately with concomitant reduction in glomerular filtration
rate (GFR). pRIFLE (paediatric Risk, Injury, Failure, Loss, End-Stage Criteria).
Causes: multi-organ failure, congenital heart surgery, nephrotoxic substances, Stem-Cell
transplantation, Sepsis, HUS.
Grade
Risk
Injury
Failure
Loss
End Stage

pRIFLE
estimated Crea Clearance
Urine Output
decrease by 25 %
< 0.5 ml/kg/hr for 8 hours
decrease by 50 %
< 0.5 ml/kg/hr for 16 hours
decrease by 75 % or
< 0.3 ml/kg/hr for 24 hours or anuric
2
< 35 ml/min/1.73m
for 12 hours
persistent failure > 4 w
persistent failure > 3 mo

Definition: Chronic Renal Failure: hyperfiltration, estimated creatinine clearance < 75

ml/min/1.73m2 , hypertension, microalbuminuria.
Causes: AKI, dysplasia, renal reflux, obstructive nephropathy, chronic GN.
Options for Renal Replacement Therapy (RRT): CVVHD (continuous venovenous
haemodialysis clearance via diffusion), CVVH (continuous venovenous haemofiltration
clearance via convection), CVVHDF (continuous venovenous haemodiafiltration clearance
via convection and diffusion), most commonly done, PD (peritoneal Dialysis), SCUF, IHD
(intermittent haemodialysis).
Variable
Convection
Diffusion
Fluid control
Uraemia control
Vascular access
Anitcoagulation

Comparison of Renal Replacement Therapy

PD
CRRT
+
++
++
++
Tenkhoff
required
not applicable
required

IHD
+++
++
++
+
required
required

[1] Kidney International 71, 2007, 1028 1035, Akcan-Arikan et al: Modified RIFLE criteria in critically ill children with acute
kidney injuryAKI in critically ill children.
[2] Am J Kidney Dis. 2005, Jan;45(1):96-101: Hui-Stickle et al: Pediatric ARF epidemiology at a tertiary care center from 1999
to 2001.
[3] Adv Chronic Kidney Dis, 2008 July ; 15(3): 278283: Goldstein et al: PROGRESSION FROM ACUTE KIDNEY INJURY TO
CHRONIC KIDNEY DISEASE: A PEDIATRIC PERSPECTIVE: An invited review for Advances in Chronic Kidney Disease
[4] Pediatr Nephrol, 2009, 24: 37-49: Walters et al: Dialysis and pediatric acute kidney injury: choice of renal support modality
[5] Pediatr Nephrol. 2005 Jul;20(7):972-6: McNiece et al: Adequacy of peritoneal dialysis in children following cardiopulmonary
bypass surgery.
[6] Schrier: http://www.kidneyatlas.org

PICU: HAEMOFILTRATION & DIALYSIS

Indications:
Correction of water overload
To remove larger quantities of water from the body than the kidney is able to achieve in
order to enable the administration of therapeutic fluids such as parenteral nutrition.
To remove excess electrolytes
Correction of disorders of acid/base homeostasis, including inborn errors of metabolism,
particularly metabolic acidosis
Liver failure (but it does not substitute liver function!).
Removal of urea and other waste products of metabolism in cases of renal failure or
hypercatabolic state
Removal of ingested poisons, drugs or toxins in sepsis.
Most common used: continuous veno-venous haemofiltration (CVVH or CVVHF) or
continuos veno-venous haemodiafiltration (CVVHDF). In CVVHF the filtrate depends on
blood flow rate (aim for 3-5ml/kg/min), the transmembrane pressure = TMP (convection),
change in oncotic pressure along the filter (and so the TMP), prefilter dilution (decreases
urea-/creatinine clearance) and the sieving coefficient (ratio between filtrate concentration
and plasma concentration for a given molecule, eg urea=1, albumin=0).The filtrate is
replaced by a glucose/electrolyte solution (replacement fluid). In CVVHDF the clearance of
small and middle sized molecules is enhanced by counter-current dialysate flow (diffusion).

Anticoagulation:
UFH @ ECMO / Anticoagulation, aim ACT 160 180sec
UFH / Protamine: 1mg Protamine post-filter for every 100U Heparin administered pre-filter
Citrate Anticoagulation: 1ml Citrate per 30ml blood flow, aiming for pre-filter
Ca++<0.4mmol/l and replace post-filter with Ca++> 1.2mmol/l (Cave Mg++, Citrate
accumulation Acidosis)
Replacement Fluid:
for Non-Citrate Anticoagulation: Na+ 140mmol/l, Ca++ 2mmol/l, Mg++ 0.5mmol/l, Cl110mmol/l, HCO3- 32mmol/l, Lactate 3mmol/l /
for Non-Citrate Anticoagulation and Lactate free: Na+ 140mmol/l, Ca++ 1.75mmol/l, Mg++
0.5mmol/l, Cl- 113.5mmol/l, HCO3- 35mmol/l, K+ 4mmol/l, Glucose 5mmol/l /
for Citrate Anticoagulation: Na+ 136mmol/l, Cl- 106mmol/l, Citrate 10mmol/l, Citric Acid
2mmol/l

Catheter/Blood Flow/Filter:
Always aim Blood Flow / Filtrate Flow ratio > 5:1!

Patient size

Catheter Size

Usual Blood Flow Rate

Maximum
recommended
Blood Flow

Haemofilter

< 3 kg

5.0F

5 ml/kg/min

50 mL/min

HF20; Filtrate 200300mL/hr

< 8 kg

6.5F

5 ml/kg/min

75 mL/min

HF20; Filtrate 200300mL/hr

10 - 15 kg

8.0F

5 ml/kg/min

150 ml/min

ST60; Filtrate 9001400mL/hr

>15 kg

11F

5 ml/kg/min

300 ml/min

ST100; Filtrate
6000mL/hr

Adult

14F

5 ml/kg/min

2000 ml/min

ST150; Filtrate
6000mL/hr

Patient monitoring:
Electrolytes (Glucose, Na+, K+, Cl-, HCO3-, Ca++) every 4hrs, hourly for 1st 4 hours if they
were abnormal.
Magnesium and Phosphate twice daily
Fluid Balance per hour = IV fluids in per hour + enteral feeds per hour urine insensible
losses drain losses Patient Fluid removed per hour
Mode

Clinical Use

QDF (Diffusion)

SCUF

Water removal

Nil

CVVH

CVVHD

CVVHDF

Clearance
depends on
TMP, QBF,
Sieving
Clearance
depends on
QBF, QDF
Improved
clearance of
small and middle
size

UFR (Convection)
=
Filtrate flow

QRF

Total Clearance

Nil

UFR

Nil

=
Filtrate flow

= QRF

UFR

QDF

=
QDF + UFR (small)

Nil

QD + UFR
(small)

QDF

=
QDF + UFR

= QRF

QDF + UFR

[1] Pediatr Nephrol 2012 Feb28: Sutherland et al: Continuous renal replacement therapy
[2] Curr Opin Pediatr 2011 Apr;23(2)181-5: Goldstein: Continuos renal replacement therapy: mechanism of clearance, fluid
removal, indications and outcome
[3] Crit Care 2011 Jan 24;15(1)202: Oudemans-van-Straaten et al: Clinical review: anitocagulation for continuous renal
replacement therapy heparin or citrate ?

PICU: TRIIODOTHYRONINE IN CARDIAC SURGERY

Definition: Triiodothyronine levels decrease in infants and children after cardiopulmonary
bypass. Lower T3 concentration is associated with more complicated recovery in PICU after
congenital heart surgery. T3 is safe and has proven clinical benefit in infants less than 5
months of age. Given prophylactically before and after cardiopulmonary bypass, T3
decreases ventilation time and inotrope requirement and improves cardiac function
compared to placebo.
Patient selection: Infants less than 5mths of age having congenital heart surgery with
cardiopulmonary bypass.
T3 Treatment Regimen:
0.4mcg/kg immediately prior to going on to CPB
0.4mcg/kg on release of aortic cross clamp
0.2mcg/kg at 3hrs after cross clamp removal
0.2mcg/kg at 6hrs after cross clamp removal
0.2mcg/kg at 9hrs after cross clamp removal
Goldshield Triiodothyronine (20mcg per vial, $200) does not need to be refrigerated before
use, is cheaper and but has a shorter shelf life. Thyrotardin-inject needs to be refrigerated
and should only be used when Goldshield Triiodothyronine is unavailable.
A SINGLE VIAL CAN BE USED FOR MULTIPLE DOSES for a single patient up to 24hrs
after opening, but must be kept in the fridge once open.
T3 Monitoring:
In order to improve our understanding of the pharmacology of T3 and for quality assurance
T3 concentration will be monitored routinely. Please only order free T3 routinely DO NOT
order other thyroid hormones unless specifically indicated !
Please request free T3 with other routine blood testing according to the following
pre-operatively in infants that it is planned to use T3
prior to the first dose of T3 in the OR
with PICU admission bloods
with the routine morning bloods on the day after surgery and
with the routine morning bloods on the second day after surgery
[1] Eur J Cardiothorac Surg, 2002. 22(6): p. 879-84.Bartkowski, R., et al: Thyroid hormones levels in infants during and after
cardiopulmonary bypass with ultrafiltration.
[2] Intensive Care Med, 2005. 31(4): p. 581-7. Plumpton, K. et al, Identifying infants at risk of marked thyroid suppression postcardiopulmonary bypass
[3] Intensive Care Med, 2009. 36(2): p. 321-8.Plumpton, K.R., B.J. Anderson, and J. Beca: Thyroid hormone and cortisol
concentrations after congenital heart surgery in infants younger than 3 months of age.
[4] Circulation, 2010. 122(11 Suppl): p. S224-33.Portman, M.A., et al., Triiodothyronine Supplementation in Infants and Children
Undergoing Cardiopulmonary Bypass (TRICC): a multicenter placebo-controlled randomized trial: age analysis.

CARDIAC DEFECTS: ANOMALOUS LEFT CORONARY ARTERY (ALCAPA)

Definition: anomalous origin of left coronary artery (LCA) from pulmonary artery; also
known as Bland-White-Garland syndrome; reaching 100% mortality if untreated. Prevalence:
0.2 0.5% in all congenital heart defects. Incidence: 1 : 300.000; ARCAPA (anomalous right
coronary artery from pulmonary artery) possible
Physiology: high PVR in infant period supports an appropriate LCA perfusion pressure;
while PVR falls coronary steal from LCA myocardial ischemia, papillary muscle
infarction, MR (left sided coronary blood flow depending on collaterals and dilated right
coronary artery
Diagnosis: average onset at 6mo - 1yr with poor weight gain, exercise intolerance, CHF,
ECG abnormalities (Q wave, negative T, inversed T, LVH, MI), Echo (flow reversal in LCA,
diastolic flow in MPA, LV dysfunction, WMA, MI, MR), Angio
Preoperative Management:
treatment of congestive heart failure
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.
Surgery: excision of ALCAPA and aortic reimplantation (direct or via tunnel Takeuchi
Operation), MV repair considered only in structural abnormal MV

Postoperative Management:
@ Transposition of great arteries
keep intubated, ventilated, sedated and paralysed for 24 - 48hrs
inotropes: milrinone plus dopamine or adrenaline (plus noradrenaline, aim MAP > 40mmHg
in neonates), aim for an appropriate coronary perfusion pressure
haemodynamics: age adjusted, SBP > 60mmHg, DBP > 30mmHg, MAP > 40mmHg in
neonates, increasing over time, CVP 8 12mmHg, LAP 8 12mmHg)
respiratory: normoxaemia, normocapnea
fluid restriction: 1ml/kg/hr
haemostasis
Specific Problems:
low CO (most common): keep paralysed, dont wean inotropes < 24hrs) early
mechanical assist ! @ low CO
anatomical coronary artery problems (check ECG, Troponin) early investigation (Echo,
Cathlab)
coronary artery spasm (start GTN 5 10mcg/kg/min)
arrhythmia: SVT (Adenosine, Digoxin), JET (slowly Amiodarone), Bradycardia (pacing), AV
Block (Pacing) @ Arrhythmia
Mitral Valve Regurgitation functional MR, treat underlying cause, correct Arrhythmia,
decrease SVR
low urine output: start PD
Outcome:
Mean ICU stay: 8 days. Mortality 30 days: up to 20 %; normal cardiac function within 1 yr
post surgery
[1] Pediatrics. 1949 Oct;4(4):498-507. Gasul et al: Anomalous origin of the left coronary artery from the pulmonary artery
(Bland-White-Garland syndrome) report of four cases.
[2] Interact Cardiovasc Thorac Surg. 2010 Jan;10(1):70-5: Ojala et al: Excellent functional result in children after correction of
anomalous origin of left coronary artery from the pulmonary artery--a population-based complete follow-up study
[3] Interact Cardiovasc Thorac Surg. 2013 Mar 7: Kazmierczak et al: Repair of anomalous origin of the left coronary artery from
the pulmonary artery in infants
[4] Ann Thorac Surg. 2014 Jan 27: Kudumula et al: Twenty-Year Outcome of Anomalous Origin of Left Coronary Artery From
Pulmonary Artery: Management of Mitral Regurgitation.

CARDIAC DEFECTS: ATRIAL SEPTAL DEFECT (ASD)

Definition: Incidence: approx. 100/100.000. Congenital defect of the atrial septum which is
either isolated or associated with other cardiac abnormailities: primum (15% isolated, 30%
total), secundum (50 - 70%), sinus venosus between right pulmonary veins and intraatrial
septum (10%), coronary sinus defect and patent foramen ovale (ostium secundum which is
< 3mm)
Physiology: usually shunts left to right through the defect. However this depends on the
size of the defect and the compliance of the ventricles. Certain situations can reverse the
shunt to right to left: PA, PHT, pulmonary vascular obstructive syndrome, Tricuspid Atresia
and severe Ebsteins anomaly. Chronic left to right shunt leads to volume overload and
subsequent pressure overload of the RA/RV interrupted intraventricular compliance
high PBF, increased PA pressures (more in adolsecents) , lower systemic CO
Clinical Findings: Isolated ASDs are usually asymptomatic in childhood. Most likely clinical
findings are failure to thrive with a 2-3/6 ESM heard loudest upper LSE +/- a widely split S2.
CXR usually non specific, may show cardiomegally. ECG may show right axis deviation with
signs of RVH. May also show RBBB with rsR in V1.
Diagnosis: ECHO, MRI (in sinus venosus defect)
Preoperative Management: Usually asymptomatic so no preoperative management
required. May need additional nutritional support if FTT.
Preoperative Preperation:

ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone in neonates
Surgery: Defect closed using a patch of pericardium or Dacron. Direct closure (suturing)
can be performed if the defect is small. Due to being intracardiac cardiopulmonary bypass
is required.
Percutaneous Repair: Several devices used, does not require CPB and rarely needs PICU
admission post operatively
Timing: PFO and small ASDs only require observation. The majority of small (< 6mm)
isolated ASDs spontaneously close by 2yrs of age. Usually ASDs are closed if the Qp:Qs
ratio is greater than 2:1 (large left to right shunt) or if there is moderately RVH. Reversible
PHT is another consideration. Qp:Qs ratio is generally obtained via cardiac catheter.
Postoperative Management:
Usually able to be extubated 4 hours post return from theatre very common for patient to
be unsettled on first night
inotropes: usually not required
haemodynamics: age adjusted, in neonates: SBP > 60 mmHg, MAP > 40mmHg, increasing
over time, CVP 8 12 mmHg)
respiratory: normoxaemia, normocapnea
fluid restriction: 1 ml/kg/hr, early feeding
haemostasis
Specific Problems:
Problems unusual
Pericardial effusion / tamponade 2 - 5%
cardiac arrhythmias 10 30% in surgical patients
AV block with device closure approx 2% (consider Steriods / Aspirin)
Embolisation of device 2 - 6%
Outcome:
Very good long term survival > 95%. Minimal difference between surgical vs percutaneous
closure.
[1] Nelsons Textbook of Pediatrics 18th Edition.
[2] UpToDate 2010. Management and Outcome of Isolated Atrial Septal Defects in Children. Vick, W, Bezold, L, Fulton, D,
Triedman, J, Kim, M
[3] J Am Coll Cardiol 1193; 22:851. Predictive Factors for Spontaneous Closure of Atrial Septal Defects Diagnosed in the First
3 Months of Life. Radzik, D, Davignon, A, van Doesburg, N, et al.
[4] UpToDate 2007. Devices for Percutaneous Closure of a Secundum Atrial Septal Defect. Weigers, S, St John Sutton, M,
Graham Jr, T, Triedman, J, Connolly, H, Yeon, S
[5] F1000 Medicine Reports 2010, 2:8. Recent Advances in Closure of Atrial Septal Defects and Patent Foramen Ovale.
Qureshi, A, Latson, L
[6] Heart 1999; 82:300-306. Transcatheter Closure of Atrial Septal Defect and Interatrial Communications with a New Self
Expanding Nitinol Double Disc Device (Amplatzer Septal Occluder): Multicentre UK Experience. Chan, K, Godman, M, Walsh,
K, Wilson, N, Redington, A, Gibbs, J
[7] Catheterization and Cardiovascular Interventions 75:767772 (2010). Atrioventricular Block After Transcatheter ASD
Closure Using the Amplatzer Septal Occluder: Risk factors and Recommendations. Shada J, Howard Weber, Ziyad H
[8] Lancet. 2014 May 31;383(9932):1921-32. Geva et al: Atrial septal defects.

