Review Article

Cardiogenic Shock in Children

Manvinder Singh Sachdev1, Neeraj Aggarwal1, Reena K Joshi2, Raja Joshi3
1
Consultant Pediatric Cardiology, 2Consultant Pediatric Anesthetist, 3Consultant Pediatric Cardiac Surgeon, Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi

Cardiogenic shock can be a major and frequently fatal,

complication of both acute and chronic disorders that
affect the function of heart to maintain adequate tissue
perfusion. Despite advances in the management of
shock, cardiac failure with cardiogenic shock continues
to be challenging clinical problem. Rapid and efficient
treatment approach is needed to prevent morbidity and
mortality associated with it.

by this mechanism comes at the cost of elevated left

ventricular diastolic filling pressure, which in fact
increases myocardial oxygen demand and can result in
pulmonary edema (3, 4, 5).
However, owing to decrease in contractility, the left and
right ventricular filling pressures increase and cardiac
output decreases. Significant arterial oxygen desaturation
often occurs in cardiogenic shock, as a result of decrease
in mixed venous oxygen saturation and intrapulmonary
shunting. Mixed venous oxygen saturation decrease
occurs as a result of increased tissue oxygen extraction
because of the low cardiac output (4,5).

Definition
Cardiogenic shock is defined as decreased cardiac
output and evidence of tissue hypoxia in the presence
of adequate intravascular volume (1). Hemodynamic
criteria for cardiogenic shock are sustained hypotension
(systolic blood pressure <2SD for age for at least 30
min) and a reduced cardiac index (<2.2 L/min/m2) in
the presence of elevated pulmonary capillary occlusion
pressure (>15 mm Hg).

Systemic effects
In the state of shock the myocardial perfusion is
compromised and in children tachycardia is a major
compensatory mechanism to improve the systemic
perfusion. The pump failure results in rise of ventricular
diastolic pressures with increase in wall stress and
myocardial oxygen requirement. Decrease in systemic
perfusion due to decreased cardiac output, intensifies
anaerobic metabolism and starts the formation of
lactic acid, which further compromises the systolic
performance of the myocardium (4, 5).

Cardiogenic shock can be diagnosed clinically at bedside

by the presence of clinical signs suggestive of poor
tissue perfusion, which include oliguria, cyanosis, cold
extremities, altered mentation and hypotension. In most
patients these signs may persist after attempts have
been made to correct hypovolemia, arrhythmia, hypoxia,
and acidosis.

Impaired myocardial function triggers quite a few

physiologic compensatory mechanisms. These include
sympathetic stimulation, which causes rise of heart
rate, cardiac contractility and renal fluid retention. Renal
fluid retention augments the left ventricular preload.
Increased heart rate and contractility cause additional
rise of myocardial oxygen demand and worsening of
myocardial ischemia (4, 5).

Pathophysiology
Cardiovascular mechanics
An acute deterioration in the cardiac functions due to
any cause may lead to cardiogenic shock. The main
mechanical defect in cardiogenic shock is the rightward
shift of the left ventricular end-systolic pressure-volume
curve due to a marked reduction in contractility (2).
Consequently, the ventricle ejects less blood volume
per beat at a similar or even lower systolic pressure.
Therefore, the end-systolic volume is usually greatly
increased and stroke volume is decreased in cardiogenic
shock. As compensation to the decreased stroke volume
a right ward shift of the curvilinear diastolic pressurevolume curve occurs, resulting in a decreased diastolic
compliance. This causes increase in diastolic filling with
resultant increase in end-diastolic pressure. The attempt
to enhance cardiac output to maintain tissue perfusion

Fluid retention and impaired left ventricular diastolic

filling activated by tachycardia and ischemia result
in pulmonary venous congestion and hypoxemia.
Sympathetically mediated vasoconstriction not only
increases systemic blood pressure but the myocardial
afterload as well, which causes additional deterioration
of cardiac performance. All these factors which cause
increase in myocardial oxygen demand with simultaneous
inadequate myocardial perfusion and myocardial
ischemia can start a vicious cycle that ultimately ends in
death, without appropriate intervention (4, 5).
Usually, in cardiogenic shock the patient has both systolic
and diastolic myocardial dysfunction. Systolic ventricular
3

Table 2: Specific causes of cardiogenic shock (Right ventricular

failure)

function gets affected by metabolic derangements that

impair myocardial contractility. All these changes cause
increase of left ventricular filling pressure at a given enddiastolic volume (diastolic dysfunction) and pulmonary
congestion and congestive heart failure.

