CONGENITAL HEART DISEASE

I. INTRODUCTION

The circulatory system is an organ system that passes nutrients (such as

amino acids, electrolytes, lymph), gases, hormones, blood cells, etc. to and
from cells in the body. It helps fight diseases, stabilize body temperature and
pH and maintains homeostasis. The humans have a closed cardiovascular
system (meaning that the blood never leaves network of arteries, veins and
capillaries). Two types of fluids move through the circulatory system: blood
and lymph. Lymph is essentially recycled blood plasma after it has been filtered
from the bold cells“.1 returned to the lymphatic system. The blood and the
lymphatic system collectively make up the Circulatory system.
II. HUMAN CARDIOVASCULAR SYSTEM

The main components of the human cardiovascular system are the heart,
blood and blood vessels. It includes: the pulmonary circulatory loop, where the
blood is oxygenated and the systemic circulate, loop, which supplies
oxygenated blood through the body. An average adult contains roughly 4.7
5.7mm of blood which consists of plasma, red blood cells, white blood cells
and the platelets.
i) Pulmonary Circulation: The pulmonary circulatory system is the portion
of the cardiovascular system in which oxygen-depleted blood is pumped
away from the heart via pulmonary artery to the lungs and oxygenated
blood from lungs is returned to the heart via the pulmonary vein.
ii) Systemic Circulation: Systemic circulation is the portion of cardiovascular
system which transports oxygenated blood via aorta away from the heart to
the body, and returns oxygen depleted blood back to the heart via superior
and inferior vena cava.
 iii) Coronary Circulation: The coronary circulatory system provides blood
supply to the heart itself, As it provides oxygenated blood to the heart, it is a
part of the systemic circulation.
III. HEART

The heart pumps oxygenated blood to the body and deoxygenated blood to the
lungs. In the human head there is one atrium and one ventricle for each
circulation-pulmonary and systemic. In all there are four chambers in heart:
right atrium, right ventricle, left atrium and left ventricle. The right atrium is
the upper chamber of the right side of heart. The deoxygenated blood returned
to the right atrium is passed into right ventricle to be pumped through the
pulmonary artery to lungs for reoxygenation and removal of carbon dioxide.
The left atrium receives oxygenated blood from lungs through the pulmonary
vein which is passed into left ventricle to be pumped through the aorta to
different organs of the body.

IV. DISORDERS OF CARDIOVASCULAR SYSTEM

The disorders of cardiovascular system, seen in children are:
1. Congenital heart defects

2. Other cardiac disorders

V. CONGENITAL HEART DISEASES
1. INTRODUCTION

Important neonatal adjustments occur immediately after birth, when the

newborn infant starts to adopt himself to extra uterine conditions. In about
80% of children the diagnosis of having a congenital malformation of the
heart, can be find out by a proper history and a well conducted physical
examination, supplemented by screening and ECG test of the child.

2. DEFINITION

Congenital heart disease refers to a problem with the hearts structure and
function due to abnormal heart development before birth. Congenital means
present at birth.

3. INCIDENCE

The overall incidence of congenital heart disease is about 6-8 per 1000live
births and 1 per 1000 at years of age.

4. ETIOLOGY

The major cause of CHD s are unknown. Most cases of CHDs were
thought to be multifactorial and result from a combination of genetic
predisposition and environmental stimulus.

Congenital heart defects may occur due to:

i) Hereditary : Those caused by the defects inherent in the genes or

germplasm. Certain genetic factors may have role in CHDs. Certain heart
diseases are common in Asian children.
ii) Vitamin deficiency
 iii) Viral infection: Mothers who have viral disease like German
measles(rubella), mumps in the 3 months of pregnancy are more prone to
give birth to children with multiple congenital anomalies.
iv) Teratogenic effects of radiation and drugs
v) Chromosomal abnormalities: a small percentage of CHD are related to
chromosomal abnormalities in particular , trisomy 21, 13,18 ad turners
syndrome.
5. PHYSICAL AND PSYCHOLOGICAL RESPONSES TO
CONGENITAL HEART DISEASE

The term CHD includes several different types of defects all of which singly
or in combination produce certain effects on the body. These include:

1. Inadequate cardiac output

2. Inadequate cardiac output into the pulmonary circulation
3. Pulmonary hypertension due to increased vascular resistance in the lungs
4. Systolic or diastolic overloading of the heart chambers
5. Lack of adequate tissue oxygenation if oxygenated blood is shunted into
the system
Various physical and psychological responses can result from these
abnormal effects and may occur sequentially or together.

