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Lecture Notes on Congenital Heart Diseases

Prepared By: Mark Fredderick R Abejo R.N, MAN

Has heart wall has 3 layers

Endocardium lines the inner chambers


of the heart, valves, chordate tendinae
and papillary muscles.

Myocardium muscular layer, middle


layer, responsible for the major
pumping action of the ventricles.

Epicardium thin
covering(mesothelium), covers the outer
surface of the heart

Pericardium invaginated sac

Visceral attached to the exterior of


myocardium

Parietal attached to the great vessels


and diaphragm

Papillary Muscle
Arise from the endocardial & myocardial surface of the
ventricles & attach to the chordae tendinae

Chordae Tendinae
Attach to the tricuspid & mitral valves & prevent
eversion during systole

Separated into 2 pumps:


right heart pumps blood through the
lungs
left heart pumps blood through the
peripheral organs

Chamber of the Heart


Atria
2 chambers, function as receiving chambers,
lies above the ventricles
Upper Chamber (connecting or receiving)
Right Atrium: receives systemic venous
blood through the superior vena cava,
inferior vena cava & coronary sinus
Left Atrium: receives oxygenated blood
returning to the heart from the lungs trough
the pulmonary veins

PEDIATRIC NURSING
Congenital Heart Diseases
Lecturer: Mark Fredderick R. Abejo RN, MAN
________________________________________________
HEART

Ventricles
2 thick-walled chambers; major
responsibility for forcing blood out of the
heart; lie below the atria
Lower Chamber (contracting or pumping)
Right Ventricle: contracts & propels
deoxygenated blood into pulmonary
circulation via the aorta during ventricular
systole; Right atrium has decreased pressure
which is 60 80 mmHg
Left Ventricle: propels blood into the
systemic circulation via aortaduring
ventricular systole; Left ventricle has
increased pressure which is 120 180 mmHg
in order to propel blood to the systemic
circulation

Cardiovascular system consists of the heart,


arteries, veins & capillaries. The major function are
circulation of blood, delivery of O2 & other nutrients to the
tissues of the body & removal of CO2 & other cellular
products metabolism
Heart

Muscular pumping organ that propel blood into the


arerial system & receive blood from the venous system
of the body.
Hollow muscular behind the sternum and between the
lungs
Located on the middle of mediastinum
Resemble like a close fist
Weighs approximately 300 400 grams

Pediatric Nursing

Heart Valves

Tricuspid

Pulmonic

Mitral

Aortic

Abejo

Lecture Notes on Congenital Heart Diseases


Prepared By: Mark Fredderick R Abejo R.N, MAN

Heart Circulation

Foramen ovale: opening between the right and left


atriumbypasses fetal lungs.
The foramen ovale is an opening in the wall that
separates the upper right and left heart chambers
(atria). This opening allows blood to flow to the
left side of the heart without going to the lungs.
Before birth, the foramen ovale is kept open by
the pressure of blood that passes through it. When
the baby takes the first breath, blood begins to
flow through the lungs, and the foramen ovale
closes .

Ductus arteriosis (DA): Opening between pulmonary


artery and descending aorta; allows fetal blood to
bypass the lungs.
There is still very little blood getting into the fetal
lungsjust enough to help it grow in utero, even
though they are collapsed until birth.

Purpose of these structures


Allow most of blood to bypass the liver and lungs.
SHUNTS-shortcuts
Normal Cardiovascular system
The right side and the left side of the heart are
anatomically and functionally separated by the
interatrial and interventricular septa.
There are no connections between major arteries
and veins.
Only capillary connections in the lungs and
systemic circulation exist.

FETO PLACENTAL CIRCULATION


Oxygenated blood from placenta to ductus
venosus inferior vena cava right
atrium.
Blood then shunts over to the left atrium
through the foramen ovale.
Then over to the left ventricle aorta
head/extremities.
The unoxygenated blood returns to the right
atrium via the superior vena cava flows into the
right ventricle exits thought the pulmonary
artery (which is connected to the aorta).
Most of this blood shunts through the ductus
arteriosis into descending aorta, and back into the
placenta.

Review of prenatal circulation- 3 essential structures

Ductus venosus (DV): opening between umbilical vein


and inferior vena cava.
The ductus venosus is a vessel that allows blood
to bypass the fetus's liver. It carries blood with
oxygen and nutrients from the umbilical cord
straight to the right side (right atrium) of the
fetus's heart. The ductus venosus closes shortly
after birth, when the umbilical cord is cut and
blood flowing between the mother and fetus stops.