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o RV infarction
o Ischemia
o Hypoxia
o Acidosis
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o Pulmonary embolism
o Pulmonary vascular disease (eg, pulmonary arterial
hypertension, veno-occlusive disease)
o Hypoxic pulmonary vasoconstriction
o Peak end-expiratory pressure
o High alveolar pressure
o Acute respiratory distress syndrome
o Pulmonary fibrosis
o Sleep disordered breathing
o Chronic obstructive pulmonary disease
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Etiology
According to etiology and pathophysiology, causes
of cardiogenic shock can be categorized as systolic
dysfunction, diastolic dysfunction, valvular dysfunction,
cardiac arrhythmias, coronary artery disease, and
mechanical complications (Table 1, 2).
Signs and symptoms develop in patients with
cardiogenic shock when pulmonary venous pressure is
increased to critical levels or tissue perfusion is limited
severely (Table 3). Cardiogenic shock is diagnosed after
excluding other possible causes of hypotension, which
include hypovolemia, hemorrhage, sepsis, pulmonary
embolism, pericardial tamponade, aortic dissection, or
preexisting valvular disease and establishing presence of
myocardial dysfunction. Shock is considered if the patient
has evidence of multisystem organ hypoperfusion
on physical examination. It is important to recognize
certain physical examination findings which differentiate

Signs and symptoms

Table 3: Physiology of symptoms and signs of cardiogenic
shock
Symptom /
sign
Tachypnea

Table 1: Specific causes of cardiogenic shock (Left

ventricular failure)
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o Ischemia/infarction
o Global hypoxemia
o Valvular disease
o Myocardial depressant drugs (eg, beta-blockers, calcium
channel blockers, antiarrhythmics)
o Myocardial contusion
o Respiratory acidosis
o Metabolic derangements (eg, acidosis,
hypophosphatemia, hypocalcemia)
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o Ischemia
o Ventricular hypertrophy
o Restrictive cardiomyopathy
o Consequence of prolonged hypovolemic or septic shock
o Ventricular interdependence
o External compression by pericardial tamponade
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o Aortic stenosis
o Hypertrophic cardiomyopathy
o Dynamic aortic outflow tract obstruction
o Coarctation of the aorta
o Malignant hypertension
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o Mitral stenosis
o Endocarditis
o Mitral aortic regurgitation
o Obstruction due to atrial myxoma or thrombus
o Papillary muscle dysfunction or rupture
o Ruptured septum or free wall arrhythmias
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Dyspnea

Pathophysiology
It is a reflex response to rise in pulmonary
venous pressure, left ventricular volume
and pressure. Caused by stimulation of
vagus nerve or J receptors in pulmonary
interstitium. Increase in capillary
permeability and slower lymphatic
drainage implicated as factors responsible
for the predominance of this symptom in
infancy.
Results due to pulmonary congestion,
compression of bronchi by dilated
pulmonary arteries or atria and pulmonary
edema.
Lack of energy to suck and tire quickly.

Feeding
difficulty
Failure to thrive Inadequate calories due to poor intake and
extra work of breathing causes increased
metabolic demand.
Irritability
Reduced O2 transport to brain
Reduced urine Impaired renal perfusion
output
Sweating
Increased sympathetic activity
Increased
Chamber dilatation
precordial
activity
Tachycardia
Increased sympathetic activity as a
compensatory means to increase O2
delivery to tissues
Gallop sounds Increased afterload, reduced compliance
Hepatomegaly Systemic venous congestion
and oedema
Reduced
Reduced tissue perfusion
capillary filling
Crepitation
Alveolar edema

Table 4: Differentiating physical examination findings of

cardiogenic shock

therapy. Serial measurements of systolic and diastolic

functions help in deciding therapy and its efficacy. Various
echocardiographic parameters help in differentiating
abnormal loading conditions of heart from alteration in
contractility (7). Antenatal echocardiogram can help in
diagnosing fetal congestive heart failure which usually
manifests as fetal hydrops.