Murmurs and
Growth cardiac failure Decreased exercise
retardation
tolerance and
dyspnoea
Physical and
Tissue psychological Decreased exercise
hypoxia
responses to
congenital heart
Tachycardia and
Cyanosis and disease tachypnea
polycythemia Recurrent
6. PATHOPHYSIOLOGYrespiratory
infections
 The exact cause is unknown.However, trhe belief is that it results from
interplay of several factors including genetics and maternal exposure to
environmental factors
 CHD results from some interference in the development of the heart
structure during fetal life
 The septal walls or valves may fail to develop completely vessels or valves
may be stenotic, narrowed or transposed.
 Structure that formed to allow fetal circulation may fail to close after birth,
altering the pressures necessary to maintain adequate blood flow
 After birth , with change from fetal to newborn circulation , pressure with
the chambers of the right side of the heart are less than those of the left side
and pulmonary vascular resistance is less than that for the systemic
circulation.
 These normal pressure gradients are necessary for adequate circulation to the
lungs and the rest of the body.
 However, these pressure gradient become disrupted if a structure has failed
to develop, a fetal structure has failed to close , a narrowing , stenosis or
transposition of a vessel has occurred.
7. THERAPEUTIC MANGEMENT
1. Prenatal education: prenatal education about avoiding certain
substances or infection is essential to promote optimal outcomes for the
fetus.
2. Genetic counselling: parents of children with CHD are encouraged to
receive genetic counselling because of the probability of having
subsequent children with a congenital heart defect.
3. Palliative care :therapeutic management of other forms of CHD focuses
on palliative care or surgical interventions.

8. CLINICAL FEATURES OF CONGENITAL HEART DISEASE

(1) Cyanosis : In congenital heart disease, there is a mixture of oxygenated

blood with deoxygenated blood resulting in central cyanosis. Cyanotic spells
are life threatening if not treated in time.
(2) Clubbing occurs :Clubbing is pronounced in children with congenital
heart disease of cyanotic variety.
(3) Squatting: is the characteristic posture adopted after exertion by ambulant
children with certain type of congenital heart disease, especially fallots.
 (4) Ventricular overload :Ventricular overload occurs in aortic stenosis and
coarctation of aorta. This is often well tolerated and may not manifest. Because
the left ventricle is capable of withstanding a huge work load, but this
adaptability may suddenly fail and result in sudden death. Systolic overload at
the right ventricle on the other hand, produces several warning symptoms
exertional dyspnoea, fatigue, cyanosis etc.
(5) Inadequate cardiac output : may result in CCF or death in early infancy.
(6) Sub-optimal development : Congenital heart disease may result in various
degrees of physical development e.g.:PDA, ASD and in coarctation of aorta.
(7) Recurrent respiratory infections :It occurs due to pulmonary congestion.
(8) Focal sepsis :Focal sepsis of the teeth, tonsils and ears lead to increased
complications and morbidity.
(9) Bacterial endocarditis: may supervene on any of the congenital heart
defects.
(10) Associated anomalies : Down’s syndrome, turner’s syndrome etc.