CARDIAC DEFECTS: ATRIO-VENTRICULAR SEPTAL DEFECT (AVSD, AVC)

Definition: Atrioventricular septal defect is also known as an endocardial cushion defect

(embryological defect of the endocardial cushion). Complete AVSD are characterized by a
primum ASD and an inlet VSD with a common AV valve (Prevalence 3% of CHD, most
common congenital lesion associated with Trisomy 21). Partial AVSD consist of a primum
ASD and TV/MV clefts. Complete lesions will be classified according to Rastellis
classification Type A, B and C. These are classified according to the common AV valves
dominance to either side. Unbalanced AVSD forms: common AVV opens predominately
towards the larger ventricle, with the contralateral ventricle being hypoplastic. Associated
defects: parachute Mitral Valve, TOF, LVOT obstruction, PDA. Non cardiac defects: @
Heterotaxy
Physiology: The shunt in AVSD is in keeping with their respective ASD and VSD left to
right usually. The degrees of shunt can be influenced by whether the common AV valve is
left or right sided dominant, degree of MR/TR or MS/TS and the type of AV connection re:
DILV. Can be complicated by LVOTO due to accessory valve tissue, AV conduction
abnormalities and left AV valve regurgitation. In Trisomy 21, the onset of pulmonary
vascular disease occurs earlier which often means earlier surgical repair.
Clinical Findings: Partial lesions mimic ASD signs. Complete usually present with FTT,
repeated respiratory tract infections +/- CHF. 3-4/6 pansystolic murmur at the lower LSE.
Mid diastolic rumble due to relative TS/MS. Systolic thrill and hyperactive precordium may
be found. CXR shows cardiomegaly; in complete AVSD: increased pulmonary vascular
markings. May show PA prominence. ECG: RVH and RBBB.
May have 1st degree Heart block and LVH. Superior QRS axis.

Diagnosis: ECHO (LAVV / RAVV ratio) and cardiac catheter

Preoperative Management: Diuretic therapy may be instituted to treat CHF. May benefit
from an ACE inhibitor. Thorough and detailed transthoracic echo to assess the morphology
of the common AV valve as well as bridging leaflets (Rastellis).
Preoperative Preparation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone in neonates
Surgery: Either a one or two patch technique. One patch involves direct closure of the VSD
and repair of the common AV valve leaflets by suturing the leaflets to the crest of the
ventricular septum. A Dacron patch is then applied to the ASD. The two patch technique
involves two Dacron patches to both septal defects and then a separate repair of the valve
leaflets. The other point of controversy is whether the valve cleft should be closed to form a
2 leaflet valve as opposed to a 3 leaflet valve. The literature leans towards closure of the
cleft if possible without causing stenosis.
Timing: Generally before 6 - 12month. Most operations occur between 3 - 6months. This is
to reduce the incidence of PHT.
Postoperative Management:
@Transposition of great arteries
keep intubated, ventilated, sedated and paralysed for 24hrs
inotropes: milrinone plus dopamine or adrenaline (plus noradrenaline)
haemodynamics: age adjusted, in neonates: SBP > 60mmHg, MAP > 40mmHg, increasing
over time, LAP 8 12mmHg, CVP 8 12mmHg)
respiratory: normoxaemia, normocapnea
fluid restriction: 1ml/kg/hr, trophic feeds
haemostasis
Specific Problems:
most common: @ Pulmonary Hypertension (especially in children with prior pulmonary
vascular disease)
postoperative pulmonary venous obstruction (in the immediate postoperative period)
check for left AVVR decrease afterload if blood pressure allows
arrhythmia: SVT, JET, Bradycardia (pacing), AV Block (pacing) @ Arrhythmia @ Pacing
low urine output: start PD
left AV valve regurgitation is the most common reason to re-operate on these children
Outcome: Long term survival very good between 80 - 95%. Some papers suggest
mortality for Trisomy 21 is now 0%. The natural history of this lesion without an operation
has a survival rate of only 4%. In balanced AVSD with associated genetic anomalies (40% 60%) show high mortaility. Unbalanced AVSD less likely to have genetic abnormalities (up to
10%)
[1] Nelsons Textbook of Pediatrics 18th Edition.
[2] Pediatric Cardiology for Practitioners 5th Edition. Park, M
[3] Heart 2006; 92:1879-1885: Craig et al: Atrioventricular Septal Defect: From Fetus to Adult
[4] Orphanet Journal of Rare Diseases 2006; 1:8: Calabro et al: Complete Atrioventricular Canal
[5] Ann Thoracic Surg. 2010 February; 89(2): 530-536: Minich et al: Partial and Transitional Atrioventricular Septal Defect
Outcomes
[6] J Am Soc Echocardiogr. 2013 Feb;26(2):208-16: Beaton et al: Predictors of repair and outcome in prenatally diagnosed
atrioventricular septal defects.
[7] Eur J Cardiothorac Surg. 2014. Buratto et al: Repair of partial atrioventricular septal defect: a 37-year experience.

CARDIAC DEFECTS: BLALOCK-TAUSSIG SHUNT (BTS) OR MODIFIED BTS (MBTS)

Definition: MBTS: Goretex graft from subclavian or innominate artery to the ipsilateral PA to
augment PBF; BTS: direct anastomosis between the transected subclavian artery (or the
innominate artery) and the pulmonary artery
Indication: to provide adequate but not excessive pulmonary blood flow, hence minimizing
the risk of congestive cardiac failure and pulmonary hypertension: TOF, TA, PAIVS,
Ebsteins Anomaly, HLHS
Physiology: aiming for a balanced circulation (SpO2 75 85% aiming for a Qp : Qs = 1 : 1);
Qp:Qs ~ 25 : (95 SaO2)
Postoperative Management:
commence Heparin 10U/kg/hr once no major bleeding
start Aspirin (5 mg/kg) on first postoperative day, once feeds tolerated, cease Heparin if no
CVL in situ
commence morphine sedation, paralyze with cis-atracurium for 12hrs until pulmonary and
systemic blood flow have balanced (discuss specifically at handover plan)
respiratory: SpO2 75 85%, may need some time to settle pulmonary blood flow and
achieve stable saturations
inotropes: usually not required
haemodynamics: SBP > 60mmHg, MAP > 40mmHg, increasing over time, LAP 8
12mmHg, CVP 8 12mmHg)
fluid restriction: 3ml/kg/hr, avoid hypovolaemia, trophic feeds
haemostasis; Hb 130 150g/l

Specific Problems:
Prostaglandin infusion should be weaned slowly, especially if infant was commenced on
more than 48hours
low diastolic BP usually indicates good shunt flow (diastolic run off), but also lower
coronary artery perfusion pressure and risk of splanchnic hypoperfusion
if low SpO2 exclude shunt occlusion (change in murmur ?, ECHO Heparin 50 U/kg,
inform surgeons), Hypovolaemia ( volume bolus), Hypotension ( volume bolus and / or
inotropes), inadaequate CO (@ Inotropes), shunt size ?
if high SpO2 with pulmonary overcirculation leading to pulmonary oedema (unilateral) may
indicate a too large shunt or PDA, which was not ligated: if still intubated: decrease FiO2 to
0.21, allow mild hypercapnea, correct hypovolaemia, increase Hb, PDA ligation if necessary,
try to extubate if feasible, non-invasive Ventilation to support CO
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Tricuspid Atresia
[2] J Card Surg. 2009 Mar-Apr;24(2):101-8: Yuan et al: The Blalock-Taussig shunt
[3] Ann Thorac Surg 2011;92:642-52: Petrucci et al: Risk Factors for Mortality and Morbidity After the Neonatal Blalock-Taussig
Shunt Procedure
[3] Partners of the Heart: Vivien Thomas and His Work with Alfred Blalock, by Vivien T. Thomas (originally published as
Pioneering Research in Surgical Shock and Cardiovascular Surgery: Vivien Thomas and His Work with Alfred Blalock),
University of Pennsylvania Press, 1985
[4] Paediatr Anaesth. 2014 Jan;24(1):114-9: Holtby HM: Anesthetic considerations for neonates undergoing modified BlalockTaussig shunt and variations.

CENTRAL SHUNT
Definition: anastomosis between the ascending aorta and the main pulmonary artery made
of PTFE
[1] Ann Thorac Surg. Nov 1991;52(5):1132-7: Watterson et al: Very small pulmonary arteries: central end-to-side shunt.

SANO SHUNT
Definition: right ventricletopulmonary artery shunt in an attempt to overcome the
obstacles noted with a systemic-to-pulmonary artery shunt (diastolic runoff and low coronary
artery perfusion pressure); in fact: higher diastolic blood pressure in the first 72 hours. After
12 months no difference in mortality.
[1] J Thorac Cardiovasc Surg. Aug 2003;126(2):504-9; discussion 509-10: Sano et al: Right ventricle-pulmonary artery shunt in
first-stage palliation of hypoplastic left heart syndrome
[2] J Card Surg. 2009 Mar-Apr;24(2):101-8: Yuan et al: The Blalock-Taussig shunt
[3] Pediatr Crit Care Med. 2006 May;7(3):238-44: Cua et al: Early postoperative outcomes in a series of infants with hypoplastic
left heart syndrome undergoing stage I palliation operation with either modified Blalock-Taussig shunt or right ventricle to
pulmonary artery conduit.
[4] N Engl J Med. 2010 May 27;362(21):1980-92: Ohye et al: Comparison of shunt types in the Norwood procedure for singleventricle lesions.

CARDIAC DEFECTS: COARCTATION OF THE AORTA (COA)

Definition: obstructive anomaly of the aortic arch; classification by Amato et al: I. CoA with
or without PDA (a. with VSD, b. with other defects), II. CoA with isthmus hypoplasia with or
without PDA (a. with VSD, b. with other defects), III. CoA with tubular hypoplasia of the
isthmus with or without PDA (a. with VSD, b. with other defects). Prevalence 5 - 8% of all
CHD. Incidence 4 : 10.000 of all life births. Localisation: proximal CoA: 10mm from the left
subclavian artery. Hypoplastic Transverse Arch (TAA): diameter TAA : diameter
descending aorta (level of diaphragm) < 0.6. Genetic association to Trisomy 13 and 18.
[Shones syndrome: CoA, supravalvular MS, parachute MV and subaortic stenosis]
Physiology: with PDA closure acute increase in LV afterload decreased CO,
increased LVEDP CCF (in extreme: myocardial ischemia) and shunt reversal along PFO
(and VSD if present) increased PBF severe CCF with systemic hypotension; in older
children with less obstruction, physiology is less severe LVH and aortic collateralisation
Diagnosis: rarely referred in newborn period (PDA): Hypertension of upper limbs usually not
present before Day 5, usually after PDA closure with signs of CCF of various degree, ECG:
signs of LVH, later RVH and LVH, CXR: cardiomegaly and pulmonary congestion, cardiac
catheterization (diagnostic and interventional), MRI
Preoperative management:
commence Prostaglandine E1 (20ng/kg/min) to maintain systemic perfusion. Intubate and
sedate to lower oxygen consumption. Hypoventilation to higher PVR and to lower SVR.
balanced circulation with PDA open and / or VSD present (aim SpO2 75 85%)

 Dopamine (5 10mcg/kg/min), Dobutamine (5 10mcg/kg/min) or Adrenaline (0.02

0.1mcg/kg/min) may be required to stabilize for low CO @ LCOS
careful fluid resuscitation (obstructive lesion, not hypovolaemic)
in older children: treat hypertension with -Blocker (ie Propanolol 1.5mg/kg/d oral or
Metoprolol 0.1mg/kg/dose IV or Esmolol infusion)
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, FISH, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10 mg/kg 12 hrs and 6 hrs pre surgery in neonates,
arterial line on right upper limb
Surgery: resection of the stenotic segment and end-to-end or end-to-side anastomosis,
patch augmentation, subclavian-flap aortoplasty or extended resection with primary
anastomposis. Prosthetic graft in older children or adolescents. Angioplasty (balloon) or
stent insertion in selected cases.
Postoperative Management:
keep intubated, ventilated, sedated and paralysed for 24 hours for patients with preceding
high PBF; elective cases can be extubated within 4hrs
inotropes: milrinone plus dopamine or adrenaline
haemodynamics: age adjusted, in neonates: SBP > 60mmHg but < 80mmHg, MAP >
40mmHg; prevent hypertension (SNP infusion or Esmolol infusion @ Vasodilators)
respiratory: normoxaemia, normocapnea
fluid restriction: 1 ml/kg/hr, trophic feeds only
haemostasis, Hb 120 140
keep normothermia
Specific Problems:
acute hypertension (increase of Noadrenaline release due to sympathetic stimulation
during repair): SNP or Esmolol infusion
postcoarctectomy syndrome: hypertension, abdominal pain, ileus (2 3days post repair)
antihypertensive treatment
PHT if high PBF was preceding (VSD or ASD) @ PHT
thoracic duct injury with @ Chylothorax
laryngeal nerve injury
spinal cord injury (A. spinalis anterior injury): 0.4 1.5%
aneurysmatic dilation (up to 35%)
Outcome:
Perioperative Mortality: < 1% in isolated CoA, but 5 7% in CoA plus VSD, up to 50% in
CoA plus HLHS or other defects. Recoarctation 5 50%. Long term antihypertensive
treatment required in 30%. Long term survival after 30 years: 80%. In Balloon Angioplasty
aortic wall injuries over time (up to 40%: dissection, Aneurysm), with Stenting up to 7%.
More reinterventions necessary with Stenting.
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Coarctation of the Aorta
[2] Pediatr Cardiol. 2011 Feb 32(2) Gillett et al: Underrecognition of Elevated Blood Pressure Readings in Children After Early
Repair of Coarctation of the Aorta
[3] Ann Thorac Surg. 2009 Dec;88(6):1923-30; discussion 1930-1: Brown et al: Recurrent coarctation: is surgical repair of
recurrent coarctation of the aorta safe and effective?
[4] Curr Opin Cardiol. 2009 Nov;24(6):509-15: Tanous et al: Coarctation of the aorta: evaluation and management.
[5] Am J Cardiol. 2013 Feb 19: Gray et al: Long-Term Follow-Up of Neonatal Coarctation and Left-Sided Cardiac Hypoplasia.
[6] J Am Coll Cardiol. 2011 Dec 13;58(25):2664-74. Forbes et al: Comparison of surgical, stent, and balloon angioplasty
treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional
Study Consortium).