Irregular pulse
Narrow pulse pressure
Distended jugular vein
Distant heart sound
Presence of S3, S4
Presence of murmur
Crackles in chest

Blood gas and electrolytes

cardiogenic shock from other causes of shock (Table 4).

Blood gas analysis in patients with acute congestive

heart failure with low cardiac output usually shows
metabolic acidosis and lactic academia, while in chronic
congestive heart failure PaCO2 is low and pH is usually
normal (8). In patients on diuretic therapy hyponatremia
and hypochloremia should be monitored by regular
electrolyte analysis.

Investigations
Cardiogenic shock is diagnosed clinically and the
diagnosis is confirmed by various laboratory tests to
define the nature of specific disease, functional status
of myocardium and other co-morbid features (Table 5).
Laboratory investigations are required repeatedly to
assess the response to the provided treatment and for
prognosis.

Hypokalemia and lactic acedemia can develop owing to

reduced perfusion to tissues. Mixed venous oxygenation
is a measure of cardiac output and serial measurements
can help in directing the therapy and its efficacy.

Radiography

Miscellaneous tests

Chest radiography can help to exclude other possible

causes of shock or chest pain. Important conditions
like air leak syndrome and assessment of pulmonary
vasculature can be diagnosed. Cardiomegaly may give
a clue towards the underlying etiology. A widened
mediastinum suggests possibility of aortic dissection.

In neonates with left ventricle failure, tests for

hypoglycemia and hypocalcemia should be done. CPKMB and troponin I levels can help to diagnose coronary
insufficiency and asphyxia. If hemoglobin is low it can
increase left to right shunt by reducing the pulmonary
vascular resistance and aggravate the failure. To rule out
autoimmune disorders anti dsDNA and ANA assays can be
done. Blood levels of carnitine, lactate and glucose may
be done to diagnose mitochondrial cardiomyopathies.
Albuminuria, increased specific gravity and microscopic
hematuria may be detected on urine analysis. Presence
of methylglutamic aciduria suggests metabolic cause for
failure.

Electrocardiography
It helps to diagnose rhythm disturbances and structural
diseases like ALCAPA (6).
Table 5: Relevant investigations in cardiogenic shock
Chest radiography Rule out tension pneumothorax/
pneumomediastinum, Cardiomegaly,
Pulmonary venous congestion, Kerley B
lines, Pleural effusion, Alveolar edema
(B/L parahilar distribution)
ECG
Chamber hypertrophy, Arrhythmia,
Heart block
Echocardiography Ejection fraction, Systemic vascular
resistance, Cardiac index, Diastolic
dysfuction, Assessment of response to
therapy
Blood gas and
Metabolic acidosis, Lactic acidosis,
electrolytes
Abnormalities of sodium/potassium/
chloride
Cardiac
Chamber pressure, Myocardial biopsy,
catheterization
Assessment of coronary vessels
Miscellaneous
Glucose, Calcium, magnesium, Cardiac
enzymes, Routine urine examination

Cardiac catheterisation
It offers functional data of the failing myocardium.
Myocardial biopsy helps in obtaining the specimen
for histological and PCR testing, may be helpful in
diagnosing underlying cause like myocarditis. Coronary
angiography may help in identifying cases of coronary
abnormalities and Kawasaki Disease.
Hemodynamic data usually reveals elevated left
ventricular and right ventricular end diastolic pressures.
Structural abnormality causing cardiogenic shock can
also be identified using angiography and hemodynamic
monitoring.

Management
Echocardiography

If a patient presents with shock an early working diagnosis

must be made, and urgent resuscitation should be done.
The working diagnosis is confirmed subsequently with
investigations.