9. CLASSIFICATION OF CONGENITAL HEART DISEASE

According to the hemodynamic status the disorder can be classified in to four:

A. Disorders with decreased pulmonary blood flow: tetralogy of fallot,

tricuspid atresia
B. Disorders with increased pulmonary blood flow: patent ductus
arteriosis(PDA),atrial septal defect(ASD), ventricular septal defect(VSD),
atrioventricular septal defect(avsd).
C. Obstructive disorders: Coarctation of aorta, aortic stenosis, pulmonary
stenosis
D. Mixed disorders: transposition of the great vessels(TGV),total
anomalous pulmonary venous return(TAPVR),Truncus arteriosus and
hypoplastic left heart syndrome

A.DISORDERS WITH DECREASED PULMONARY BLOOD FLOW:

TETRALOGY OF FALLOT, TRICUSPID ATRESIA

Introduction

Defects involving decreased pulmonary blood flow occur when there is some
obstruction of blood flow to the lungs. As a result of the obstruction ,
pressure in the right side of the heart increases and becomes greater than that
of the left side of the heart. Blood from the higher pressure right side of the
heart then shunts to the lower pressure left side through a structural defect.
Subsequently, deoxygenated blood mixes with oxygenated blood on the left
side of the heart. This mixed blood which is low in oxygen, is pumped via
 the systemic circulation to the body tissue. Disorders with in this
classification includes:

1) Tetralogy of fallot
2) Tricuspid atresia
1) TETRALOGY OF FALLOT
Alternative names:TET,TOF
Definition
Tetralogy of fallot refers to a type of congenital heart defect of conotruncal
family of heat lesion in which the primary defect is an anterior deviation of
the infundibular septum(the mascular septum that seperates the aortic and
the pulmonary outflow).

The consequences of this defects are:

 Overriding aorta(the artery that carries oxygen rich blood to the body) that
is shifted over the right ventricle and ventricular septal defect, instead of
coming out only from the left ventricle.
 Narrowing of the pulmonary out flow tract (the valve and artery that
connect the heart with lungs)
 Ventricular septal defect( hole between the right and left ventricle)
 Thickened mascular wall of the right ventricle (right ventricular
hypertrophy)

Risk factors

Factors that increase the risk for this condition during pregnancy include:

a) Alcoholism in the mother

b) Diabetes
 c) Mother who is over 40 years old
d) Poor nutrition during pregnancy
e) Rubella or other viral illnesses during pregnancy

Pathophysiology

Symptoms

1. Cyanosis is not present at birth, but occurs later in the 1 st year of life. It
becomes more pronounced when the baby is upset and after the ductus
close during the first month of life. It can be seen evidently in the
mucusmembrane of lips, mouth, and pharynx and in fingernails and
toenails.
2. Clubbing of fingers or toes occurs by the age of 1-2 years
3. Usually the infants entire skin is dusky, bluish colour
4. Exercise causes severe dyspnoea. Infants and young children may rest for
a limited time, but then they must rest, infants assume a knee chest
position rather than extending their extremities when they lie down.
5. Paroxysmal dyspnoea attack(anoxic blue spells) may occur during the first
24 month of life and lasts for a few minutes to hours.
6. Difficult feeding
7. Failure to gain weight and poor nutritional status
8. Poor growth and development
9. Mental slowness results from chronic hypoxia

Diagnostic tests

i) Chest X ray: it shows enlarged ventricle on the right side, the large aorta
and the decreased in the size of pulmonary artery
ii) Complete blood count: it shows polycythemia and arterial oxygen
unsaturation
iii) Echocardiogram:it shows evidence of aortic override, thick anterior right
ventricular wall and large aorta
iv) Electrocardiogram:right ventricular hypertrophy
v) MRI of the heart
vi) Cardiac catheterisation:it reveals systolic hypertension in the right
ventricle, with a rapid fall in pressure as the catheter goes into pulmonary
artery

Complication

a) Delayed growth and development

b) Irregular heartrhythm
c) Seizure during periods when there is not enough oxygen
d) Death

Prognosis

1) Most cases can be corrected with surgery

2) Patient who have continued , severe leakiness of the pulmonary valve may
need to have the valve replaced
3) Regular follow up with a cardiologist to monitor for life threatening
arrhythmias

2.TRICUSPID ATRESIA

Alternative name: tri atresia

Definition: Tricuspid atresia is a type of CHD in which the tricuspid heart
valve is missing or abnormally developed. The defect blocks flow from the
right atrium to the right ventricle

Incidence

Tricuspid atresia is an uncommon form of CHD that affect about 5 in every

100,000 live births

About 20% of patients with this condition will also have other heart problem

Pathophysiology

In tricuspid atresia, blood returning from the systemic circulation to the right
atrium cannot directly enter the right ventricle due to agenesis of the
tricuspid valve.