CARDIAC DEFECTS: DOUBLE OUTLET RIGHT VENTRICLE (DORV)

Definition: Malposition of the great arteries, where the valvular area of LVOT and RVOT are
supported by the right ventricle (100% + >50%), VSD present (subaortic 65%, subpulmonary
25%, double committed 3%, non-committed 7%, AVSD/Heterotaxy), variable RVOT
obstruction. Prevalence 1.3% of congenital heart disease
Physiology: various clinical pictures, depending on position relation of VSD to the great
arteries, obstruction of RVOT and concomitant malformations:
VSD Type DORV with subaortic (or double committed) VSD and mild valvular/subvalvular
PS high Qp CCF (@ VSD)
TOF Type DORV with subaortic (or double committed) VSD and moderate/severe
valvular/subvalvular PS low Qp Cyanosis and TET-Spells (@ TOF)
TGA Type DORV with subpulmonary VSD and aortic coarctation (in 50% of the cases, @
CoA) very high Qp CCF (@ TGA)
non-committed or @ AVSD/@ Heterotaxy depend on the concomitant malformations
Diagnosis: Echo, MRT, CT
Preoperative Management:
depending on primary physiology:
low Qp: commence Prostaglandin E1 = Alprostadil (starting dose 20ng/kg/min) to augment
PBF, consider: balloon valvuloplasty, ductal stent, RVOT stent in @ TOF Type DORV or
BAS in @ TGA Type DORV
high Qp with CCF: NIV, IPPV, (Digoxin), Frusemide (1mg/kg up to QID), Spironolactone
(1mg/kg BD), aim for afterload reduction: Milrinone 0.25 0.5mcg/kg/min or Captopril (0.1
2mg/kg TDS)
Preoperative Preperation:
ECG, CXR, CUS, FBE, FISH, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10 mg/kg 12 hrs and 6 hrs pre surgery in neonates.
Surgery: biventricular approach in @ TOF type or @ VSD type or @ TGA type DORV, in
@ TGA type DORV with valvular / subvalvular pulmonary PS: Rastelli OP, REV procedure,
Metras modification, Nikaidoh procedure (aortic root translocation and biventricular outflow
reconstruction). Univentricular approach in complex DORV with @ Glenn, @ Fontan surgery
Postoperative Management:
@ BT-Shunt
@ Glenn Shunt
@ Fontan circulation
Specific Problems:
arrhythmia (AV Block) @ Arrhythmia
Outcome:
Depending on presentation, clinical, anatomical presentation and type of surgery. Rastelli
surgery appears less favourable results than REV or Metras procedure
[1] J Thorac Cardiovasc Surg. 2008 Feb;135(2):331-8. Hu et al: Strategy for biventricular outflow tract reconstruction: Rastelli,
REV, or Nikaidoh procedure?

CARDIAC DEFECTS: EBSTEINS ANOMALY

Definition: atrialization of the right ventricle with inferior placement of the septal and
posterior leaflet of the tricuspid valve, most common redundant anterior leaflet. Associated
defects: ASD. Prevalence: < 1% of congenital heart disease. Incidence: 0.5 2.5 : 100.000
Physiology: various clinical picture, depending on position of leaflet: TR, RV hypoplasia,
RVOTO, systolic and diastolic dysfunction of LV and associated defects. RA enlargement
and accessory pathways predispose to arrhythmias. Cyanosis with right left shunt
Diagnosis: Echo (Gose Score), CXR (cardiomegaly)
Differential Diagnosis: aberrant tendinous chords
Preoperative Management:
Neonates: CCF due to TR and RV dysfunction, commence Prostaglandin E1 = Alprostadil
(starting dose 20ng/kg/min) to augment PBF, however a large PDA with left right shunt
can cause systemic hypoperfusion (circular shunt due to PR) cease Alprostadil
Adolescents / Adults: right heart failure due to TR @ Cardiomyopathy and @ Arrhythmia
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10 mg/kg 12 hrs and 6 hrs pre surgery in neonates.
Surgery: depending on clinical and anatomical presentation: biventricular approach with
Tricuspid Valve repair: Annuloplasty +/- prosthetic ring, Tricuspid Valve replacement
(mechanical or bioprosthetic), plication of atrialized part of right ventricle, reduction of right

atrioplasty, relief of RVOTO, if present. univentricular approach with systemic-pulmonary

shunt with or without closure of Tricuspid Valve, 1 ventricle repair @ Glenn, @ Fontan
surgery
Postoperative Management:
in Tricuspid Valve repair or replacement: anticipate RV dysfunction: Milrinone (0.25
1mcg/kg/min), Arrhythmias @ Arrhythmia, Anticoagulation: commence Heparin 10U/kg/hr
once no bleeding, increase Heparin dose further to adjust for therapeutic aPTT. Vitamin K
Antagonist in mechanical valve (INR 2.5 3.5) once stable and all drains removed. Continue
UFH until INR > 2.0 for two consecutive days @ Anticoagulation
1 ventricle repair @ Glenn Shunt
Fontan surgey @ Fontan circulation
Specific Problems:
arrhythmia (accessory pathways !) @ Arrhythmia
TR
Outcome:
Depending on presentation, clinical, anatomical presentation and type of surgery
[1] Heart. 2008 Feb;94(2):237-43: Paranon et al: Ebstein's anomaly of the tricuspid valve: from fetus to adult: congenital heart
disease.
[2] Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2009.12:63-65: Bove et al: How I Manage Neonatal Ebsteins
Anomaly
[3] Ann Thorac Surg. 2013 Nov;96(5):1703-9. Davies et al: Current spectrum of surgical procedures performed for Ebstein's
malformation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database
[4] Eur J Cardiothorac Surg. 2013 Sep 11. Vouhe PR: Management of neonatal Ebstein's anomaly: towards a rational
approach?

CARDIAC DEFECTS: FONTAN CIRCULATION

(TOTAL CAVOPULMONARY ANASTOMOSIS, TCPC)

Definition: separates pulmonary and systemic blood flow in single ventricle physiology;
accomplished by intraatrial lateral-tunneling of the IVC flow into RPA or extracardiac via
conduit (extracardiac Fontan)
Physiology:
better systemic oxygenation
reduced volume load to systemic ventricle
may be fenestrated to obtain adequate CO postoperative
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2).
Risk assessment for Fontan candidates:
elevated PVR (> 4 Wood units or mPAP > 15mmHg)
impaired ventricular function (EF < 45%)
impaired ventricular diastolic function
AV valve incompetence (LVEDP > 12mmhg)
small PA size (McGoon ratio)
subaortic obstruction

Postoperative Management:
Option for Anticoagulation: commence Heparin 10U/kg/hr once no major bleeding ,
further long-term anticoagulation with Vitamin-K antagonist (INR 2 3)
respiratory: normoxaemia, in fenestrated Fontan SpO2 > 80%, normocapnea (mild
hypercapnea improves oxygenation !), try to extubate early if feasible
inotropes: usually not required, if so milrinone to decrease PVR and SVR and improve
ventricular dysfunction
goal is to lower PVR as possible, as main determinant factor for CO in postoperative
Fontan circulation, extubate early !
fluid restriction: 2ml/kg/hr for the first day, feed early
haemostasis (restrictive transfusion strategy if stable)
remove any SVC / CVL as soon as possible
Specific Problems:
pleural effusions replace losses (full / half / quarter)
low CO: Hypovolemia (low CVP, low LAP) volume
Obstruction SVC / PA anastomosis (high CVP, low LAP) ECHO, Angio
high PVR (high CVP, low LAP) lower PVR: Milrinone, @ Nitric Oxide
exclude pulmonary venous obstruction (ECHO)
ventricular dysfunction (high CVP, high LAP) commence low dose Milrinone
(0.25mcg/kg/min) as more common diastolic dysfunction, exclude AV valve regurgitation
(ECHO)
persistent hypoxaemia: fenestration, low CO ECHO, low SmvO2, unrecognized
abnormal systemic connection ECHO, Angio, lung disease
systemic venous hypertension pleural effusion, ascites drain early; if chronic: proteinloosing enteropathy
dysrrhythmias avoid inotropic, chronotropic support if feasible, AV sequential pacing if
nevessary @ Arrhythmias
Outcome:
low perioperative mortality < 2%
higher mortality in redo Fontan / Revision Fontan (12%)
freedom of death or transplantation after 1 y: 80%, 5 yrs: 77%, 10 yrs: 75%, 25 yrs: 54%
major morbidity: tacharrhythmias, thrombotic events, PLE, plastic bronchitis
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Tricuspid Atresia
[2] Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S57-69: Giglia et al: Preoperative pulmonary hemodynamics and assessment
of operability: is there a pulmonary vascular resistance that precludes cardiac operation?
[3] Interact Cardiovasc Thorac Surg. 2010 Mar;10(3):428-33: Gewillig et al: The Fontan circulation: who controls cardiac
output?
[4] Interact Cardiovasc Thorac Surg. 2010 Feb;10(2):262-5. Gewillig et al: Volume load paradox while preparing for the Fontan:
not too much for the ventricle, not too little for the lungs
[5] Pediatr Cardiol. 2007 Nov-Dec;28(6):448-56: Deal t al: Arrhythmia management in the Fontan patient.
[6] Circulation. 2008 Jan 1;117(1):85-92: Khairy et al: Long-term survival, modes of death, and predictors of mortality in patients
with Fontan surgery.
[7] Pediatr Crit Care Med. 2011 Vol. 12, No.1: 39-45: Cholette et al: Children with single-ventricle physiology do not benefit from
higher haemoglobin levels post cavopulmonary connection: Results of a prospective, randomized, controlled trial of a restrictive
versus liberal red-cell transfusion strategy

CARDIAC DEFECTS: GLENN SHUNT

(BIDIRECTIONAL CAVOPULMONARY ANASTOMOSIS, BCPC)

Definition: transection of the SVC, which is anastomosed to RPA Hemi-Fontan: confluence

of SVC and RA anastomosed to RPA; redirection of IVC and coronary sinus via baffle
through ASD into LA
Physiology:
aim for SpO2 75 85%
LV preload and cardiac output maintained by IVC flow
Pulmonary blood flow maintained by SVC flow. Transpulmonary gradient: SVC (CVP)
LAP
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2).
Postoperative Management:
commence Heparin 10U/kg/hr once no major bleeding, change to Aspirin (5 mg/kg) orally
once enteral feeds tolerated
respiratory: SpO2 75 85%, may need some time to settle pulmonary blood flow and
achieve stable saturations, normocapnea (hypercapnea to improve oxygenation), try to
extubate early if feasible

 inotropes: usually not required, if so milrinone to decrease PVR and SVR and improve
ventricular dysfunction
fluid restriction: 2ml/kg/hr for the first day, feed early
haemostasis. Restrictive transfusion if stable.
remove all central lines as soon as possible
Specific Problems:
persistent hypoxaemia (SpO2 < 70%) may indicate a mechanical obstruction of SVC
RPA (> 2 5mmHg) anastomosis
elevated PVR leading to hypoxaemia (increased transpulmonary gradient >18 mmHg) if
intubated, aim for extubation if feasible; try higher FiO2, normal pH, trial of NO. Do not
hyperventilate decreased cerebral blood flow ! Evacuate any pleural effusion early !
increased LAP pressure (> 12mmHg) commence milrinone, check ECHO for ventricular
function, AV valve regurgitation
consistent pulmonary venous congestion check for anomalous connection SVC to LA
risk of air embolism due to right left shunt (from IVC territory)
Outcome:
good palliation in younger children; but as child grows IVC blood flow increases
desaturation @ Fontan Circulation
1 ventricular repair preferable: forward-flow / pulsatile flow into the PA in selected
patients to prevent pulmonary AV fistulas
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Tricuspid Atresia
[2] Ann Thorac Surg. 1999 Sep;68(3):976-81; discussion 982: Mavroudis et al: Bidirectional Glenn shunt in association with
congenital heart repairs: the 1(1/2) ventricular repair
[3] Arch Surg 1963;86:101: Shumacker: Discussion of Reed WA, Kittle CF, Heilbrunn A: Superior vena cava-pulmonary artery
anastomosis
[4] Acta Med Scand 1956;154: 15161.Robicsek et al: A new method for the treatment of congenital heart disease associated
with impaired pulmonary circulation
[5] Pediatr Crit Care Med. 2011 Vol. 12, No.1: 39-45: Cholette et al: Children with single-ventricle physiology do not benefit from
higher haemoglobin levels post cavopulmonary connection: Results of a prospective, randomized, controlled trial of a restrictive
versus liberal red-cell transfusion strategy
[6] J Thorac Cardiovasc Surg. 2013 Feb;145(2):451-4. Ferns et al: Is additional pulsatile pulmonary blood flow beneficial to
patients with bidirectional Glenn?

CARDIAC DEFECTS: HETEROTAXY SYNDROME

Definition: Situs ambiguous, subtype into asplenia / polyspenia or isomerism of right atrial
appendage / isomerism of left atrial appendage (morphological two left atrium pulmonary
veins connect to right atrium). Incidence 1 4 : 100.000
Physiology: various physiology, depending on the subtype and associated cardiac defects
right atrial isomerism: RVOTO (90%, PA in 20%), AV discordance, DORV, TGA, TAPVD,
bilateral Sinus node, bilateral right sided lungs, Asplenia, Malrotation, Cilia dysfunction
left atrial isomerism: 66% usually mild congenital heart disease (common atrium, balanced
or unbalanced @ AVSD, @ TAPVD), hypoplastic Sinus node, bilateral left sided lungs,
Polyspenia
Diagnosis: ECHO, US abdomen (biliary atresia, duodenal atresia, asplenia / polyspenia),
Contrast upper GI for excluding malrotation
Preoperative Preperation:
ECG, CXR, CUS, abdominal US, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2),
Platelets (2), Cryoprecipitate (2).
Preoperative Management:
depending on underlying lesion
Surgery: depending on clinical and anatomical presentation: biventricular approach as
underlying lesion. univentricular approach with @ HLHS, @ BT Shunt, @ Glenn, @
Fontan or Kawashima operation for single ventricle palliation and interrupted IVC with
azygous / hemiazygous continuation (superior cavopulmonary anastomosis, hepatic venous
return into atrium)
Outcome:
high mortality (asplenia > 85%, polyspenia >50% mortality in first year of life)
Kawashima operation: development of pulmonary arteriovenous malformations with
progressive desaturation ( redirection of hepatic blood flow into pulmonary circulation)
[1] Cardiol Young. 2007 Sep;17 Suppl 2:1-28: Jacobs et al: The nomenclature, definition and classification of cardiac structures
in the setting of heterotaxy.
[2] Circ J. 2012;76(9):2066-75. Shiraishi et al: Human heterotaxy syndrome from molecular genetics to clinical features,
management, and prognosis.
[3] Pediatr Cardiol. 2012 Apr;33(4):596-600. Eronen et al: Outcome of left atrial isomerism at a single institution.
[4] Pediatr Cardiol. 2013 Feb;34(2):302-7. Eronen et al: The outcome of patients with right atrial isomerism is poor.