The test is useful to diagnose anatomic causes, functional

status and as a follow up assessment of response to

Fluid resuscitation to correct hypovolemia and

hypotension is the most important and initial step
unless pulmonary edema is present (9). It is advisable
to give small fluid boluses (5-10 ml/kg) rather than
large bolus to avoid precipitation of pulmonary edema.
Central venous and arterial lines should be inserted.
Multiparameter monitor is a must for proper monitoring
of the patients with cardiogenic shock. Oxygenation and
airway protection are essential and if needed intubation
and mechanical ventilation should be started. Blood
gas analysis and correction of electrolyte and acid-base
abnormalities, such as hypokalemia, hypomagnesaemia,
and acidosis are critical (Table 6).

action, which improves renal flow and naturesis.

Hence, often used in cardiogenic shock following
cardiopulmonary bypass. At 5-15g/kg/min it has
inotropic effect ( effect). At doses higher than 20g/
kg it may compromise renal flow, increase pulmonary
vascular resistance, oxygen demand and ventricular
afterload. It should not be mixed with soda bicarbonate
infusion(11-13).
Dobutamine
Dobutamine acts predominantly on 1 than 2 and
receptors and does not depend on norepinephrine stores
to produce the desired effects. In the recommended
dose of 2-20g/kg/min it causes mild vasodilatation,
increases the cardiac output, reduces the systemic
resistance with minimal alteration of blood pressure and
heart rate. It has a short half life of 2-3minutes and does
not alter or impair renal flow (11, 14 16). It is preferred
in perioperative cases as it is less arrhythmogenic and
augments cardiac output. In patients with postoperative
failure, a combination of 3-5g/kg/min of dopamine
with dobutamine 5-10g/kg/min and afterload reducing
agents as milrinone is often used.

Table 6: Goals of treatment

General supportive care to maintain systemic perfusion
Relieve pulmonary and systemic congestion
Improve myocardial performance
Optimize afterload
Treat the underlying cause and prevent disease progression

Diuretics
Diuretics are used primarily for relief of systemic and
pulmonary vascular congestion. Major agents used are
loop diuretics, thiazide and aldosterone antagonists.
Furosemide is the most commonly used diuretic for
optimizing the preload in cardiogenic shock. It has
a rapid onset of action (2-5 min) and duration of
action is 3 hours. It can be administered as a bolus or
as a continuous infusion. Use of continuous infusion
results in less hemodynamic instability and electrolyte
imbalances (10). Common adverse effects of furosemide
include hypokalemia, metabolic alkalosis, hypocalcemia,
hyponatremia and hyperuricemia. Ototoxicity is a rare
complication and more commonly reported in neonates
and infants and is usually reversible.

Epinephrine
It has both and effects. In patients with cardiogenic
shock and in postoperative situations with intense
vasoconstriction it may improve cardiac output (11). It
has a short half life of 2-3minutes. It increases the risk
of arrhythmia by its excessive chronotropic action and
causes down regulation of receptors on long term
use. It should preferably be administered through
central venous catheter. It should be used as short term
treatment for patients unresponsive to other drugs and
should be tapered off as early as possible.
Norepinephrine

Thiazides have a slower onset of action and are available

in oral form so their use in acute heart failure is limited.
They are commonly used for chronic congestive heart
failure.

It should be used cautiously due to its deleterious effects

on afterload, renal flow and myocardial demand. It has a
short half life of 2-3min.

The aldosterone antagonist, spironolactone is a weak

diuretic and is rarely used alone as a diuretic. It is usually
added to loop diuretics or thiazides to antagonize their
kaliuretic action. It is given orally and the onset of action
takes 2-3 days.

Isoproterenol
It has 1 and 2 effects owing to which it improves
contractility and heart rate along with vasodilatation.
Valuable for managing patients with acute heart failure
complicated by increased reactive pulmonary vascular
resistance or complete heart block. It does not alter
renal blood flow. Care should be taken to maintain
intravascular volume during infusion.