Subsequently ,deoxygenated blood then passes through an opening in the

atrial septum into the left atrium, never entering the pulmonary vasculture.
Thus, deoxygenated blood mixes with oxygenated blood in the left atrium.

The blood then travels to the lungs through a PDA.

The foramen ovale and ductus arteriosus must remain open for the newborn

To maintain minimally adequate oxygenation.

Symptoms are the following

1. Cyanosis: (bluish discoloration of the skin) is evident at birth and

depends upon the limitation of pulmonary artery blood flow.
2. Increased ventricular impulse
3. Holosystolic murmurs audiable along the left sterna boarder
4. Easily fatigability
5. Polycythemia
6. Occasional hypoxia
7. Shortness of birth
8. Fast breathing
 9. Poor growth

Diagnostic evaluation

a. Electrocardiography:Left axis deviation and left ventricular

hypertrophy are generally seen
b. Echocardiogram:it reveals the presence of fibromuscular membrane
in the place of tricuspid valve,variably small right ventricle, VSD and
the large left ventricle and aorta can be evaluated
c. Chest X-ray
d. Cardiac cathetreisation:it shows normal or slightly elevated right
atrial pressure with a prominent a wave
e. MRI of the heart

Complication

i) Irregular, fast heart rhythms(arrhythmias)

ii) Baffle syndrome causing inferior or superior vena cava syndrome
iii) Pulmonary artery thrombosis
iv) Chronic diarrhoea(from a disease called protein loosing enteropathy)
v) Heart failure
vi) Fluid in the abdomen and in the lungs

v)Blockage of the artificial shunt

vi)Strokes and other nuerological complications

vii)sudden death

Prognosis: Improvement can be expected with most surgical procedures.

Unexpected death may occur with heart arrhythmias.

B}DISORDERS WITH INCREASED PULMONARY FLOW

1. Atrial septal defect

2. Ventricular septal defect
3. Patent ductus arteriosis
4. Atrioventricular septal defect
Most congenital heart defects involve increased pulmonary blood flow.
Normally, the left side of the heart has a higher pressure than the right side.
Defects with connections involving the left and right sides will shunt blood
from the higher pressure left side to the lower pressure right side. Even a
small pressure gradient such as a 1-3 mm difference between the left and
right sides will produce a movement of blood from the left to the right. In
turn, the increase of blood on the right side of the heart will cause a greater
amount of blood to move through the heart.If the amount of blood flowing to
the lungs is large, the child may develop heart failure early in life. In
addition, right ventricular hypertrophy may result. Sometimes with
ventricular hypertrophy the right side of the heart pumps so forcefully that
left to right shunting is reversed to right to left shunting. If this occurs,
deoxygenated blood mixes with oxygenated blood, thereby lowering the
overall blood oxygen saturation level. Excessive blood flow to the lungs can
produce a compensatory response such as tachypnea or tachycardia.

1. ATRIAL SEPTAL DEFECT(ASD)

Definition
Atrial septal defect is a congenital heart defect in which the wall that
separates the upper heart chambers does not close completely.
Congenital means the defect is present at birth.
Causes
ASD is not very common. When the person has no other congenital
defect, symptoms may be absent, particularly in children. Symptoms may
begin any time after birth through childhood.
Pathophysiology
 With ASD, blood flows between the openings from the left atrium to the
right atrium due to pressure differences.The shunting increases the blood
volume entering the right atrium. This in turn, leads to increased blood
flow into the lungs. The defect if untreated can cause problems such as
pulmonary hypertension, heart failure, atrial arrhythmias, or stroke.
Symptoms
i) Difficulty breathing
ii) Frequent respiratory infection in children
iii) Shortness of breath with activity