CARDIAC DEFECTS: HYPOPLASTIC LEFT HEART SYNDROME (HLHS)

Definition: abnormal left heart development, resulting in underdevelopment of LV, aortic

valve, Aorta, mitral valve and aortic arch. Incidence 0.2 : 1000 of all live births.
Physiology: PDA dependant systemic perfusion PDA closes results in acidosis,
hypoxaemia and shock; in patient with restrictive ASD / PFO cardiogenic shock develops
right after birth
Diagnosis: prominent loud single II. heart sound, ECHO
Differential diagnosis: Coarctation, critical AS, IAA (all PDA dependant systemic lesions)
Preoperative Management:
secure vascular access (two peripheral IVs or UVC) no multiple attempts !
commence Prostaglandin E1 (20ng/kg/min) to maintain systemic perfusion. Watch for
apnea, manage conservatively if tolerated.
If detoriating with pulmonary over-circulation (increasing saturations, decreasing perfusion).
Intubate and sedate to lower oxygen consumption with Hypoventilation to higher PVR (aim
pH 7.30 7.35) and to lower SVR (hypercarbia), SNP (0.5 4mcg/kg/min to lower SVR aim MAP > 35mmHg in neonates).
with restrictive ASD / PFO: immediate Intubation and Resuscitation, urgent BAS or
septectomy (10% of HLHS)
no enteral feeds until surgical correction, consider gastric protection

Management of low CO preoperative:

SpO2 > 85 excessive pulmonary blood flow NIV, Intubation, Sedation, Filling, SNP
Impaired systemic ventricular function (+/- tricuspid regurgitation) consider Intubation,
Dobutamine, Milrinone
SpO2 < 65% and Acidosis: confirm Diagnosis of restrictive ASD via ECHO Intubation,
Resuscitation, urgenat BAS
restrictive PDA (variable SpO2) increase Prostin
Preoperative Preperation:
ECG, CXR, CUS, renal US, FISH, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2),
Platelets (2), Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in
neonates.
Surgery: primary cardiac transplantation. Staged repair: Norwood procedure (preferably
if stable on Day 3 or 4 of life) or Hybrid procedure (bilateral PA band, PDA stenting)
Norwood procedure, later @ Glenn Shunt (4 8mth) @ Fontan (18mth 4yrs).
Norwood procedure: reconstruction of aortic root and aortic arch, disconnection of
pulmonary trunk and incorporating the RV stump into systemic circulation; lung perfusion by
BT Shunt (diastolic runoff possible) or Sano-Shunt (obstruction possible)

Postoperative Management:
@ Transposition of great arteries
keep intubated, ventilated, sedated and paralysed for 24 - 48hrs
inotropes: Milrinone plus Dopamine or Adrenaline (plus Noradrenaline, aim MAP >
40mmHg in neonates), aim for maximal vasodilation to reduce strain on RV
haemodynamics: SBP > 60mmHg, MAP >35mmHg, increasing over time, CVP 8
12mmHg)
respiratory: balance circulation (SpO2 70 85%), normocapnea
fluid restriction: 1ml/kg/hr
haemostasis
introduce feeds when haemodynamically stable (not before Day 2)
introduce Captopril gradually when tolerating feeds and haemodynamically stable
commence Aspirin 5mg/kg OD once enteral full feeds established
consider Clopidogrel 0.2mg/kg OD (all surgical lines and pacing lines must be removed
prior) in selected patients
consider long term central venous access (Broviac) for feeding intolerance and need for
long term parenteral nutrition
Specific Problems:
low CO: keep paralysed, dont wean inotropes < 24hrs adaption of the RV for systemic
circulation
anatomical coronary artery problems (check ECG, Troponin) early investigation (Echo,
Cathlab)
coronary artery spasm (start GTN 5 10mcg/kg/min)
arrhythmia: @ Arrhythmia and @ Pacing
low urine output: start PD
ECHO assessment: function of systemic RV, patency of BT shunt / Vmax across Sano
shunt, degree of Tricuscpid regurgitation, adequacy of ASD, evaluation of Neoaorta and
Arch
feeding intolerance: consider feeding protocol
Outcome:
Mean ICU stay: 5days. Mortality 30days: up to 40%; HLHS w/ restrictive ASD and BAS:
mortality up to 50%. Consider Intermediate Care Support post ICU.
[1] Lab Invest 1952, 1(1): 61-70: Lev M: Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes
[2] Pediatr Clin North Am 1958, 5(4):1029-1056: Noonan et al: The hypoplastic left heart syndrome; an analysis of 101 cases
[3] Cardiol Clin 1989; 7:377385: Norwood: Hypoplastic left heart syndrome
[4] Ann Thorac Surg 1991; 52:688695: Norwood: Hypoplastic left heart syndrome
[5] N Engl J Med 1983; 308:2326: Norwood et al: Physiologic repair of aortic atresiahypoplastic left heart syndrome
[6] Circulation 2004;109;2326-2330: Vlahos et al: Hypoplastic Left Heart Syndrome With Intact or Highly Restrictive Atrial
Septum: Outcome After Neonatal Transcatheter Atrial Septostomy
[7] Am Heart J. 2011 Jan;161(1):138-44: Trivedi et al: Arrhythmias in patients with hypoplastic left heart syndrome
[8] Korean Circ J. 2010 Mar;40(3):103-11: Honjo et al: Hybrid palliation for neonates with hypoplastic left heart syndrome:
current strategies and outcomes
[9] Pediatric Anesthesia 2010 20: 3846: Naguib et al: Anesthetic management of the hybrid stage 1 procedure for hypoplastic
left heart syndrome (HLHS)
[10] N Engl J Med 2010;362:1980-92: Ohye et al: Comparison of Shunt Types in the Norwood Procedure for Single-Ventricle
Lesions
[11] Eur J Cardiothorac Surg. 2013 Mar 7. Mnsterer et al: Treatment of right ventricle to pulmonary artery conduit stenosis in
infants with hypoplastic left heart syndrome.
[12] Circulation. 2012 Sep 11;126(11 Suppl 1):S123-31. Baba et al: Hybrid versus Norwood strategies for single-ventricle
palliation.
[13] Heart. 2014 Jan 10. Lloyd et al: Analysis of preoperative condition and interstage mortality in Norwood and hybrid
procedures for hypoplastic left heart syndrome using the Aristotle scoring system.

CARDIAC DEFECTS: INTERRUPTED AORTIC ARCH (IAA)

Definition: obstructive anomaly of the aortic arch; classification by Celoria et al: Type A
(20%): IAA distal to left subclavian artery, type B (78%) between left subclavian and left
carotid artery, type C (2%) proximal to left carotid artery. Most of them associated with VSD
or other defects. Prevalence 1% of all CHD. Incidence 4 : 10.000 of all life births. Genetic
association to Di-George-Syndrome
Physiology: with PDA closure acute increase in LV afterload decreased CO,
increased LVEDP CCF (in extreme: myocardial ischemia) and shunt reversal along PFO
(and VSD if present) increased PBF severe CCF with systemic hypotension
Diagnosis: Hypertension of upper limbs usually not present before Day 5, usually after PDA
closure with signs of CCF of various degree, ECG: signs of RVH. CXR: cardiomegaly and
pulmonary congestion, ECHO (always evaluate left sided structures), cardiac catheterization
(diagnostic and interventional), MRI
Preoperative management:
commence Prostaglandin E1 (20ng/kg/min) to maintain systemic perfusion. Intubate and
sedate to lower oxygen consumption. Hypoventilation to higher PVR and to lower SVR.
balanced circulation with PDA open and / or VSD present (aim SpO2 75 85%)
Dopamine (5 10mcg/kg/min), Dobutamine (5 10mcg/kg/min) or Adrenaline (0.02
0.1mcg/kg/min) may be required to stabilize for low CO @ LCOS
carefully fluid resuscitation (obstructive lesion, not hypovolaemic !)
Calcium infusion if Di-George Syndrome @ Inotropes

Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, FISH, PRBC(4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.
Surgery: preferred single stage repair with end-to-end or end-to-side anastomosis, patch
augmentation, subclavian-flap aortoplasty or extended resection with primary anastomosis
and also VSD closure. In patients with severe LVOTO @ HLHS
Postoperative Management:
keep intubated, ventilated, sedated and paralysed for 24 hours for patients with preceding
high PBF; elective cases can be extubated before
inotropes: milrinone (plus dopamine)
haemodynamics: age adjusted, in neonates: SBP > 60mmHg but < 80mmHg, MAP
>40mmHg; prevent hypertension (SNP infusion or Esmolol infusion)
respiratory: normoxaemia, normocapnea
fluid restriction: 1ml/kg/hr, careful trophic feeds
haemostasis, Hb 100 140
keep normothermia
Specific Problems:
acute hypertension (increase of Noradrenaline release due to sympathetic stimulation
during repair): SNP or Esmolol infusion
postcoarctectomy syndrome: hypertension, abdominal pain, ileus (2 3dys post repair)
antihypertensive treatment
PHT if high PBF was preceding (VSD or ASD) @ Pulmonary Hypertension
thoracic duct injury with Chylothorax @ Chylothorax
laryngeal nerve injury
spinal cord injury (A. spinalis anterior injury): 0.4 1.5%
neurological injury due to DHCA
Outcome:
Perioperative Mortality: 5 7%. Recoarctation 5 50%. Long term antihypertensive
treatment required in 30%. Long term survival after 10yrs: 94% (IAA and VSD), 72% (IAA
and TGA), 5 yrs: 47% (IAA and other defects)
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Interrupted Aortic Arch
[2] Eur J Cardiothorac Surg. 2009 Apr;35(4):565-8: Kobayashi et al: Outcomes following surgical repair of aortic arch
obstructions with associated cardiac anomalies
[3] J Thorac Cardiovasc Surg. 2013 Sep 24. Lee et al: Outcomes of patients born with single-ventricle physiology and aortic
arch obstruction: The 26-year Melbourne experience.

CARDIAC DEFECTS: LEFT VENTRICULAR OUTFLOW OBSTRUCTION (LVOTO)

Definition: wide morphological spectrum with mild aortic stenosis (AS) to HLHS; most
common valvular AS (70 80%), followed by subvalvular AS (10 20%) and supravalvular
AS (rare), which is common in Williams Syndrome (1 : 20.000 live birth: mental retardation,
behavioral traits, elfin facies with AS and peripheral PA stenosis). All LVOTO can be
associated with other defects.
Physiology: LVH due to increase in afterload, subendocardial ischemia coronary
ischemia with cardiomyopathy; reduced blood fow through LVOT hypoplasia of mitral
valve, LV and aortic arch
Diagnosis: ECG (left axis, LVH), ECHO, in critical AS early presentation with cardiogenic
shock when PDA closes, in mild forms: failure to thrive, increased WOB. Only 15% present
under age 1yr.
Management preoperatively with critical AS:
PGE1 infusion (20ng/kg/min) to maintain PDA patency for systemic perfusion
Intubation and ventilation as required
Milrinone (0.25 0.5mcg/kg/min) and Noradrenaline (0.02 0.1mcg/kg/min) to improve
CO, avoid -adrenergic drugs (worsening diastolic dysfunction, Tachycardia)
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.
Surgery: dependant on morphological structures, development of LV and LVOT and
associated defects (aortic root and mitral valve diameter, LV/RV ratio): single ventricle
pathway @ HLHS) or two-ventricle surgery for AS: surgical valvotomy, percutaneous balloon
valvotomy, dilation of LVOTO (Konno procedure) and/or aortic valve replacement with
pulmonary autograft and pulmonary homograft (Ross procedure) or combined: RossKonno procedure. Yasui operation in patient with good sized ventricules and VSD (a
combination of integrated aortic arch reconstruction, redirection of LV outflow through the
VSD to both the semilunar valves and establishment of RV-PA continuity using a valved
conduit)
Postoperative Management:
@ Transposition of great arteries
keep intubated, ventilated, sedated and paralysed for 24 - 48hrs
inotropes: Milrinone plus Dopamine or Adrenaline (plus Noradrenaline, aim MAP >
40mmHg in neonates)
haemodynamics: SBP >60 mmHg, MAP >40mmHg, increasing over time, LAP 8
12mmHg, CVP 8 12mmHg)
respiratory: normoxaemia, normocapnea
fluid restriction: 1ml/kg/hr, trophic feeds
haemostasis
Specific Problems:
low CO: keep paralysed, dont wean inotropes < 24hrs
anatomical coronary artery problems (check ECG, Troponin) early investigation (ECHO,
Cathlab)
coronary artery spasm: start GTN 5 10mcg/kg/min
arrhythmia: SVT (adenosine, digoxin), JET (slowly amiodarone), Bradycardia (pacing), AV
Block (pacing) @ Arrhythmia
low urine output: start PD

 severe AI (15 20%) mainly in balloon valvotomy

residual AS
no anticoagulation required in Ross-Konno procedure (in valved conduits consider
anticoagulation)
Outcome:
depending on the underlying lesion; average PICU stay: 5 - 8dys
pulmonary homograft needs reoperation with age
autograft may develop AI
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Left Ventricular Outflow Obstruction
[2] J Thorac Cardiovasc Surg. 1966 Apr;51(4):484-92: Cornell et al: Supravalvular aortic stenosis
[3] Eur J Cardiothorac Surg. 2007 Jun;31(6):1013-21: Alsoufi et al: Management options in neonates and infants with critical left
ventricular outflow tract obstruction
[3] Circulation. 2006 Nov 28;114(22):2412-22: Aboulhosn et al: Left ventricular outflow obstruction: subaortic stenosis, bicuspid
aortic valve, supravalvar aortic stenosis, and coarctation of the aorta
[4] Ann Thorac Surg. 2003 Nov;76(5):1398-411: Brown et al: Surgery for aortic stenosis in children: a 40-year experience
[5] J Card Surg. 2012 Jan;27(1):103-11. Anagnostopoulos et al: Surgical management of left ventricular outflow tract
obstruction.
[6] Eur J Cardiothorac Surg. 2010 Feb;37(2):279-88. Hickey et al: Ross and Yasui operations for complex biventricular repair in
infants with critical left ventricular outflow tract obstruction.

CARDIAC DEFECTS: PATENT DUCTUS ARTERIOSUS (PDA)

Definition: postnatal communication between MPA and descending aorta. Incidence 0.3 4
: 10.000
Physiology: functionally closure of DA within 10 15hrs postnatally (triggered by rise in
paO2 and decrease in PGE2 and PGI2, however VLBW and especially ELBW infants have a
symptomic PDA persistent (reduced sensitivity to all above). PDA leads to increased PBF
and increased LV volume load
Diagnosis: Echo (PDA > 1.4mm/kg, LA or LV enlargement, diastolic flow reversal in
descending aorta indicate haemodynamic significance), BNP > 1000pg/ml
Problems in PDA:
Neonates: prolonged ventilation BPD / CLD, IVH, PVL, NEC
Children / Adolescents: LVH ( CCF), Eisenmenger syndrome, growth restriction,
aneurysmal dilation of PDA, calcification, infective Endocarditis
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (2), FFP (2), Platelets (1),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates
Surgery: transcatheter PDA closure or surgical ligation by left posterolateral
thoracotomy or VAT

Postoperative Management:
usually unproblematic postoperative course, nil support required, early extubation if
feasible
Outcome:
Low mortality and morbidity (potentially adverse events: laryngeal nerve damage,
chylothorax, pneumothorax, bleeding)
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Aortopulmonary Septal Defects and Patent Ductus
Arteriosus
[2] Ann Surg. 46:33; 1907: Munro: Surgery of the vascular system, I. Ligation of the ductus arteriosus
[3] Ann Pediatric Card. 2009;2: 36-40:Azhar et al: Transcatheter closure of patent ductus arteriosus: evaluating the effect of the
learning curve on outcome.
[4] Clin Cardiol. 2014 Jan 6. Wang et al: Catheterization Therapy vs Surgical Closure in Pediatric Patients With Patent Ductus
Arteriosus: A Meta-Analysis.

CARDIAC DEFECTS: PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

(PA with IVS)

Definition: atretic pulmonary valve leads to an hypertrophic and hypoplastic RV cavity.