Pediatric Nursing

Ductus venosus- shunts from liver to IVF


Foramen ovale- shunts bet 2 atrias
Ductus arteriosus- from pulmonary artery to aorta
2 ways to facilitate closure of foramen ovale
1. Tangential Footstep
slap foot of baby
never stimulate baby to cry if secretions not full
drained to prevent aspiration
check characteristic of cry
- normal cry- strong, vigorous and lusty cry
- cri-du-chat syndrome- chromosomal obliteration
cat like cry
2. Proper position
right side lying pos.
will increase pressure on left and foramen ovale
will close
NOTE: Foramen Ovale and Ductus arteriosus will begin to
close within 24h

Factors contributing to Congenital cardiac anomalies


Drugs ex. Thalidomide, Dilantin, Lithium
Alcohol and illegal drugs
Chromosomal abnormalities ex. Trisomy 21
(Downs syndrome)
Infections during pregnancy such as Rubella
Connective tissue disorders
Inborn errors of metabolism
Family history of congenital anomalies
Shunts

What are shunts?


They are anomalous connections between the
right side and left side of the heart or between the
venous and arterial circulation

Abejo

Lecture Notes on Congenital Heart Diseases


Prepared By: Mark Fredderick R Abejo R.N, MAN

Direction of Shunts
Since pressures in the left side of the heart are usually
higher than the right side, in shunts the blood flows from
left to right. However right to left shunts occur when the
following conditions are present:
Severe pulmonary hypertension
Right ventricular outflow obstruction
as in
Tetralogy of
Fallot
Obstruction to the inflow of blood as in tricuspid
atresia
Pulmonary arteriovenous fistula
Anomalous origin of the major arteries

Birth changes

Assessment of cardiac function

History
History of heart disease in the family
Contact with known teratogens, such as rubella
during pregnancy
Presence of chromosomal abnormalities
(Downs)
Poor weight gain and/or feeding behavior
Exercise intolerance and/or fatigue during feeds
Sweating during feeding
Frequent respiratory infections
Respiratory difficulties, such as tachypnea,
dyspnea, and shortness of breath.
Recent streptococcal infection (may lead to
valve damage)

Palpation
Quality and symmetry of pulses
Auscultation
Heart rate and rhythm
Presence of murmurs
Height and weight
Position of comfort
Remember, squatting/fetal positions are
often comfortable for a child with a
CHD.

Tests of cardiac function


Electrocardiography
Records electricity generated by the
beating heart
Painless but scary, child must be still

Infant cries, lungs expand


Ductus arteriosis closes as resistance decreases though
the pulmonary vasculature (infants now need to use
their own lungs)
Clamping cord causes ductus venosus to clot (infant
needs to use own liver)
Venous return from lungs causes increase in left atrium
pressure.
Increased left atrium pressure causes left to right blood
flow through patent foramen ovale (which is a bad
thingwe no longer want to bypass the lungs)
The foramen ovale is a one way valve so it closes
permanently.

Congenital Heart Diseases

Exercise stress test


Monitoring of heart rate, BP, ECG, and
oxygen consumption at rest and during
exercise on a tread mill or bicycle
Chest x-ray
Shows accurate picture of heart size and
contour; size of the heart chambers
Used more as a screening tool
Echocardiography
Ultrasound (high frequency sound waves
produce an image of heart structures)
The primary diagnostic test for heart
disease.

Two Major Types


1.
2.

Acyanotic Heart Defect Left to Right Shunting


Cyanotic Heart Defect Right to Left Shunting

ACYANOTIC HEART DEFECT


I.

Atrial Septal Defect - failure of foramen ovale to


close

Physical exam
Begins with observation of general appearance,
then the specifics
Use general assessment techniques but look
specifically for the following:
Inspection:
Nutritional state: failure to thrive or poor
weight gain
Skin color: cyanosis and pallor
Chest deformities- enlarged heart
Unusual pulsations of neck veins seen in
some patients
Respiratory pattern- tachypnea, dyspnea,
presense of expiratory grunt
Clubbing of fingers (now rarely seen in
children d/t advances in surgical
techniques)

Pediatric Nursing

Abejo

Lecture Notes on Congenital Heart Diseases


Prepared By: Mark Fredderick R Abejo R.N, MAN

A.

Signs & Symptoms

B.

systolic murmurs at upper border of


sternum
cardiac catheterization reveals increased
O2
saturation at R side of heart
ECG reveals hypertrophy of R side of
heart

Management
open heart surgery
long term antibiotic to prevent
subacute bacterial endocarditis

II. Ventricular Septal Defect - opening bet 2


ventricles

B.

Diagnostics in PDA

C.

A.

B.