Diagnostic evaluation

i) Physical examination: abnormal heart sounds may be heard while

listening to the chest with a stethoscope.
ii) Cardiac catheterisation
iii) Chest x ray
iv) Coronary angiography
v) Echocardiography
vi) Heart MRI
vii) Trans esophageal echocardiography(TEE)

Complications

a) Arrhymias
b) Heart failure
c) Pulmonary hypertension
d) stroke

2.VENTRICULAR SEPTAL DEFECT

Alternative Names : Ventricular Septal Defect (VSD)
Definition
Ventricular septal defect describes one or more holes in the wall that seperates the
right and left ventricles of the heart. Ventricular septal defect is one of the most
common congenital (present from birth)heart defects. It may occur by itself or with
other congenital diseases.
Causes
The cause of vsd is not yet known. This defect often occurs along with other
congenital heart defects.
Pathophysiology
In VSD, there is an abnormal opening between the right and left ventricles. The
opening varies in size from as small as a pinhole to a complete opening between the
ventricles, so that the right and left sides are as one. Children with small VSDs may
remain asymptomatic in other children, blood shunts across the opening in the septum
pulmonary vascular resistance and systemic vascular resistance determine the
direction of the blood flow. A left to right shunt results when pulmonary vascularate
resistance is low. Increased pulmonary vascular resistance leads to increased
pulmonary artery pressure (pulmonary hypertension) and right ventricular
hypertrophy. When the pulmonary vascular resistance exceeds the systemic vascular
resistance right to left shunting of blood across the VSD occurs resulting in
Eisenmenger’s syndrome (particularly hypertension and cyanosis).Heart failure
commonly occurs in children with moderate to severe VSD. Childrens with VSD are
also at risk for the development of aortic valve regurgitation as well as infective
endocarditis.

Symptoms : Includes the following

 i) Shortness of breath
ii) Fast breathing
iii) Paleness
iv) Failure to gain weight
v) Fast heart rate
vi) Sweating while feeding
vii) Frequent respiratory infection

Diagnostic evaluation

Auscultation usually reveals a heart murmur (the sound of blood crossing the hole)
The loudness of the murmur is related to the size of the defect and amount of blood
crossing the defect.

Tests may include

i) Chest X-Ray : It shows if there is a large heart with fluid in the lungs
ii) Electrocardiography : It shows sign of an enlarged left ventricle
iii) Echocardiogram: It is used to make a definite diagnosis
iv) Cardiac catheterization (rarely needed ,unless there are concerns of high blood
pressure in the lungs)
v) MRI Heart: It is used to find out how much blood is getting to the lungs.

Complications

i) Heart failure
ii) Infective endocarditis (bacterial infection of the heart)
iii) Aortic insufficiency (leaking of the valve that separates the left ventricle
from the aorta)
iv) Damage to the electrical conduction system of the heart during
surgery(causing arrhythmias)
v) Delayed growth and development (failure to thrive in infancy)
vi) Pulmonary hypertension (high blood pressure in the lungs)leading to failure
of the right side of the heart

Prognosis:

Many small defects will close by themselves for those defects that do not
spontaneously close the outcome is good with surgical repair.Complications may
result if a large defect is not treated.
 3.Patent DuctusArteriosus

Alternative name : PDA

Definition: Patent ductus arteriosus (PDA) is a condition in which a blood vessel

called the ductus arteriousus fails to close normally in an infant soon after birth(the
word patent means open)

Pulmonary valve stenosis

Alternative names: valvular pulmonary stenosis, heart valve pulmonary stenosis,

pulmonary stenosis, stenosis pulmonary valve

Definition

Pulmonary valve stenosis is a heart valve disorder that involves the pulmonary
valve. This valve separates the right ventricle and the pulmonary artery. The
pulmonary artery carries oxygen poor blood flows to the lungs.