Pulmonary blood flow depends on PDA. Incidence 3 : 10.000 and / or MAPCAs (major
aortopulmonary collateral arteries)
Physiology: decreased pulmonary blood flow (TET-spells) due to RVOT obstruction and
increased PVR and small PAs or / and congestive cardiac failure (diuretics, Digoxin,
nutrition). Dual pulmonary blood supply in MAPCAs
Diagnosis: ECHO, Angiography for coronary anatomy and to rule out ventriculocoronary
fistula, Diagnosis of MAPCA
Management preoperatively:
PGE1 infusion (20ng/kg/min) to maintain PDA patency
for persistent hypoperfusion with restrictive intraatrial communication BAS
aim for SpO2 75 85%
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery.
Surgery: dependant on anatomical associations, degree of right ventricular hypoplasia and
development of pulmonary arteries (Z-score of tricuspid valve, branch PAs < 2.5mm):
in selected patients: balloon valvotomy (would require ongoing PGE1 infusion as the RV
needs time for remodeling) for Pulmonary Stenosis (with antegrade flow through RVOT)

 univentricular approach (Z-score < - 4): systemic-pulmonary shunt (@ BT-Shunt) @ Glenn

Shunt @ Fontan Circulation
biventricular repair (Z-score > -2) @ TOF
Creation of an aortopulmonary window for uni- or biventricular repair with / or without
unifokalisation
Reconstruction of the RVOT
partial biventricular repair (1.5 ventricle repair) @ Glenn Shunt
Postoperative Management:
As per @ BT-Shunt, @ Glenn Shunt or @ Fontan Circulation
Specific Problems:
high inotropic support and / or severe cyanosis may be indicators for inappropriate RV size
and function
To prevent shunt thrombosis commence Heparin 10U/kg/hr once no major bleeding
Outcome:
Long Term Survival: 86% depending on repair, underlying genetic defects (Di-GeorgeSyndrome)
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Pulmonary Atresia with intact ventricular septum
[2] Cardiol Young. 2005 Oct;15(5):447-68: Freedom et al: The significance of ventriculo-coronary arterial connections in the
setting of pulmonary atresia with an intact ventricular septum
[3] Ann Thorac Surg. 2013 Feb 28. Mainwaring et al: Hemodynamic Assessment After Complete Repair of Pulmonary Atresia
With Major Aortopulmonary Collaterals.
[4] Ann Thorac Surg. 2012 Sep;94(3):842-8. Mainwaring R et al: Late outcomes in patients undergoing aortopulmonary window
for pulmonary atresia/stenosis and major aortopulmonary collaterals.
[5] J Thorac Cardiovasc Surg. 2010 Nov;140(5):1092-103. Carotti et al: Determinants of outcome after surgical treatment of
pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

CARDIAC DEFECTS: SINGLE VENTRICLE PHYSIOLOGY - FUNCTIONALLY

UNIVENTRICULAR HEART, PARALLEL CIRCULATION (PC)
Definition:
Either natural or palliated situation where blood to the pulmonary circulation is supplied in
parallel rather than serial to the systemic circulation by the same pumping chamber(s).
Anatomy: A single ventricle (SV) physiology or PC exists in:
a. True SV anatomy maintained by natural (Ductus Arteriosus or MAPCAS) or artificial
systemic-to-pulmonary artery shunts (classic or modified Blalock-Taussig Shunt / central
shunt / Sano shunt).
b. Two ventricle anatomy with obstructed pulmonary or systemic outflow (PA/VSD,
IAA, critical PS, AS, Coarctation) maintained by natural (Ductus Arteriosus or MAPCAS) or
artificial systemic-to-pulmonary artery shunts (classic or modified Blalock-Taussig Shunt,
central shunt).
c. Two ventricle anatomy with unobstructed pulmonary or systemic outflow, but large,
non-restrictive intra- or extracardiac shunt lesion (ASD, VSD, AVSD, AP Window, Truncus
arteriosus, PDA).
Pathophysiology:
Nature foresees a maximally dilated pulmonary vascular bed (eg nitric oxide pathway)
compared to a variably constricted systemic vascular bed (PVRSVR), therefore, the
common pathophysiologic feature in PC is an unrestrictive pulmonary blood flow with
increased pulmonary (Qp) compared to systemic (Qs) blood flow - Qp/Qs>1 - resulting in
volume overload, and subsequent failure of the systemic ventricle(s). The acuity of the latter
is dependent on the underlying anatomy (a>b>c), age of the infant (physiologic drop in PVR
after birth), associated cardiac lesion (AV valve competency), and concurrent illness
(bronchiolitis etc).
Further specific pathophysiologic consideration:
Ad a. More critical heart failure in single right ventricle anatomy. Deep cyanosis due to
mixing at various levels. Potential for acute pulmonary stealing in the distressed child, with
sudden loss of systemic cardiac output.
Ad b. Moderate cyanosis. Impaired gut perfusion with increased risk of NEC in the lesions
with systemic outflow obstruction.
Ad c. Pulmonary vascular endothelial dysfunction with vascular hyper-reactivity and mainly
postoperative risk of PHT.
Management:
Whether pre- or postoperatively, core concept in managing SV physiology or PC is to
balance the circulation, ie to optimize systemic output and control pulmonary blood flow.
The optimal Qp/Qs for a balanced circulation is 0.8 1. The latter can be estimated from
formula (SaO2SmvO2) / (SpvO2 SpaO2). Simplification 1: SmvO2 = SpaO2, and
SpvO2 = 100. Simplification 2: if SaO2 > 80 in room air, then Qp : Qs > 1.0
General considerations: Anticipate @ LCOS (clinical, Lactate, SmvO2 [30 5], pCO2 [7
1]), Optimise oxygen balance (DO2 [HR x Hb x SaO2 x PL x Contr x AL] VO2 [MV,
analgesia, sedation, paralysis, normo-/hypothermia]) early, and Avoid resuscitating
inaedequate haemodynamic indices late. Manipulation of Qp: PVR (SaO2 80, pCO2,
PEEP, IT, Hct). Manipulation of Qs: SVR (vasodilatation)
[1] Cardiol Young 2003;13:316-322. Lawrenson J et al. Manipulating parallel circuits.
[2] Cardiol Young 2004;14(Suppl 1):52-60. Nelson DP et al. Neonatal physiology of the functionally univentricular heart.
[3] Arch Dis Child Fetal Neonatal Ed 2005;90:F97-F102. Theilen U et Shekerdemian L. The intensive care of infants with
hypoplastic left heart syndrome.
[5] Congenit Heart Dis. 2012 Sep-Oct;7(5):466-78. Lowry: Resuscitation and perioperative management of the high-risk single
ventricle patient: first-stage palliation.

CARDIAC DEFECTS: TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR)

Definition: connection of the pulmonary to remnants of embryologic venous circulation

rather than the left atrium. This may include all four pulmonary veins (TAPVR) or only part of
it (PAPVR). Classification by Darling, Rothney and Craig: supracardiac 50% (pulmonary
veins drain via innominate vein intro RA), infracardiac 20% (pulmonary veins drain via portal
vein into RA), intracardiac 20% (pulmonary veins drain via coronary sinus into RA) and
mixed type 10%. An ASD is always present. 1/3 of all patients have associated other
defects. Incidence 8 : 100.000 of live birth. Prevalence 2.2% in CHD
Physiology: depending on the PBF, which is anatomically dependant on the left to right
shunt (abnormal pulmonary venous drainage), the obstruction of the pulmonary drainage - if
present - and on the right to left shunt (ASD and PDA). If severely obstructed pulmonary
venous drainage decreased PBF: severe pulmonary edema and PHT associated with
severe right to left shunt (ASD and PDA) hypoxaemia, both leading to severely depressed
CO. In mild to moderate pulmonary venous obstruction, usually increased PBF with falling
PVR CCF with PHT. If no pulmonary venous obstruction than only mildly increased PBF,
mild cyanosis CCF develops later
Diagnosis: depending on the degree of pulmonary venous obstruction and PBF: early
presentation with severe cyanosis, low CO in neonatal period in severely obstructed
patients, later presentation usually implies mild to moderate obstruction only CHO, cardiac
catherisation, Echo (flow acceleration > 2m/sec indicates obstruction), Angiography, MRI
Differentialdiagnosis: Sepsis, PPHN, Cor triatrium

Preoperative Management:
no or mild obstruction require usually no special treatment
moderate obstruction presenting with CCF require anti-failure treatment (@
Cardiomyopathy)
severe obstruction: Intubation, ventilation and sedation, stabilization of cardiac output @
Inotropes) and treatment of @ Pulmonary Hypertension, even @ ECMO. Commence
Prostaglandin E1 (20 ng/kg/min) to maintain systemic perfusion, however this may decrease
further PBF and pronounce cyanosis
Surgery: depending on age, presentation, anatomy: various technique with ligation of the
aberrant vein and reanastomosis to the left atrial appendage or intracardiac baffle
Postoperative Management:
keep intubated, ventilated, well sedated (Fentanyl for any noxious stimulus) and paralysed
for 24 - 48hrs
inotropes: Milrinone plus Dopamine or Adrenaline (plus Noradrenaline, aim MAP >
40mmHg in neonates)
haemodynamics: SBP > 60mmHg, MAP > 40mmHg, increasing over time, CVP 8
12mmHg, PAP < SBP
respiratory: balanced circulation (SpO2 75 85%) in single-ventricle physiology,
normoxaemia in biventricular, normocapnea
fluid restriction: 1ml/kg/hr, trophic feeds
haemostasis
Specific Problems:
most common: @ Pulmonary Hypertension (especially in post pulmonary venous
obstruction patients and younger patients)
postoperative pulmonary venous obstruction (in the immediate postoperative period) @
Chylothorax. Differential Diagnosis: primary Lymphangioectasy
arrhythmia: SVT (adenosine, digoxin), JET (slowly amiodarone), Bradycardia (@ Pacing),
AV Block (@ Pacing) @ Arrhythmia
low urine output: start PD
Outcome:
Mean ICU stay: 5days. Mortality 30days: up to 90% in single-ventricle TAPVR, but 5 35%
in two-ventricle physiology. Reobstruction of pulmonary venous flow possible (2.5 13%)
[1] Lab Invest. 1957 Jan-Feb;6(1):44-64: Craig et al: Total pulmonary venous drainage into the right side of the heart; report of
17 autopsied cases not associated with other major cardiovascular anomalies.
[2] Tex Heart Inst J. 1985 Jun;12(2):131-41: Reardon et al: Total anomalous pulmonary venous return: report of 201 patients
treated surgically
[3] Ann Thorac Surg. 2005 Feb;79(2):596-606; discussion 596-606: Hancock Friesen et al: Total anomalous pulmonary venous
connection: an analysis of current management strategies in a single institution.
[4] Circulation. 2007 Mar 27;115(12):1591-8. Karamlou T et al:vFactors associated with mortality and reoperation in 377
children with total anomalous pulmonary venous connection.

CARDIAC DEFECTS: TRANSPOSITION OF GREAT ARTERIES (TGA)

Definition: Defined as aorta arising from anatomic RV, and PA arising from anatomic LV.
Most common form: AV concordance and VA discordance with associated VSD (40%),
Coarctation / IAA (10%), LVOTO (10%), coronary branching anomalies (> 30%, Leiden
classification). Less common forms: Taussig-Bing anomaly (TGA with outlet VSD & DORV);
congenitally corrected TGA (ccTGA, VA discordance and AV discordance).
Physiology: Amount of mixing crucial. TGA/VSD cyanotic and more prone to CHF, TGA/IVS
deep cyanosis and postnatal CVS collapse. While the RV is abnormally thick walled, the LV
is usually thin, and pLV is determing timing of surgery.
Diagnosis: ECHO, postductal SpO2 may be higher than preductal SpO2
Preoperative Management:
Ideal Monitoring: ECG, pre- and postductal SpO2, NIVBP, NIRS.
Ideal Installations: Leave UVC and UAC if surgery within 24 -48 hrs; patients on PGE1
require 2x iv access at all times. Consider PICC line if on PGE1 for > 48hrs.
Proposed Investigations Echo immediately upon admission, then as per need, or once per
week. Echo will attempt to define coronary artery anatomy but recognition there will be
limitation to administration.
One cranial ultrasound between BAS & surgery, more as per clinical indication.
Probable Therapeutic Interventions Prostaglandin E1 (PGE1, Alprostadil) 200mcg/kg in
50ml Dextrose 5% at 10 - 100ng/kg/min as per Guardrail. Balloon Atrio-Septostomy
Indications: If SaO2 <70% on PGE1 performed via umbilical or femoral vein under echo or
fluoroscopic control. Requires anaesthesia, IPPV, and close non-invasive monitoring. Most
children do not need intra-arterial monitoring or insertion of a central line for inotropic

administration. Usual post-procedure plan: wake, wean & extubate, and attempt wean off
PGE1 asap. Suggested Fluids / Nutrition Oral or NG feeds
Preoperative Preperation: ECG, CXR, CUS, FBE, Clotting, U&C, FISH, Electrolytes, PRBC
(4, irradiated), FFP (2), Platelets (2), Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs
and 6hrs pre surgery in neonates.
Surgery: Timing: depending on pLV and defect (TGA / IVS 10days, TGA / VSD 3mths,
Taussig-Bing 9mths), Technique: Arterial Switch Operation (ASO), PAB and systemic
shunt in unprepared LV, Mustard/Senning for atrial switch, Rastelli Operation for patients
with malalagniment of VSD and/or LVOTO. Note: ASO possible after Mustard / Senning;
may need PAB to retrain LV function.
Ideal Monitoring: Invasive systemic BP, CVP or RAP, LAP, NIRS, transcutaneous SaO2,
inline SvO2 (PediaSat), urine output, core temperature
Ideal Installations: Right radial arterial line, 3-lumen PediaSat right IJV, or 2-lumen
PediaSat right IJV plus direct RA line, direct left atrial line, 2x peripheral iv access, IDC,
advance naso-pharyngeal to esophageal temperature probe, naso-gastric tube, double
atrial and ventricular pacing wires
Proposed Investigations:
On admission: CXR, ABG and VBG (for PediaSat recalibration), ACT (and TEG), clotting,
FBC, RFT, LFT, electrolytes, BNP, Troponin I, 12-lead ECG
Transthoracic echo as per discussion at handover (LVsystF, LVdiastF, RVF, PAP, PS, AI,
coronary blood flow).
ABG (and VBG if no PediaSat) 1 - 2hrly for the first 6 - 12hrs, more frequently in periods of
instability or manipulation of inotropes
Subsequent daily checks: CXR, Echo, ABG and VBG (for PediaSat recalibration), clotting,
FBC, RFT, LFT, electrolytes, BNP, Troponin I, 12-lead ECG
Probable Therapeutic Interventions
Airway and Ventilation: aim for TV 6 - 8ml/kg and pCO2 35-40mmHg, PEEP min
5cmH2O. If stable @ 2hrs after PICU admission: ET suctioning and standard lung
recruitment maneuvre (PEEP no greater than 15 cmH2O for 2mins, decrements by 5cmH2O
every 30secs to 5 cmH2O), which should be repeated after each airway disconnection.
Antibiotic Prophylaxis @ Cardiac Admission
Prophylactic Triiodothyronin @ T3 in cardiac surgery
Analgesia and Sedation: Morphine infusion up to 50mcg/kg/min, Midazolam infusion
maximum 30mcg/kg/min, iv Paracetamol from admission on; if required add
Dexmedetomidine 0.2 - 0.7mcg/kg/min after cessation of paralysis
Paralysis: Cisatracurium iv bolus 0.15mg/kg, then continuously @ 1 - 10mcg/kg/min as
per Guardrail until next morning after Echo
Bleeding from wound or chest drains should be closely observed at all times from time of
admission on; output of > 10ml/kg anytime, > 5ml/kg/hr in the first 2hrs, or > 1ml/kg/hr
beyond 4hrs postop should result in readiness for administration of blood products,
coagulation tests (platelets, INR, aPTT, fibrinogen and TEG), and notification of the cardiac
surgical fellow. Beware of sudden increase in output as well as sudden stop !
Cardiovascular Agents;
Dopamine 5 - 10mcg/kg/min plus Milrinone 0.25 - 0.75mcg/kg/min preferably via separate
lines, or
Adrenaline max 0.03 - 0.08mcg/kg/min plus Milrinone 0.25 0.75mcg/kg/min via direct RA
line if available; Adrenaline can go with Dopamine line, but preferably not with Milrinone
Substitution of or switching off inotropes should not be undertaken in the first 6 - 12hrs
post-op
Accepted vasodilators in acute phase: Milrinone (if renal failure max 0.3mcg/kg/min) and
Dexmedetomidine
Do not cease inotropic support until after extubation (except in longterm MV patients)

Aim for Haemodynamic Steady-State

Defined as SBP > 65mmHg, MAP 45mmHg, LAP 5 - 8mmHg, SvO2 60%, Lactate <
3mmol/L
If intotropic score exeeds 15 (Dopamine dose + [100] x Adrenaline dose + [10] x Milrinone
dose in mcg/kg/min: vigorous investigation of causes, and consider reopening of sternum.
If inotropic score exeeds 20, causes need to vigorously be investigated and / or elective
ECLS (VAD or VA ECMO) considered.
The pace maker should be readily available, and the sensitivity and output thresholds of
pacing wires measured / tested and recorded, unless already provided by Anaesthesia or
otherwise agreed on at the handover (@ Pacing)
Vigorous temperature control (36.5 0.5 C) from admission on is of crucial importance.
Peritoneal dialysis should be started with low threshold indication; begin with Physioneal
1.37%, 10ml/kg cycle volumes @ room temperature, 1hrly cycle times, and no additives
(Heparin or KCl).
Suggested Fluids / Nutrition / Electrolytes: Fluid restriction 30% first 24hrs, as of day 2
50% until extubated, increments of 20ml/kg per day thereafter - max TF (=100%) for iv
120ml/kg/d, for enteral 150ml/kg/d. For maintenance use Hartmanns & 5% Dextrose. Start
NG feeds @ 1ml/hr (EBM or substitute) 6hrs post-CPB. Vigorous fluid restriction, avoid fluid
boluses. Keep Mg++ >1mmol/l, K+ 4 - 5mmol/l, and Ca++ >1.2mmol/l at all times, but avoid
fast bolus.
Specific Problems:
low CO 6 12hrs post CPB: keep paralysed, dont wean inotropes < 24 hours) adaption
of the LV for systemic circulation @ LCOS
anatomical coronary problems. Higher risk if single coronary, or intramural course, or
coronary looping (LAD and/or Cx off RCA and vice-versa). May cause ischaemic ECG
changes, or ventricular ectopics or arrhythmias. (check ECG, Troponin) early
investigation (ECHO, Cathlab)
arrhythmia: SVT, JET, AV Block (exclude coronary abnormality) @ Arrhythmia
low urine output: start PD
pulmonary artery hypertension (rare) @ PHT
Expected Schedule: Cease paralysis within 24hrs; start wake and wean day 2; remove
intracardiac lines, chest drains, PD and pacing wires day 2 or 3; extubation expected day 3
(48hrs post-op); discharge to ward day 4; discharge home day 14. Surgical mortality
TGA/IVS < 2%, TGA/VSD < 4%, Taussig Bing 6%.
Proposed Pre-Discharge Preparations:
if required low flow nasal-prong oxygen
Inotropes ceased
On increasing enteral, and decreasing iv fluids/feeds
FBC, electrolytes, RFT, LFT, coag, BNP, Troponin I
12 lead ECG and CXR and ECHO not older than 24hrs
Usual medication with Frusemide 1mg/kg 8hrly and Spironolactone 1mg/kg BD, additional
afterload reduction with Lisinopril 0.1mg/kg daily as per discussion with cardiologist and
cardiac surgeon
@ PICU discharge
[1] J Thorac Cardiovasc Surg, 1981 Oct;82(4):629-31: Lecompte at al: Anatomic correction of transposition of the great arteries.
[2] Surgery. 1959 Jun;45(6):966-80: Senning: Surgical correction of transposition of the great vessels
[3] Circulation, 2003;107;996-1002: Hoffmann et al: Efficacy and Safety of Milrinone in Preventing Low Cardiac Output
Syndrome in Infants and Children After Corrective Surgery for Congenital Heart Disease
[4] Pediatr Crit Care Med. 2010 Mar;11(2):234-8: Gaies et al: Vasoactive-inotropic score as a predictor of morbidity and
mortality in infants after cardiopulmonary bypass
[5] Am J Cardiol. 2013 Feb 19. Junge et al: Comparison of Late Results of Arterial Switch Versus Atrial Switch (Mustard
Procedure) Operation for Transposition of the Great Arteries.
[6] Front Pediatr. 2013 Jun 6;1:11. Unolt et al: Transposition of Great Arteries: New Insights into the Pathogenesis.