Ionotropes
Dopamine
Effective in acute cardiac failure and cardiogenic shock,
particularly when a postoperative patient has low cardiac
output, it has a short half life of 3-4 minutes.

Nonglycosidal, Noncatecholamine Agents

Phosphodiesterase III inhibitors are one of the most
effective drugs which help to augment the stroke
volume in shock. They act by improving the contractility

The effect of dopamine varies with the dose infused.

When used at 3-5g/kg/min it has best dopaminergic
6

and simultaneously reducing the afterload. Amrinone

and milrinone belong to this group. They are potent
vasodilators and ionotropes, but the onset of action
is slower than adrenergic agents. Milrinone is used
widely as it is free of harmful side effects of amrinone.
Complications of amrinone include hypotension,
ventricular ectopy and thrombocytopenia (Table 7) (17 -23).

used for more than 72 hours.

Nitroglycerine
Nitroglycerine increases venous capacitance and reduces
filling pressures. It is mostly used in conditions with
increased preload and pulmonary venous congestion. If
the patients cardiac output is compromised side effects
are increased.

Digoxin
It is the most widely used and studied drug in congestive
heart failure. However it is not generally preferred in
cardiogenic shock as there is a risk of increased toxicity
in such situation. It acts by inhibiting sarcolemal Na+K+ATPase activity which causes increase in intracellular
calcium and thereby augments ventricular contractility.
Heart rate and conduction are slowed as well. Used
most often when myocardial contractility needs to be
improved. Use of digoxin in large left to right shunts is
controversial. It gives subjective benefit in those with
systolic dysfunction due to neurohormonal modulating
effect. It is useful to manage fetal congestive cardiac
failure induced by tachycardia (24).

Newer Drugs
Levosimendan
Levosimendan, a distinct calcium sensitizer, is a new
class of inotropes that share the in vitro properties of
calcium sensitization and phosphodiesterase inhibition.
It has an action of stabilization of interaction between
calcium and troponin C by its binding to troponin C in a
calcium-dependent manner, thereby improving inotropy
without any adverse effect on lusitropy. Its vasodilatory
effect is possibly by activation of several potassium
channels and other less understood mechanisms. Unlike
other inotropes, levosimendan use does not result in
significant increase in myocardial oxygen consumption,
arrhythmia, and neurohormonal activation. In large
controlled trials in patients with decompensated heart
failure, intravenous levosimendan was found to be
more effective than placebo or dobutamine for overall
hemodynamic improvement. Significant benefits were
also seen for mortality (versus placebo or dobutamine).
The pharmacokinetic profile of levosimendan in children
is similar to that in adult patients with congestive heart
failure (26 -29).

Vasodilators
These drugs are important in the management of
patients with cardiogenic shock secondary to left to
right shunt, postoperative low cardiac output, severe
atrio-ventricular valve regurgitation and dilated
cardiomyopathy. Mostly used along with ionotropes
and diuretics to improve cardiac function by favorably
altering afterload and preload. The patients should
be monitored for systolic blood pressure and filling
pressures (25). Drugs like prazosin and hydralazine are
not widely used in children in acute setting except in
cases of scorpion sting poisoning.

Neseritide
B-type natriuretic peptide (BNP) is an endogenous
cardiac neurohormone, produced in the ventricles in
response to pressure and volume elevation. Neseritide is
identical to endogenous BNP and serves to compensate
for deteriorating cardiac function causing preload and
afterload reductions, natriuresis, diuresis, suppression of
the renin-angiotensin-aldosterone system, and lowering
of norepinephrine. Based on its unique pharmacologic
profile, neseritide results in clinically significant balanced
vasodilatation of arteries and veins, and may be well
suited for patients presenting with various scenarios
of decompensated heart failure usually due to volume
overload. In clinical trials, neseritide has been shown
to decrease pulmonary capillary wedge pressure,
pulmonary artery pressure, right atrial pressure, and
systemic vascular resistance, as well as increase cardiac
index and stroke volume index. Heart rate variability also
improved with neseritide. Experience with neseritide
in pediatrics is limited though it has shown promising
results (30, 31).