Stenosis occurs when the valve cannot open wide enough. As a result, less blood
flows to the lungs.

Causes

i) Neonatal respiratory distress

ii) Genetic disorders
iii) Mothers who had rubella during pregnancy

Pathophysiology

Failure of the ductus arteriosis to close leads to continued blood flow from the
aorta to the pulmonary artery. Blood returning to the left atrium passes to the
left Ventricle, enters the aorta, and then travels to the pulmonary artery via the
PDA instead of entering the systemic circulation. This altered blood flow
pattern increases the work load of the left side of the heart. Pulmonary vascular
congestion occurs, causing an increase In pressure. Right ventricular pressure
increases in an attempt to overcome this increase in pulmonary pressure.
Eventually right ventricular hypertrophy occurs.

Symptoms

A small PDA may not cause any symptoms. However, some infants may not
tolerate a PDA, especially if it is large and may have symtoms such as:

1) Bounding pulse
2) Fast breathing
3) Poor feeding habits
4) Shortness of breath
5) Tiring very easily
6) Sweating while feeding
7) Poor growth

Diagnostic evaluation

1. Babies with PDA often have a characteristic heart murmur that can be
heard with a stethoscope
2. Chest X ray
3. Echocardiogram

Complications

If the patent ductus is not closed, the infant has a risk of developing heart
failure, pulmonary artery hypertension or infective endocarditis

4.ATRIOVENTRICULAR SEPTAL DEFECT

 [ENDOCARDIAL CUSHION DEFECT]

Definition

Endocardial cushion defect is an abnormal heart condition in which the walls

separating all chambers of the heart are poorly formed or absent. It is a CHD,
which means it present from birth.

Causes and risk factors

Endoardial cushion defect occurs while a baby is still growing In the womb.
ECD Is strong, associated with Down syndrome. Several gene changes are
also connected to ECD. However. the exact cause of ECD is unknown.

Endocardial cushion defect may be associated with other congenital heart

defects such as:

 Double outlet right ventricle

 Single ventricle
 Transposition of the great vessels
 Tetralogy of fallot.

Pathophysiology

AV canal defect occurs as a rank of failure of endocardial cushions to fuse.

These cushions are needed to separate the central parts of the heart near the
tricuspid and mitral valves. The complete AV canal defect involves atrial and
ventricular septal defects as well as a common AV orifice and a common AV
valve. Partial and transitional forms of AV canal defect also occur. Involving
variations of the complete form.

The complete AV canal defect permits oxygenated blood from the lungs to enter
the left atrium and ventricle, crossing over the atrial or ventricular septum and
returning to the lungs via the pulmonary artery. This recirculation problem,
which typically involves a left-to-right shunt,is inefficient because the left
ventricle must pump blood back to the lungs and also meet the body‘s
peripheral demand for oxygenated blood. Subsequently,the left ventricle must
pump two to three times more blood than in a normal heart. Therefore. this
speciflc type of cardiac defect causes a large left to right shunt; an increased
workload of the left ventricle, and pulmonary arterial pressure, resulting in an
increased amount of blood in the lungs and causing pulmonary edema.
Symptoms
i) Baby tires easily
ii) Bluish skin colour
iii) Failure to gain weight and grow
iv) Lack of appitite
v) Pallor
vi) Rapid breathing
vii) Rapid heartbeat
viii) Sweating
ix) Swollen legs or abdomen
x) Trouble breathing

Diagnostic evaluation

These are includes the following:

1. An abnormal electrocardiogram (ECG)

2. An enlarged heart
3. Heart murmur.

Tests to diagnose ECD include the following:

1. Ultrasound of the heart (echocardiogram) to see blood flow

2. An electrocardiogram (ECG), which measures the electrical activity in the
heart

3.Chest X-ray, which may show an enlarged heart

4.MRI heart, which provides a detailed image of the heart through the use of
powerful magnets

5.Cardiac catheterization (in some cases), a procedure in which a thin tube

(catheter) is placed into the heart to see blood flow and take accurate
measurements of blood pressure and oxygen levels

Complications

1. Congestive heart failure

2. Death
3. Eisenmenger's syndrome
4. High blood pressure in the lungs
5. Irreversible damage to the lungs.

Prognosis

How well the baby does depends on the severity of the ECD, the child's overall
health, and whether lung disease has already developed. Many children live
normal, active lives after the ECD is corrected.