CARDIAC DEFECTS: TETRALOGY OF FALLOT (TOF)

Definition: TOF is the most common cyanotic congenital heart disease, accounts for 5% to
10% of all congenital heart disease, and has an incidence of approximately 1 : 2000 live
births. Classically described with the cardiac constellation of, 1. ventricular septal defect
(usually large), 2. overriding aorta, 3. right ventricular outflow tract obstruction (subvalvular,
valvular, supravalvular), 4. right ventricular hypertrophy. A wide spectrum of TOF exists,
including the variants of pulmonary stenosis (TOF-PS), pulmonary atresia (TOF-PA/MAPCA)
and absent pulmonary valve (TOF-APV). See also @ Pulmonary Atresia. Right sided arch
25% of cases.
Physiology: decreased pulmonary blood flow (TET-spells) due to RVOT obstruction and
increased PVR or / and congestive cardiac failure (diuretics, Digoxin, nutrition)
Diagnosis: Echo
Management of cyanotic spells:
oxygenation
volume expansion: 10 ml/kg IV colloid/crystalloid
sedation: IV Morphine 10 - 50mcg/kg; or Fentanyl 1 mcg/kg
posture: head down position/knee chest (increase VR; mild pressure over femoral arteries
to increase SVR)
vasopressor: Metaraminol 5 - 10mcg/kg IV or Phenylephrine 5 10mcg/kg, then 1 5mcg/kg/min IV/(SC)
Esmolol 100mcg/kg 500mcg/kg (titratable) +/- infusion

Preoperative Preparation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.
Surgery: Timing: dependant on anatomical associations, degree of pulmonary stenosis and
development of pulmonary arteries:
1. Complete correction at 3 6mths
Patch repair VSD
resection of infundibular muscle
Pulmonary valvotomy +/- transannular patch.
2. Staged procedure with BTS (@ BT-Shunt) in severe cyanosis or hypercyanotic spells in
neonatal period.
10 - 15% require re-operation to relieve RVOTO, +/- residual VSD. Majority require
pulmonary valve replacement for incompetence in the medium-to-long term.
Postoperative Management:
@ Transposition of great arteries
keep intubated, ventilated, sedated and paralysed for 24hrs
inotropes: Milrinone plus Dopamine or Noradrenaline
haemodynamics: age adjusted, in neonates: SBP > 60mmHg, MAP >40mmHg, increasing
over time, LAP 8 12mmHg, CVP 8 12mmHg)
respiratory: normoxaemia, normocapnea
fluid restriction: 1ml/kg/hr, trophic feeds
haemostasis
keep normothermia (except in JET cool to 35C)
Specific Problems:
low cardiac output state: expect 6 - 12 hrs post bypass, mainly diastolic dysfunction
Arrthymias: JET; decrease inotropes, overdrive pacing, add noradrenaline, cooling,
Amiodarone (@ Arrhythmia)
diastolic RV dysfunction: restrictive physiology, usually transient phenomena lasting 48 72hrs. may require inotropes for improving of diastolic dysfunction.
residual RVOTO: ECHO confirmation +/- reoperation
pulmonary regurgitation almost always develops, may improve as diastolic dysfunction
improves
residual VSD: as above
Outcome:
Perioperative; 3 - 8%. Long term survival 85 - 90%.
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Tetralogy of Fallot with and without pulmonary atresia
[2] Lancet, 2009; 374: 1462-71: Apitz et al: Tetralogy of Fallot
[3] World J Surg, 34: 658-668; 2010: Starr J: Starr et al: Tetralogy of Fallot: Yesterday and Today.
[4] DVD 2004: Something the Lord made (Alan Rickman, Mos Def)
[5] Pediatr Cardiol. 2013 Mar 5. Dodgen et al: Characteristics and Hemodynamic Effects of Extubation Failure in Children
Undergoing Complete Repair for Tetralogy of Fallot.

CARDIAC DEFECTS: TRICUSPID ATRESIA

Definition: complete obstruction RA RV (muscular TA, membranous, valvular TA and @

Ebsteins anomaly) with presence of PFO / ASD, VSD, and increased MV annulus
hypertrophic LV, hypoplastic RV
Physiology: depends on position of great arteries and VSD physiology: 60% normal great
arteries with restrictive VSD and pulmonary stenosis or atresia causes decreased PBF and
cyanosis (PBF depending on VSD and PDA), 10% normal great arteries with unrestrictive
VSD causes increased PBF and CCF, 30% with D-TGA (10% with pulmonary stenosis or
atresia, 20% without pulmonary stenosis)
Diagnosis: ECG (left axis, LVH, P tricuspidale), ECHO, [Angiography]
Management preoperatively:
with decreased PBF:
PGE1 infusion (20ng/kg/min) to maintain PDA patency
BAS or Blade Septostomy in older children if ASD restrictive
Intubation and ventilation, hence lowering PVR
aim for SpO2 75 85%
with increased PBF:
anticongestive treatment
aim for SpO2 75 85%
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.

Surgery: dependant on anatomical associations and PBF:

with decreased PBF: BT-Shunt, modified BT Shunt in newborns
with increased PBF: PA Banding (careful in subaortic obstruction !) or Damus-Kaye-Stansel
(end-to-side anastomosis PA to the ascending aorta) for patients with LVOTO; in older
children: bidirectional Glenn Shunt or Total-cavopulmonary connection (Fontan)
in selected cases Bjork procedure
Postoperative Management for Tricuspid-Atresia:
@ BT-Shunt
@ Glenn Shunt
@ Fontan Circulation
Postoperative Management for TA and PA-Banding:
commence morphine sedation, paralyze with cis-atracurium for 12hrs until pulmonary and
systemic blood flow have balanced (discuss specifically at handover plan)
respiratory: SpO2 75 85%, may need some time to settle pulmonary blood flow and
achieve stable saturations
inotropes: usually not required
haemodynamics: SBP > 60mmHg, MAP > 40mmHg, increasing over time, LAP 8
12mmHg, CVP 8 12mmHg)
fluid restriction: 3 ml/kg/hr, early feeds if stable
haemostasis
Specific Problems:
persistent low SpO2 indicate a too tight PA band (Differential Diagnosis: Hypovolaemia,
low CO @ LCOS)
persistent high SpO2 indicate a too loose band child is growing into it; manipulate Qp:Qs
by pH (Acidosis), pO2(decrease FiO2), pCO2(mild hypercapnea), systemic vasodilators
(SNP)
Outcome:
depending on the underlying lesion; average PICU stay: 2 days
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Tricuspid Atresia
[2] Surg Gynecol Obstet 1952;95:213: Muller et al: The treatment of certain congenital malformations of the heart by the
creation of pulmonic stenosis to reduce pulmonary hypertension and excessive pulmonary blood flow: a preliminary report.
[3] Am Heart J. 1992 Mar;123(3):758-67. Chopra P et al: Corrective surgery for tricuspid atresia: which modification of FontanKreutzer procedure should be used? A review.

CARDIAC DEFECTS: PERSISTENT TRUNCUS ARTERIOSUS

Definition: single arterial trunk, overriding VSD, arising from normally formed ventricles.
Absent or diminutive PDA. Classification by Collett and Edwards or Van Praagh in regards to
position of PAs. 100% mortality within the first year if untreated. Prevalence 3% in CHD.
Incidence 5 15 : 100.000
Physiology: parallel circulation. With decreasing PVR increased PBF and increased flow
to LA and LV CCF
Diagnosis: presents usually 1 2weeks postnatally (decreasing PVR) and subsequently
CCF with failure to thrive, dyspnea, diaphoresis. Echo, in selected cases, Cardiac
Catheterization
Preoperative management:
balanced circulation (aim for SpO2 75 85%)
Hypoventilation to higher PVR and to lower SVR, SNP (0.5 4mcg/kg/min to lower SVR aim MAP > 35mmHg in neonates). Intubate and sedate to lower oxygen consumption.
Preoperative Preparation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, FISH, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.
Surgery: Timing: detaching of the PAs, ventriculotomy to close the VSD, valved RV-PA
conduit. In severe truncal valve insufficieny aortic homograft with reimplantation of
coronary arteries

Postoperative Management:
@ Transposition of great arteries
keep intubated, ventilated, sedated and paralysed for 24 hours
inotropes: Milrinone plus Dopamine or Norepinephrine, aim MAP > 40mmHg in neonates)
haemodynamics: age adjusted, in neonates: SBP > 60mmHg, MAP > 40mmHg, increasing
over time, LAP 8 12mmHg, CVP 8 12mmHg)
respiratory: normoxaemia, normocapnea
fluid restriction: 1 ml/kg/hr, trophic feeds
haemostasis
keep normothermia
Specific Problems:
low cardiac output state: expect 6 - 12 hrs post bypass
Pulmonary Hypertension crisis: especially in neonates @ Pulmonary Hypertension
Arrthymias: JET decrease inotropes, overdrive pacing, add Noradrenaline, cooling,
Amiodarone (@ Arrhythmia)
diastolic RV dysfunction: restrictive physiology, usually transient phenomena lasting 48
72 hrs
residual truncal valve insufficiency: mild to moderate is usually well tolerated, however if
severe may need reinvestigation
Outcome:
Perioperative Mortality: 10%. Long term survival after 5, 10, 15 years: 90%, 85%, 83%
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Persistent Truncus Arteriosus
[2] Surg Clin North Am. 1949 Aug;29(4):1245-70: Collett et al: Persistent truncus arteriosus; a classification according to
anatomic types.
[3] Am J Cardiol. 1965 Sep;16(3):406-25: Van Praagh et al: The anatomy of common aorticopulmonary trunk (truncus
arteriosus communis) and its embryologic implications. A study of 57 necropsy cases
[4] Cardiol Young. 2005 Feb;15 Suppl 1:125-31: Backer et al: Techniques for repairing the aortic and truncal valves
[5] Cardiol Young. 2012 Dec;22(6):755-60. Shamszad et al: Intensive care management of neonates with d-transposition of the
great arteries and common arterial trunk.

CARDIAC DEFECTS: VENTRICULAR SEPTAL DEFECT (VSD)

Definition: interventricular septal communication, defined by their location, whereas the IVS
can be divided into a muscular and membranous portion, hence perimembranous VSD
(80%) or muscular VSD (inlet / trabecular / outlet = infundibular region); most common CHD:
incidence: 1.7 53 : 1000 of all live birth; Prevalence: 5% in lifeborn babies; Frequency:
20% as a single lesion, 50% as part of lesion in CHD, total 40% of all CHD
Physiology: depending on size, PVR, RVP and LVP and eventual prolapse of aortic valve
and/or obstruction of the pulmonary or systemic outflow tract in neonates with increased
PVR minimal shunting, while drop in PVR and as more unrestrictive left to right shunt
volume load LA and LV CCF if long standing Eisenmenger Syndrome
Diagnosis: Auscultation: holosystolic murmur, cyanosis and clubbing in Eisenmenger
syndrome, ECG (LVH), CXR (increased PBF, in Eisenmenger: reduced PBF), ECHO (size,
location, shunt, haemodynamic evaluation), MRI, Cardiac Catheterization (PVR, response to
pulmonary vasodiltators, Qp : Qs in older patients)
Preoperative Management:
treat CCF: CPAP / IPPV if required: (Digoxin), Frusemide (1mg/kg up to QID),
Spironolactone (1mg/kg BD), aim for afterload reduction: Milrinone 0.25 0.5mcg/kg/min or
Captopril (0.1 2mg/kg TDS)
Preoperative Preperation:
ECG, CXR, CUS, FBE, Clotting, U&C, Electrolytes, PRBC (4), FFP (2), Platelets (2),
Cryoprecipitate (2). Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.

Surgery: PAB (@ TA) for protection of pulmonary circulation in neonate with multiple VSD
or apical VSD and CCF adjustable PAB possible; in children > 3mths (Qp : Qs > 1.5 : 1).
surgical closure via transatrial approach, sometimes by right ventriculotomy, rarely by left
apical ventriculotomy. Catheter closure by Amplatzer device. Heart-Lung Transplantation in
Eisenmenger Syndrome (Bosentan ?)
Postoperative Management:
inotropes: Milrinone plus Dopamine or Adrenaline (plus Noradrenaline, aim MAP >
40mmHg in neonates)
haemodynamics: age adjusted, in neonates: SBP > 60mmHg, MAP > 40mmHg, increasing
over time, CVP 8 12mmHg)
respiratory: normoxaemia, normocapnea
fluid restriction: 1ml/kg/hr, early feeds
continue preoperative diuretic therapy
haemostasis
if Catheter closure: Aspirin 5mg/kg OD once feeds tolerated
Specific Problems:
risk of PHT (@ Pulmonary Hypertension) in smaller children and Qp : Qs > 2 : 1 preop`
risk of PHT (@ Pulmonary Hypertension) in older children and Qp : Qs > 1.5 : 1
risk of pulmonary edema with pre-OP high Qp : Qs Diuresis, moderate PEEP
arrhythmia: complete heart block, SVT, JET (@ Arrhythmia)
Outcome:
Average ICU stay: 2days; mortality < 1% in isolated VSD, but up 5 10% in multiple defects;
residual shunts in 30% after surgical closure, majority closes spontaneously
[1] Br Heart J. 1980 Mar;43(3):332-43: Soto et al: Classification of ventricular septal defects
[2] Circulation. 2006 Nov 14;114(20):2190-7: Minette et al: Ventricular septal defects
[3] Cardiol Young. 2007 Jun;17(3):243-53: Butera et al: Percutaneous closure of ventricular septal defects
[4] Front Pediatr. 2013 Jul 31;1:16. Corno A et al: Multiple ventricular septal defects: a new strategy.
[5] Lancet. 2011 Mar 26;377(9771):1103-12. Penny et al: Ventricular septal defect

HEART FAILURE: CARDIOMYOPATHY [CM] - (DILATIVE DCM / HYPERTROPHIC HCM)

Incidence: DCM 0.6 : 100.000, HCM 0.5 : 100.000, Causes: DCM (66% idiopathic, other:
myocarditis, neuromuscular disorder, familial, inborn errors of metabolism), HCM (74%
idiopathic, other: malformation, inborn errors of metabolism, neuromuscular disorder)
Basic Investigation: ECG, CXR, ECHO, FBE, Clotting, U&C, Electrolytes (incl. Ca++, Mg++,
Fe++, PO4---), CRP, ESR, Albumin, LFT, TFT, BNP, Troponin I, Troponin T, Lactate, ABG,
VBG.
Extended Investigation: Cardiac MRI. 24hr Holter Monitor. Blood: Amino acids, Carnitine,
Acyl-Carnitine, Ammonia, Cu, Caeruloplasmin, Transferrin-Isoforms, Pyruvate, Selenium,
Vitamins. Urine: Amino acids, Organic acids, Oligosaccharide screen, MPS screen.
Autoimmune: ANA, ENA. Genetic: FISH. Endomyocardial Biopsy.
Investigation for Myocarditis: Blood & Urine for viral cultures (Echo, Adeno, Parvo,
Coxsachie, CMV, Parainfluenza, Influenza, HIV, Hepatitis screen) and for bacterial (incl.
atypical) / fungal / rickettsial / protozoal and helminithic culture. NPA. ETA.
Identifying the High Risk Patient: LVEF < 20% or LVEDD > 70mm or increased C/T ratio
> 0.7 or MR > 3 or complex ventricular arrhythmia (NSVT).
Acute Management Guideline for DCM

NO

Ward Management
ACEi
Blocker
oral diuretics

YES

Is there low perfusion ?