ACE inhibitors (captopril, enalapril)

ACE inhibitors, captopril, enalpril, favorably alter
the maladaptive mechanism of renin-angiotensin
system. Hemodynamic status improves by its actions
including ventricular remodeling, reducing systemic
vascular resistance and increasing venous capacitance.
Monitoring of blood pressure and neutrophil count are
needed. However it has limited use in acute stage.
Sodium nitroprusside
It effectively reduces afterload and decreases filling
pressures, systemic and pulmonary vascular resistance. It
is often used in acute cases and in postoperative patients
who need afterload reduction. Hypotension is a common
side effect which can easily be managed by reducing
or stopping the continuous infusion. Monitoring for
thiocyanate toxicity is a possible side effect when drug is

shock secondary to severe myocardial dysfunction. As

more positive results come in this can truly revolutionize the
survival of critically sick children with cardiogenic shock (35).

Mechanical Afterload Reduction

Intra aortic balloon pump (IABP)
It is not used widely in children as there are few
clinical settings in children unlike in adults due to
technical problems related to catheter size. It has been
effectively used in cases with coronary artery disease
as in Kawasaki disease or ALCAPA and in postoperative
setting with reduced cardiac output to achieve diastolic
augmentation of blood pressure. It may be considered as
an option for end stage failure (32 -34).

Ventricular assist device (VAD)

This modality is still in the developmental stages in
children and may be used as a bridge to transplant or if
there is difficulty in weaning from bypass. They may also
be of use in children with fulminant myocarditis where
they may help in stabilization of patients till the time the
myocardium starts recovering.

Extracorporal membrane oxygenation (ECMO)

Abdominal compression devices

More and more information and experiences are coming

in for the use of ECMO in children with cardiogenic

Antishock trousers or ventilator reservoir bags are used

to reduce right heart volume overload in children with

Table 7: Summary of management of cardiogenic shock

Monitor CVP, PCWP, LAP,
cardiac chamber size on
echocardiography
Monitor CI, BP, EF, SVO2

Increase stroke volume Optimize preload by volume expansion albeit small boluses with careful
monitoring for development of hepatomegaly, crepitations

SVR,BP, peripheral
perfusion

Reduce afterload

Increase contractility

Mechanical support

Treat underlying cause correct acidosis, treat hypoxia, inotropic agents, Beta
adrenergic agents, phosphodiesterase inhibitors
Ionodilators like milrinone, levosimendan vasodilators like nitroprusside,
nitroglycerine, ACE inhibitors,
IABP, ECMO, VAD

CVP central venous pressure, PCWP pulmonary capillary wedge pressure, LAP left atrial pressure, CI cardiac index, BP blood
pressure, SVO2 mixed venous saturation, SVR systemic vascular resistance, IABP intraaortic balloon pressure, ECMO extracorporeal
membranous oxygenation, VAD ventricular assist device
Algorithm for Inotropic Management
Clinical condition
Tachycardia, cold peripheries, feeble
pulses, sluggish capillary refill, reduced
urine output, Low BP, hyperlactatemia

Interpretation
Low cardiac output

Tachycardia, feeble pulses, cold

peripheries, sluggish CRT, reduced urine
output, normal BP, hyperlactatemia
Heart rate settling, good central pulses
weak peripheral pulses, cold peripheries,
BP normal to high, hyperlactatemia
Tachycardia, good pulses, warm
peripheries, urine output decreases, BP
slightly low, hyperlactatemia
Good pulses, cold peripheries, low
urine output, BP normal to high,
hyperlactatemia
HR settled, good pulses, warm extremities,
good urine output, normal BP, normal
lactate
Tachycardia, good volume pulses, warm
extremities, falling urine output, BP
maintained, lactates start increasing
HR settled, good pulses, warm extremities,
good urine output, normal BP, normal
lactate, extubated.