C}OBSTRUCTIVE DISORDERS

Obstructive disorders

Coarctation aortic pulmonary stenosis

Of aorta stenosis
1. COARCTATION OF AORTA
Definition
Aortic coarctation is a narrowing of part of the aorta. It is a type of birth defect.
Coarctation means narrowing.

Pathophysiology
Coarctation of the aorta occurs most often in the area near the ductus arteriosus.
The narrowing can be preductal or postductal .As a result of the narrowing,
blood flow impeded, causing pressure to increase in the area proximal to the
defect and to decrease in the area distal to it.thus blood pressure is increased in
the heart and upper portions of the body and decreased in the lower poetions of
the body.left ventricular afterload is increased and in the some children this
may lead to heart failure .collateral circulation also may develop as the body
attempts to ensure adequate blood flow to the desending aorta. Due to the
elevation in blood pressure the child is also aortic rupture aortic aneurism and
CVA.

2.PULMONARY VALVE STENOSIS

Alternative names; Valvular pulmonary stenosis heart valve pulmonary stenosis

,pulmonary stenosis pulmonary valve.

Definition: Pulmonary valve stenosis is a heart valve disorder that involves the
pulmonary valve.This valve separates the right ventricle (One of the chambers
in the heart) and the pulmonary artery.The pulmonary artery carries oxygen
poor bloods to the lungs.

Stenosis occurs when the valve cannot open wide enough. As a result,less
blood flows to the lungs

Causes and incidence: The cause is unknown,but genetics may play a role, The
defect may occur alone or with other chd

Pathophysiology

Pulmonary stenosis may occur as a muscular obstruction below the pulmonary

valve an obstruction at the valve or a narrowing of the pulmonary artery above
the valve . Valve obstruction is the most common form of pulmonary stenosis.
Normally the pulmonary valve is constructed with three thin and pliable valve
leaflets they spread apart easily allowing the right ventricle to eject blood freely
into the pulmonary artery. The most common problem causing pulmonary
stenosis is that the pulmonary valve leaflets are thickened and fused toghether
along their separation lines causing the obstruction to blood flow The right
ventricle has an additional workload causing the muscle to thickness resulting
the right ventricle hypertrophy and decreased pulmonary blood flow. When the
pulmonary valve is severely obstructed the right ventricle cannot eject sufficient
blood into the pulmonary artery. As a result pressure in the right atrium
increases,which could lead to a repositioning of the foramen ovale if this occurs
deoxygenated blood would pass through the foramen ovale into the left side of
the heart and would then be pumbed to the systemic circulation. In some cases
PDA may be present thus allowing for some compensation by shunting blood
from the aorta to the pulmonary circulation for oxygenation.

Symptoms are the following

 Abdominal distention
  Bluish coloration to the skin(cyanosis) in some patients
 Chest pain
 Fain
 Fatigue
 Poor weight gain of failure to thrive in infants with severe blockage
 Shortness of breath
 Sudden death

Note Patients with mild to moderate blockage may not have any symptoms
.There may be no symptoms until the disorder is severe. Symptoms when
present may get worse with exercise or activity.

Diagnostic Evaluation

Physical examination: One can a heart murmur when listening to the heart
using a stethoscope. Murmurs are lowing whooshing or rasping sound heard
during a heartbeat.