Is there congestion ?
NO

PICU admission
NIV CPAP
iv Adrenaline
(max. 0.05 microg/kg/min)
observe closely
for need for ECLS

YES
PICU admission
iv Frusemide infusion
consider NIV CPAP
observe for minimum of 48 hr
watch BNP & renal function
PICU admission
iv Frusemide infusion
consider NIV CPAP
introduce carefully Milrinone
consider agonists
observe for 4 days

1. CPAP or BIPAP. Only intubate when ECLS is on Stand-By

2. Fluid and Water Restriction to 25% maintenance
3. Commence iv Frusemide infusion. Aim for euvolaemic state in DCM. Mild
hypervolaemia may be needed in HCM. Watch renal function. Avoid hypovolaemia.
4. Commence Spironolactone (1mg/kg BD)
5. Commence Milrinone if tolerated
6. Commence -agonists as indicated. Dopamine, Dobutamine or Adrenaline up to
0.05mcg/kg/min
7. Consider Levosimendan
8. Consider biventricular pacing
9. Consider antiarrhythmic therapy. Consult cardiology. Do not use -blocker when on
agonists. - Blocker might be useful in HCM
10. Consider anticoagulation therapy
11. Ensure appropriate nutrition. Supplemental therapy with carnitine, co-enzyme Q10,
vitamins as indicated.
11. Early use of ECLS if patient is deteriorating or not improving @ ECMO or @ VAD
12. specific guided therapy in Pompe disease: myozyme

HEART FAILURE: MYOCARDITIS

See @ Cardiomyopathy, but cardiac MRI or Endomyocardial Biopsy to confirm Diagnosis
Symptoms: Very nonspecific in children: malaise, fever, poor appetite, tachypnea,
tachycardia, chest pain, abdominal pain, myalgia, fatigue, cough, oedema, hepatomegaly,
murmur.
Investigations: See also cardiomyopathy. Nonspecific T changes on ECG. Cardiac
enzymes are not elevated in most patients with myocarditis. Echo is mandatory to assess
function (systolic and diastolic dysfunction).
Treatment:
1. Symptomatic: see @ Cardiomyopathy
2. IVIG: early, high dose IVIG: 2g/kg over 24hrs
3. in chronic DCM: consider Interferon with persistent viral genomes
Common Differential Diagnosis of CM in regards to age
< 1 year Myocarditis, Endocardial Fibroelastosis, Barth Syndrome, Carnitine Deficiency,
Selenium Deficiency, Anomalous Left Coronary Artery, Kawasaki Disease, Critical Aortic
Stenosis, Supraventricular Tachycardia, Arterio-Venous Malformation, Calcium Deficiency,
Hypoglycemia, Left Ventricular Non-Compaction, Mitochondrial Cardiomyopathy, Nemaline
Myopathy, Minicore-Multicore Myopathy, Myotubular Myopathy
> 1 year and < 10 years Familial DCM, Barth Syndrome, Myocarditis, Arrhythmogenic RV
dysplasia, Endocardial Fibroelastosis, Carnitine Deficiency, Selenium Deficiency,
Anomalous Left Coronary Artery, Kawasaki Disease, Supraventricular Tachycardia, Toxic
(Adriamycin), Ketothiolase Deficiency, Ipecac Toxicity, Systemic Lupus Erythematosis,
Polyarteritis Nodosa, Haemolytic-Uremic Syndrome, Mitochondrial Cardiomyopathy,
Nemaline Myopathy, Minicore-Multicore Myopathy, Myotubular Myopathy
> 10 years Familial DCM, X-linked DCM, Myocarditis, Supraventricular Tachycardia,
Congenital Heart Disease, Mitochondrial CM, Chagas Disease, Arrhythmogenic RV
dysplasia, Eosinophilic Cardiomyopathy, Toxic (Adriamycin), Phaeochromocytoma,
Duchenne/Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy,
Hemochromatosis, Limb-Girdle Muscular Dystrophy, Myotonic Dystrophy, Peripartum
Cardiomyopathy, Alcoholic Cardiomyopathy
[1] Academic Emergency Medicine, 2008, 15, 355 362: Tallmann et all: Noninvasive ventilation outcomes in 2430 acute
decompensated heart failure patients: an ADHERE registry analysis
[2] European Journal of Pediatrics, 2010, 169, 269 279: Kantor et all: Clinical Practice: Heart Failure in Children. Part I.
clinical evaluation, diagnostic testing and initial management
[3] American Journal of Medicine, 2006, 119, S37 S44: Hill et all: Beyond diuretics: management of volume overload in acute
heart failure syndromes
[4] New England Journal of Medicine, 1999, 341, 709 717: Pitt et all: The Effect of Spironolactone on Morbidity and Mortality
in Patients with Severe Heart Failure
[5] Circulation. 2003;107: 996 1002: Hoffmann et all: Efficacy and Safety of Milrinone in Preventing Low Cardiac Output
Syndrome in Infants and Children After Corrective Surgery for Congenital Heart Disease
[6] Journal of Intensive Care Medicine, 2006, 21, 183 187: Egan et all: Levosimendan for Low Cardiac Output: A Pediatric
Experience
[7] Current Cardiology Reviews, 2009, 5, 40 44: S. Batra et all: Cardiac Resynchronization Therapy in Children
[8] The Journal of Pediatrics, 2001, 138(4), 457-458: Bruns et all: Carvedilol as therapy in pediatric heart failure: An initial
multicenter experience
[9] New England Journal of Medicine, 2006, 355, 1873 1884: Birks et all: Left ventricular assist device and drug therapy for
the therapy of heart failure
[10] Circulation, 2004, 109, 1250 1258: Mahrholdt: Cardiovascular MRI assessment of human myocarditis: a comparison to
histology and molecular pathology
[11] Circulation, 1994, 89, 252-257: Drucker et all: Gamma-globulin treatment of acute myocarditis in the pediatric population
[12] Circulation, 2003, 107, 2793 2798: Kuhl et all: Interferon-beta treatment eliminates cardiotropic viruses and improves left
ventricular function in patient with myocardial persistence of viral genomes and left ventricular dysfunction.
[13] Heart Fail Clin. 2010 Oct;6(4):401-13. Wilkinson et al: The pediatric cardiomyopathy registry and heart failure: key results
from the first 15 years.
[14] Curr Opin Cardiol. 2010 Mar;25(2):80-7. Silva et al: Current management of pediatric dilated cardiomyopathy.

HEART FAILURE: ECMO

ExtraCorporal Membrane Oxygenation: for cardiovascular or respiratory or combined
cardiovascular
Inclusion criteria: > 34 / 40 weeks gestation age, reversible cardiac, pulmonary, or
cardiopulmonary failure, mechanical ventilation < 14 days.
Exclusion criteria: major intracranial hemorrhage, lethal malformation, severe neurological
injury, untreatable cardiac or pulmonary malformation.
Clinical Indications: failure to wean off cardiopulmonary bypass, Oxygenation Index > 40
on two or more ABG despite maximum therapy [OI = (MAP * FiO2 * 100) / PaO2], intractable
metabolic acidosis, progressive intractable pulmonary or cardiac failure
Flow rates on full VA ECMO support:
in patients < 10kg aim for 100 - 150ml/kg/min
in patients > 10kg aim for CI 2.4L/min/m2
consider higher flow rates in septic patients, univentricular hearts with open systemicpulmonary shunts and extracardiac shunts
Flow rates on full VV ECMO support:
in patients < 10kg aim for 70 - 140ml/kg/min
in patients > 10kg aim for CI 1.8L/min/m2
consider higher flow rates in septic patients, univentricular hearts (cave: inappropriate high
SmvO2 might indicate recirculation)
Cannulation: ABG, FBE, Coags, Urea & Creatinine & Electrolytes, Ca++, Mg++, LFT, SBR,
ABG, obtain blood culture / urine for mcs / ETT aspirate for mcs, optimize coagulation if
possible, consider blood sample storage for genetic analysis, Cephazolin (50mg/kg iv) 30 60min prior to procedure, CXR, ECHO, cranial Ultrasound, arterial line placed and secured,
central line placed (not RIJ or R Subclavian) and secured, chest drain placed, if required and
secured, position child: neck cannulation: position patient head outwards in bed space and
neck overextended with roll under shoulders / transthoracic cannulation: supine position with
roll under back, 2 x venous line extension, Fentanyl 5mcgg/kg iv bolus, Vecuronium
0.1mg/kg iv, consider fluid resuscitation / enhance inotropic support if needed, surgical
preparation, give Heparin on surgical request (50 - 100U/kg if appropriate for pt situation),
surgical cannulation & connection of tubing (< 15kg , > 15kg 3/8 circuit), set FiO2
100% and sweep gas flow, turn RPM to 1000 - 1200, unclamp venous line, unclamp arterial
line, increase slowly RPM to desired flow, reduce inotropic / vasoconstrictor accordingly,
observe ABP / inlet pressure / outlet pressure / CVP, recheck ACT every 30min, commence
Heparin infusion 20U/kg/hr once ACT < 250, once on full flow, change to ventilation rest
settings (PEEP 10, PS + 10, Vt 6ml/kg, RR 10, FiO2 30 40% in VA ECMO FiO2 60% in VV
ECMO), secure cannula position, commence analgesia & sedation & paralysis, CXR, ECHO,
fluid restriction to 60%.

Anitcoagulation: Keep ATIII level > 80%: No. IU = (desired actual level) X Wt, Heparin
[Patients < 10kg: 5 KU / 50 ml 0.9% NaCl, Patients > 10kg: 25 KU / 50 ml 0.9% NaCl]
Commence Heparin on 20U/kg/hr, adjust in regards to ACT.
ACT
<160
160-180
180-200
200-220
220-240
240-260
260-280

Bolus (U/Kg)
50
30
20
0
0
0
0

% rate change
+15%
+10%
+10%
0
-10%
-10%
-10%

Weaning in VA ECMO: ensure volume status is adequate, ventilate patient with acceptable
settings (as blood flow through lungs increased), decrease pump flows by 10ml/min every
60min down to a minimum of 40ml/kg or 250ml/min total flow through oxygenator, ABG
15min post each wean of flow, ECHO at lower flows, [do NOT turn sweep gas off all flow
going through circuit bypasses lungs. Minimum sweep gas setting is 200ml/min], bridge or
decannulate
Weaning in VV ECMO: commence full ventilation, sweep gas FiO2 at 0.21 for approx 10
minutes to flush O2 from oxygenator, turn sweep gas to minimum setting of 200 ml/min, run
ACTs 200-220 whilst pt still on ECMO circuit, observe patient saturation, ABG after 30min
(oxygenator continues to oxygenate for approx 20min after sweep gas flow is ceased),
organize for decannulation
[1] Cardiol Young, 2007; Sep;17 Suppl 2:104-15: Cooper et al: Cardiac extracorporeal life support: state of the art in 2007
[2] http://www.elsonet.org
[3] Lancet, 1996 Jul 13; 348(9020):75-82: UK collaborative randomised trial of neonatal extracorporeal membrane oxygenation.
UK Collaborative ECMO Trail Group
[4] Cochrane Database Syst Rev. 2008 Jul 16;(3):CD001340: Mugford et al: Extracorporeal membrane oxygenation for severe
respiratory failure in newborn infants.
[5] Lancet. 2009 Oct 17;374(9698):1351-63: Peek et al: Efficacy and economic assessment of conventional ventilatory support
versus extracorporeal membrane oxygenation for severe adult respiratory failure (CESAR): a multicentre randomised controlled
trial.
[6] Intensive Care Med (2012) 38:210-220: MacLaren et al: Contemporary extracorporeal membrane oxygenation for adult
respiratory failure: life support in the new era
[7] Artif Organs. 2013 Jan;37(1):21-8. Kotani et al: Evolution of technology, establishment of program, and clinical outcomes in
pediatric extracorporeal membrane oxygenation: the "sickkids" experience.
[8] Ped Critical Care: June 2013 - Volume 14 - Supplement 1 5_suppl: MacLaren et al: Joint Statement on Mechanical
Circulatory Support in Children: A Consensus Review from the Pediatric Cardiac Intensive Care Society and Extracorporeal
Life Support Organization

HEART FAILURE: ANTIBIOTICS FOR ECMO PATIENTS

Indication

Prophylaxis

ECMO (cannulation,
chest revision,
reoperation on ECMO,
decannulation) if not on
anitbiotics with both
gramnegative and
grampositive cover
already

Cephazolin 50mg/kg up to
1g IV
or (if Cephazolin
unavailable)

Penicillin / Cephalosporin
allergy

plus
Vancomycin 25mg/kg up
to 1.5g (child < 12yrs:
30mg/kg up to 1.5g)
Vancomycin 25mg/kg up
to 1.5g (child < 12yrs:
30mg/kg up to 1.5g) IV
plus
Gentamicin 2mg/kg IV

ECMO (cannulation,
chest revision,
reoperation on ECMO,
decannulation) if on
anitbiotics with both
gramnegative and
grampositive cover
already

Duration

Optimal timing for BetaLactams: administer 30

60min before incision

2 dose 25mg/kg if
operation > 3hrs, continue
25mg/kg 8hrly, remember
to cease after 24hrs

Optimal timing for BetaLactams: administer 30

60min before incision; for
Vancomycin: slow
infusion, starting 60min
before, and finishing
immediately before
incision, CVL not required

2 dose 25mg/kg if
operation > 3hrs, continue
25mg/kg 8hrly, remember
to cease after 24hrs; no
further doses of
Vancomycin required

Optimal timing for BetaLactams: administer 30

60min before incision; for
Vancomycin: slow
infusion, starting 60min
before, and finishing
immediately before
incision, CVL not required

No further doses required

n/a

n/a

nd

Cephalothin 50mg/kg up
to 2g IV
Cephazolin 50mg/kg up to
1g IV

Known MRSA infection or

colonization, currently or
in the past

Timing

No further prophylaxis
required

nd

[1] Therapeutic Guidelines: Antibiotic, 2006: Therapeutic Guidelines Ltd, Melbourne.

[2] Ann Thorac Surg 2006, 81:397-404: The Society of Thoracic Surgeons Practice Guideline Series: Antibiotic Prophylaxis in
Cardiac Surgery, Part 1: Duration.
[3] The Society of Thoracic Surgeons Practice Guideline Series: Antibiotic Prophylaxis in Cardiac Surgery, Part 2: Antibiotic
Choice. Ann Thorac Surg 2007;83:1596-76.