Low cardiac output with

maintained BP

Treatment strategy
Optimise preload albeit in small volumes (5ml/kg) with
careful monitoring for hepatomegaly and crepitations
Dopamine 10 ug/kg/min, Dobutamine 10 ug/kg/min,
may increase up to 15 ug/kg/min, Elective ventilation in
case of respiratory distress or a very critically sick child.
Add furosemide infusion 0.05-0.1 mg/kg/hr Add
Inodilators (Milrinone/Levosimendan)

High afterload

Add milrinone or increase it if already added

hypovolemia (probably
Volume bolus
due to milrinone induced
vasodilation)
High afterload
Go up on milrinone

Optimal CO

Maintain same inotropic support, start weaning from

ventilation

Falling CO

Increase inodilators (milrinone)

CO optimized

Plan inotrope tapering (slow and gradual tapering)

after adding digoxin and vasodilators like enalapril.

right heart failure. Expereince with these modalities is

limited.

Treatment of Diastolic Failure

Diastolic failure is defined as increase in end diastolic
pressures when the ventricular volume is normal.
Therapy for this type of heart failure is of uncertain
success. Medications used for the management of
diastolic failure include low dose diuretics, beta blockers,
calcium channel blockers and ACE inhibitors, but their
effectiveness is limited. Constrictive pericarditis must
be ruled out in patients who present with symptoms
suggestive of diastolic failure.

Adjunctive Therapy
Bed rest
Rest helps to reduce the work load of compromised
myocardium. Activity can be gradually increased
according to the response to treatment.
Position

Treatment of Underlying Condition

The patient should be placed in semi-Fowler position

either by modified cardiac chair or elevating head and
shoulders to 450. It may help to improve the pulmonary
function by easing respiration and reducing pulmonary
pooling.

Transcatheter interventions may be needed for treatment

of defects such as critical aortic stenosis or coarctation.
After stabilizing the patient early surgical correction
of large left to right shunts is needed at the earliest.
Procedures like dynamic cardiomyoplasty may be useful
in some patients. In addition supportive treatments
which include control of infection, anemia, arrhythmias,
hypertension and metabolic deficiencies are required.

Oxygen
Oxygen can be given through mask or nasal prongs
with adequate humidification. It is useful to improve
oxygenation. In neonatal period oxygen should be
administered with caution in patients with duct
dependent cardiac defects.

Administration of intravenous immunoglobulin has

been observed to be useful in patients with myocarditis.
Treating the mother in antenatal period with digoxin
can help to control supraventricular tachycardia causing
failure in the foetus. If the patient fails to respond to any
treatment the final option for end stage failure would be
cardiac transplant.

Positive pressure ventilation

Intubation and mechanical ventilation in cardiogenic
shock can improve blood gas tension and reduce work
of breathing in infants. It is useful in patients with left
ventricular dysfunction mainly due to reduction in
afterload.

Counseling
An important part of management includes discussion of
the problem, genetic implications, treatment modalities
and the prognosis with the parents. According to many
authors the term failure should be preferably avoided
when discussing with parents and terms like congestion
seem to be accepted more readily. The family will need
continuous support of the primary pediatrician.

Prostaglandin (PGE1)
The drug can help to maintain the ductal patency in
duct dependent systemic circulation like hypoplastic left
heart syndrome and improve systemic perfusion and
reduce pulmonary congestion. The dose of PGE1 is 0.050.1g/kg/min infusion.
Diet

References

Adequate calorie and protein intake should be

maintained to meet the increased metabolic needs.
Calorie requirement in infants may be high upto 130170Cal/kg/day. Salt restriction should be done in the
presence of severe systemic congestion or edema. Use
of low solute formula with 7-12meq/L may be required
in infants. Salt restriction has to be individualized so
that unnecessary weight loss can be prevented. Iron
supplement 2-3mg/kg should be started as it benefits by
improving anaemia. L Carnitine supplementation should
be done in patients with dilated cardiomyopathy. The
dose of carnitine is 20-35mg/kg/dose orally three times
a day. In severely symptomatic children nasogastric tube
feeds can help to improve calorie intake and weight gain.

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