Signs & Symptoms


systolic murmurs at lower border of
sternum
cardiac catheterization reveals increased
O2 saturation at R side of heart
ECG reveals hypertrophy of R side of
heart

EKG: LVH and LAH


Chest x-ray:
Cardiomegaly with prominence of
the apex suggesting LV dilatation
Echocardiogram in PDA
Dilated main pulmonary artery
Left ventricular volume overload
On the doppler, blood flow will be
seen from the aorta to the
pulmonary artery
Cardiac catheterization in PDA
There is a step up in O2 saturation
in the pulmonary artery
On aortic angiography, x-ray
contrast flows from the aorta to the
pulmonary artery

Therapy of PDA
Prostaglandin
Transcatheter closure of the PDA
Surgical ligation of the PDA

IV. Pulmunary Stenosis narrowing of valve of


pulmonary artery

Management
open heart surgery
long term antibiotic to prevent
subacute bacterial endocarditis

III. Patent Dactus Arteriosus - failure of ductus


arteriosus to close
A.

Pediatric Nursing

Key PE finding in PDA


A thrill and a continuous
machinery like murmur at the
upper LSB or left infraclavicular
region.

Abejo

Lecture Notes on Congenital Heart Diseases


Prepared By: Mark Fredderick R Abejo R.N, MAN

V.

A.

Signs & Symptoms


typical systolic ejection murmur
S2 sound widely split
ECG: right ventricular hypertrophy

B.

Management
Surgery
Balloon Septostomy, is the widening of
a foramen ovale, patent foramen ovale
(PFO), or atrial septal defect (ASD) via
cardiac catheterization (heart cath)
using a balloon catheter. This procedure
allows a greater amount of oxygenated
blood to enter the systemic circulation
in some cases of cyanotic congenital
heart defect (CHD).

B.

Symptoms of Coarctation of the aorta


Headaches
Leg
fatigue
and
intermittent
claudication
Signs of CHF due to uncontrolled
hypertension

C.

PE findings in coarctation of the aorta


Hypertension with differential BP
between the upper and lower extremity
Thrills at the supra sternal notch
Collateral vessels in the scapula
Systolic ejection murmur in the
interscapular region
A systolic murmur is heard in the 2nd
RSB if a concomitant bicuspid aortic
valve is present

D.

Diagnostics in coarctation of the aorta


EKG : LVH
Chest x-ray:
the 3 sign _ dilated aorta and
cardiomegaly
Rib notching posteriorly
Echocardiogram:
The coarctation maybe visualized
depending on the site.
The pressure gradient can be
measured by doppler.
CT scan or MRI angiogram: the
presence and the site of the coarctation
is confirmed.

E.

Complications
CHF
Aortic rupture
Bacterial endocarditis
CVA due to rupture of Berry aneurysm

F.

Therapy
Antihypertensive therapy
Treatment of CHF
Balloon dilation
Surgery

Aortic Stenosis narrowing of valve of aorta

C.

Signs & Symptoms


typical murmur - rough systolic sound
and thrill
if active manifest angina like symptoms
ECG: Left ventricular hypertrophy

D.

Management
Surgery
Balloon Septostomy

VI. Coartation of the Aorta


It is a constriction in the aorta usually after
the origin of left subclavian artery
It presents as hypertension. The symptoms
and findings depends on the severity of the
coarctation and site.
A.

Pediatric Nursing

Pathophysiology in coarctation of the


aorta
The hypertension is due to the gradient
across the coarctation
And the response of the reninangiotensin-aldosterone system

Abejo

Lecture Notes on Congenital Heart Diseases


Prepared By: Mark Fredderick R Abejo R.N, MAN

CYANOTIC HEART DEFECT


I. Transposition of Great Arteries (TOGA)
aorta arising from Rt ventricle; pulmonary
artery arising form Lt ventricle

A.

Signs & Symptoms


cyanosis after 1st cry (due no
exygenation)
polycythemia increased RBC
=compensatory due to O2
supply=viscous blood=thrombus =
embolus = stroke
ECG: cardiomegaly
Cardiac cath: decreased O2 saturation

B.

Management
Surgery

A.

Signs & Symptoms


Rt ventricular hypertrophy
high degree of cyanosis
polycythemia
severe dyspnea squatting position
relief , inhibit venous return facilitate
lung expansion.
growth retardation due no O2
tet spell or blue spells- short episodes
of hypoxia
syncope
clubbing of fingernails due to
chronic tissue hypoxia
mental retardation due decreased O2
in brain
boot shaped heart x-ray

B.

Management

II. Tetralogy of Fallot


P pulmonary stenosis
V ventricular SD
O overriding or dextroposition of aorta
R Rt ventricular hypertrophy

Pediatric Nursing

O2
no valsalva maneuver , fiber diet
laxative
morphine hypoxia
propranolol decrease heart spasms
BLT blalock taussig procedure palliative repair
Brock procedure complete procedure

Abejo

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