HEART FAILURE: HEART FAILURE AND VAD

Modified Ross Heart Failure Classification
Class
I
II
III
IV

Symptoms
Asymptomatic
mild tachypneoa or diaphoresis with feeding
Dyspnea on exertion in older children
marked tachypnea or diaphoresis with feeding; marked
Dyspnea on exertion
prolonged feeding time with failure to thrive
Tachypnea, retractions, grunting or diaphoresis at rest

Causes: congenital, skeletal myopathy with cardiac involvment (eg Duchenne, Becker,
Barth Syndrome, Myotonic Dystrophy), metabolic disorders with cardiac involvement
(Carnitine deficiency, Glykogen Storage disease, mitochondrial disease), Cardiomyopathy
(primary, secondary) @ Cardiomyopathy, acquired (rheumatic heart disease, myocarditis @
Myocarditis, Tachyarrythmia, toxic, antineoplastic drugs, nutritional deficiency)
Treatment: Treatment of the underlying cause and @ Cardiomyopathy
Mechanical Treatment Options (ECLS):
Short term support: IABP (diastolic augmentation), Impella axial flow (minimum weight
25kg), centrifugal pumps (Levitronix), ECMO @ ECMO
Mid Term support / Bridge to transplantation / Bridge to recovery: Thoratec, @ Berlin
Heart
[1] European Journal of Pediatrics, 2010, 169, 403 410: Kantor et all: Clinical Practice: Heart Failure in Children. Part II.
Current maintenance therapy and new therapeutic approaches
[2] Circulation 110:975981: Stevenson LW: Left ventricular assist device as destination for patients undergoing intravenous
inotropic therapy: a subset analysis from REMATCH (Randomized Evaluation of Mechanical Assistance in Treatment of
Chronic Heart Failure).
[3] Ann Thorac Surg 1999; 67: 1415-20. Akomea-Agyin: Intraaortic balloon pumpi in children.
[4] J. Thorac. Cardiovasc. Surg., July 1, 2011; 142(1): 60 65. Lamarche et al: Comparative outcomes in cardiogenic shock
patients managed with Impella microaxial pump or extracorporeal life support
[5] John et al. J Thorac Cardiovasc Surg.2011; 141: 932-939. Outcomes of a multicenter trial of the Levitronix CentriMag
ventricular assist system.
[6] J Heart Lung Transplant 2001; 20: 439-48.Reinhartz O, Keith FM, El-Banayosy A, et al. Multicenter experience with the
thoratec ventricular assist device in children and adolescents.
[7] Current Cardiology Reviews, 2010, 6, 46-53. Gazit et al: Mechanical Circulatory Support of the Critically Ill Child Awaiting
Heart Transplantation.
[8] J Heart Lung Transplant. 2012 Feb;31(2):117-26. Kirklin et al: The Fourth INTERMACS Annual Report: 4,000 implants and
counting.
[9] Interact Cardiovasc Thorac Surg. 2012 Sep;15(3):426-31. The profile of the systemic inflammatory response in children
undergoing ventricular assist device support.

HEART FAILURE: BERLIN HEART VAD EXCOR

Definition: paracorporal, pneumatically driven, pulsatile flow mechanical support device
driven by a central driving unit (Ikus) and different sizes of blood pumps (10, 25, 30, 50, 60,
80ml), can be used as RVAD, LVAD or BIVAD.
Aim: Bridge to Transplant or Bridge to Recovery
Standard Settings:
Chamber Size
10 ml
25 ml
30 ml
50 ml
60 ml
80 ml

Driving pressures for Systole and Diastole

Systole (LVAD / RVAD)
Diastole (LVAD / RVAD)
225 / 175
- 50 / - 50
175 / 150
- 50 / - 50
175 / 150
- 50 / - 50
175 / 150
- 25 / - 25
200 / 150
- 25 / - 25
225 / 175
- 25 / - 25

Anticoagulation guide:

start UFH
+ > 24hr post OP
+ no bleeding
+ platelets > 20.000
+ normal TEG
start LMWH
+ if age < 12months
+ creatinine normal
+ no bleeding
or if
+ unable to tolerate PO
+ unstable INRs
+ convert after UFH and no bleeding
start Vitamin K Antagonist
(bridge with LMWH)
+ if age > 12month
+ enteral feeds tolerated
start Platelet inhibitors
Dipyramidole
+ if Platelets > 40.000
+ postop Day 2 and
ADP > 50%
and Aspirin
+ if platelets > 40.0000
+ postop Day 4 and drains removed
and ARA > 50%

Medications / Dosing / Target

start with
a) < 12month:
15 U/kg/hr (no bolus) and increase after
6hrs to 28U/kg/hr
b) >12month:
10U/kg/hr (no bolus) and increase after
6hrs to 20U/kg/hr
aim ATIII > 70%
start with Enoxaparin
a) < 3month: 1.5mg/kg BD
b) > 3month: 1mg/kg BD
or if low INR 2.0 2.7: 1mg/kg/ OD
or if low INR < 2.0:
1mg/kg BD
aim ATIII > 75%

aPTT 1.5 2.5 of normal

(check every 6hrs)
or Anti-Xa 0.35
0.50U/ml (draw level
nd
4hrs after 2 dose)

Anti-Xa 0.6 1.0U/ml

th
nd
(draw level 4 after 2
dose until stable)

Warfarin 0.2mg/kg/day
(maximum 5mg/day)

INR 2.7 3.5

(use LMWH if unstable
INRs)

Dipyramidole 1mg/kg/dose QID

(maximum 15mg/kg/day)

ADP activity < 50%

Aspirin 1mg/kg/day

ARA activity < 30%

Trouble Shooting: always inform PICU consultant for any changes !

Insufficient filling of VAD (VAD Diastole)

check Hb and drain losses replace volume
check CXR (Pneumothorax ?), Ventilation settings aim for early
changes in intrathoracic pressure
extubation to increase CO (negative impact of positive intrathroacic
pressure)
Tamponade
ECHO warranted, inform surgeon ASAP
lower PVR @ Pulmonary Hypertension, check right heart function on
increased PVR
ECHO
check for mechanical obstruction: extracorporal / intracorporal
Kink in inflow cannula
(ECHO)
right heart failure
check right heart function with ECHO inotropic support of right
(LVAD only)
heart (Dobutamine, Milrinone), @ NO, RVAD
increase negative vacuum pressure (be careful not to suck air in)
too low negative vacuum pressure
Driving pressures
VAD rate too high
lower VAD rate, decrease % systole
Hypovolaemia

Insufficient emptying of VAD (VAD Systole)

increased PVR (in RVAD)
lower PVR @ Pulmonary Hypertension
increased SVR (in LVAD)
lower SVR @ Vasodilators
Kink in outflow cannula
check for mechanical obstruction
systolic drive pressure too low
increase systolic driving pressure ( Driving pressures)
[1] J of Cardiovasc Trans Res (2010) 3:612-617: Bryant 3rd: Current Use of the EXCOR Pediatric Ventricular Assist Device
[2] Artif Organs. 2010 Dec;34(12):1082-6: Humpl et al: The Berlin Heart EXCOR Pediatrics-The SickKids Experience 20042008
[3] Ann Thorac Surg. 2005 Jan;79(1):53-60; discussion 61: Groetzner et al: Cardiac transplantation in pediatric patients: fifteenyear experience of a single center
[4] J Heart Lung Transplant. 2009 Apr;28(4):399-401. Irving et al: Successful bridge to transplant with the Berlin Heart after
cavopulmonary shunt
[5] Am Heart J. 2011 Sep;162(3):425-35.Almond et al: Berlin Heart EXCOR Pediatric ventricular assist device Investigational
Device Exemption study: study design and rationale
[6] J Thorac Cardiovasc Surg. 2011 Mar;141(3):616-23, 623: Hetzer et al: Single center experience with treatment of
cardiogenic shock in children by pediatric ventricular assist devices
[7] Artif Organs 2012 Jul;36(7):635-9: Sharma et al: Ventricular assist device support in children and adolescents with heart
failure: the Childrens Medical Center of Dallas experience
[8] Ann N Y Acad Sci. 2013 Jul;1291:96. Fraser et al: The Berlin Heart EXCOR Pediatric ventricular assist device: history,
North American experience, and future directions.
[9] Paediatr Anaesth. 2010 Sep;20(9):812-20. Pratap et al: Anesthetic management of children with in situ Berlin Heart
EXCOR.
[10] J Thorac Cardiovasc Surg. 2014 Feb;147(2):697-705. Weinstein et al: The use of the Berlin Heart EXCOR in patients with
functional single ventricle.

HEART FAILURE: HEART TRANSPLANTATION (HTX)

Indication: life expectancy < 2years and/or unacceptable quality of life, end stage CHD,
DCM, HCM (@ Cardiomyopathy)
Risk Profile: PVR (low risk: PVR 4WU or TPG 10mmHg, medium risk: PVR 5 9 WU or
TPG 10 20mmHg, high risk/contraindicated: PVR > 9WU or TPG 15mmHg. In high
risk patients: trial with pulmonary vasodilator in Cardiac Cath (NO, Prostacycline). Donor:
Size mismatch up to 4:1, good systolic function (EF > 50%), Serology for EBV, CMV, HIV,
HTLV, Hepatitis, Syphilis, Toxoplasmosis
Contraindication: Recipient (MDT decision), metastatic incurable neoplasm, severe
Sepsis, fixed PHT (consider Heart-Lung Tranplantation). Donor: Aids, HTLV Infection or
Hepatitis B Antigen positive.
Preoperative Preparation:
ECG, CXR, FBE, Clotting, U&C, Electrolytes, BNP, LFTs, ABO, HLA, CMV, EBV, HSV, HIV,
VZV, Measles, Hepatitis serology, ECHO, Cardiac Cath (PVR, TPG), Angio CT, MRI, V/Q
scan
Surgery: Previous biatrial, now commonly bicaval technique

Postoperative Management:
keep intubated, ventilated, sedated for 24hrs, (longer with open chest @ Open Chest)
inotropes: Dobutamine or Isoprenaline, Milrinone plus Adrenaline (despite denervation the
donor heart responds well to exogenous inotropes), SNP for increased SVR. Consider
potential combination of Milrinone and Adrenaline 0.05mcg/kg/min
haemodynamics: age donor / recipient adjusted. Early recovery systolic function. Diastolic
function longer impaired (Milrinone)
respiratory: normoxaemia, normocapnea, may consider NO for RV afterload reduction (@
NO)
fluid restriction: 1ml/kg/hr
haemostasis
Antibiotic prophylaxis until drains removed. PJP prophylaxis. Ganciclovir if donor
CMV positive/recipient negative
Immunosuppression: Methylprednisolone 15 - 20mg/kg/dose BD for 2days,
Thymoglobulin 1.5mg/kg/dose OD for 5days or Basiliximab, consider IVIG 0.4g/kg/dose OD
for 5days, Calci-neurininhibitor: Cyclosporine or Tacrolimus (0.05mg/kg/dose BD) adjusted
to level, Mycophenolate mofetil (MMF) 30mg/kg/dose BD or Azathioprine 3mg/kg/dose OD
adjusted to level
Specific Problems:
early graft failure: dominant left heart failure mechanical support, Retransplantation
right heart failure (especially in setting of preoperatively increased PVR/TPG): iNO (@
NO), Milrinone, Dobutamine or Adrenaline (@ Inotropes), consider mechanical support
low CO: keep paralysed, dont wean inotropes < 24hrs, pacing (infant 140bpm, adolescent
100bpm), consider mechanical assist
acute rejection (rare in the first 7 10days): LV dysfunction, arrhythmia. Diagnosis: Biopsy
shows lymphocytic infiltrates. Therapy: Methylprednisolone high dose
Longterm Morbidity & Mortality:
Renal failure, Cardiac Allograft Disease (CAD), Lymphoma, Neoplasia, PTLD (Post
Transplant Lymphoproliferative Disease; usually EBV related. Therapy: temporarily decrease
immunosuppression, (antiviral treatment), Retuximab
Outcome:
1y 90%, 5y 80%, 10y 70%
[1] Paediatric Heart Transplant Society: http://www.uab.edu/phts/
[2] Curr Cardiol Rev. 2011 May;7(2):72-84: Chinnock et al: Heart transplantation for congenital heart disease in the first year of
life.
[3] Eur J Cardiothorac Surg. 2012 Jun 24. Seddio et al: Is heart transplantation for complex congenital heart disease a good
option? A 25-year single centre experience.
[4] Curr Treat Options Cardiovasc Med. 2011 Oct;13(5):425-43: Gazit et al: Perioperative management of the pediatric cardiac
transplantation patient.
[5] Lancet. 2006 Jul 1;368(9529):53-69: Webber et al: Heart and lung transplantation in children.

HEART FAILURE: ABO INCOMPATIBLE HTX

UNOS Policy: ABO incompatible HTX for children up to 2years (with acceptable
isohemoglutinin titres less than 1:4)
Recipient
0

Donor
compatible
0

Donor
incompatible

AB

0
0
A
A

A
B

AB

RBC

AB

AB or A
AB or B

0
0

Anti A
Anti B
Anti A
Anti B

AB

A or 0

AB

A or 0

AB

B or 0

AB

B or 0

B
0

AB

AB
AB
AB
AB

Anti A
Anti B
Anti A
Anti B

Plasma /
Platelets
0

A
0

B
B

Antibodies to
avoid

AB
A
B
0

Anti A
Anti B
Anti A
Anti B

AB
AB
AB
AB

Preoperative Preparation:
Isohaemagglutinin titres. Avoid transfusion. Consider transfusion in regards to guide.
Perioperative Management:
if elevated Iso-titre: Plasma exchange once on CPB, Iso-haemagglutinin quick test before
AoCx release, repeat Plasma exchange as required.
Postoperative Management:
Isohaemaggluttinin titres daily of ABOi HTX. Repeat Plasma exchange if required
Outcome:
Similar long term outcome. Reduced rate of infection and rejection compared to ABO
compatible HTX
[1] Paediatric Heart Transplant Society: http://www.uab.edu/phts/
[2] Heart Lung Transplant. 2012 Feb;31(2):173-9: Henderson et al: ABO-incompatible heart transplantation: analysis of the
Pediatric Heart Transplant Study (PHTS) database.

RESUSCITATION DRUG CHART

[1] APLS, the pediatric emergency medicine resource,4e; Jones and Bartlett Publishers

RESUSCITATION QUICK CHART

AGE/WT

BP SYS

HR

RR

ETT

ETT lip/nose

DC Shock 4 J/kg

Fluid 20 ml/kg

Term / 3.5

50

100 - 180

40 - 60

3.0

8.5 / 10.5

14

70

3m/5

50

100 - 180

30 - 50

3.5

9.5 / 11

20

100

6m/8

60

100 - 160

30 - 50

4.0

10 / 13

32

160

1 y / 10

65

100 - 140

25 - 45

4.0

11 / 14

40

200

2 y / 13

65

80 - 130

20 - 30

4.5

12 / 15

50

260

5 y / 17

70

70 - 110

15 - 25

5.0

14 / 17

70

340

10 y / 30

85

60 - 105

15 - 20

6.0

17 / 21

120

600

14 y / 50

90

50 - 100

15 - 20

7.5

19 / 23

200

1000

17 y+ / 70

90

50 - 100

15 - 20

7.5

19 / 23

360

1000

Amiodarone
[150mg/3ml]

Dextrose 10% [ml]

Mannitol 20%
[ml]

0.4

0.3

15

7.5

3m/5

0.6

0.5

25

12.5

6m/8

0.8

0.8

40

20

1 y / 10

1.0

0.1

1.0

50

25

2 y / 13

1.5

0.2

1.3

65

32

5 y / 17

2.0

0.2

1.7

85

40

10 y / 30

3.0

0.3

3.0

150

65

14 y / 50

5.0

0.5

5.0

250

125

17 y+ / 70

10.0

1.0

7.0

500

250

AGE/WT

Epi 1:10000

Term / 3.5

Epi 1:1000

NEONATAL LIFE SUPPORT USA

[1] Circulation. 2010 Nov 2;122(18 Suppl 3):S909-19. Kattwinkel J et al: Part 15: neonatal resuscitation: 2010 American Heart
Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.

NEONATAL LIFE SUPPORT UK

[1] Resuscitation Council UK

Paediatric Advanced Life Support Australia

[1] APLS, the pediatric emergency medicine resource,4e; Jones and Bartlett Publishers
[2] Resuscitation Council UK

Pediatric Advanced Life Support USA

[1] Circulation. 2010 Nov 2;122(18 Suppl 3):S876-908. Kleinman et al: Part 14: pediatric advanced life support: 2010 American
Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.