Tests used to diagnose pulmonary stenosis may include:

 Cardiac catheterization
 Chest X ray
 Electrocardiography
 Echocardiogram
 Heart MRI

Treatment: Sometimes treatment may not be needed if the disorder is mild

When there are also other heart defects medications may be used to

 Help blood flow through the heart (prostaglandins)

 Help the heart beat stronger
 Prevent clots (blood thinners)
 Remove excess fluids(water pills)
 Treat abnormal heartbeats and rhy

Complication are the following:

 Cyanosis
 Death
 Heart Failure
  Leaking of blood back into the right ventricle(pulmonary regurgitation)
after repair
 Right ventricular hypertrophy (enlargement)

Prognosis: About one-third of patient with mild stenosis get better one third stay
the same and one –third get worse. The outcome is good with successful surgery
or balloon dilation. Other congenitial heart defects may be factor in the outlook.
Some valves can last for decades. Other wear out and will need to be replaced.

D}MIXED DISORDERS
1) Transposition of great vessets
2) Total ano,nalous pulmonary venous return
3) Trucus arteriosus
4) Hypoplastic left heart syndrome.
Mixed defects are congenital heart defects that involve a mixing
of well-oxygenated blood with poorly oxygenated blood. As a result,
systemic blood flow contains lower oxygen content. Cardiac output is
decreased and heart failure occurs.

TRANSPOSITION OF THE GREAT VESSELS (ARTERIES)

Alternative names TGA, d-TGA “d” indicates dextro positioned aorta

Definition: Transposition of the great vessels (TGV) is a congenital heart

defects in which the two major vessels that carry blood away from the heart- the
aorta and towards the lungs pulmonary artery-are switched (transposed)
Incidence

 Transposition of the great vessel it is a common cyanotic congenital

anomaly accounting for 5% of all CHD
 Most infants with the diagnosis of a cyanotic congenital heart disease
have transposition of the great vessels
 Males are more frequently affected than females
 Causes and risk factors: The cause of most congenital heart defects is
unknown. When associated with other cardiac defects such as pulmonary
stenosis or right aortic arch can be associated with deletion of
chromosome 22q 11( CATCH)22 DiGeorge Syndrome)

Factors in the mother that may increase the risk of this condition include:

 Age over 40
 Alcoholism
 Diabetes
 Poor nutrition during pregnancy(prenatal nutrition)
 Rubella or other viral illness during pregnancy

Pathophysiology

In TGV there is no connection or communication between the pulmonary and

systemic circulation TGV creates a situation in which poorly oxygenated blood
returning to the right atrium and ventricle is then pumped out to the aorta and
back to the body. Oxygenated blood returning from the lungs to the left atrium
and ventricle is then send back to the lungs through the pulmonary artery.
Unless there is a connection somewhere in the circulation where the oxygen rich
and oxygen poor blood can mix all the organs of the body will be poorly
oxygenated. Often the ductusarteriosus remains patent allowing for some
mixing of blood Similaraly if a VSD is also present mixing of blood may occur
and cyanosis will be delayed. However these associated defects can lead to
increases pulmonary blood flow that increases pressure in the pulmonary
circulation This predisposes the child to heart failure

Symptoms

 Cyanosis blueness of the skin is depends upon the amount of admixture

of blood that results from the coexisting defects a extreme cyanosis is
soon after birth.
 Cyanosis infants have dyspoeic and unable to suck
 Hyperapnea occurs as the infant attempt to compensate for the decreased
arterial oxygen saturation
 Clubbing of the fingures or toes
 Growth retarded
 Poor feeding
 Shortness of breath

Diagnostic evaluation: These includes the following

Physical Examination: The healthcare provider may detect a heart murmur on

auscultation. The babys mouth and skin will be cyanotic.

Tests often include the following are as follows

 Cardiac catherization
 Chest X ray may show cardiomegaly and increased pulmonary
vasculature
  Electrocardiography It may be normal at birth later it shows right
ventricular hypertrophy
 Echocardiogram ( if done before birth it is called a fetal echocardiogram)
 Pulse oximetry (to check blood oxygen level)

Complications: These are as follows

 Arrhythmias
 Coronary artery problem
 Heart valve problems

Prognosis: The child symptoms will improve after surgery to correct the defect
Most infant who undergo arterial switch do not have symptoms after syrgery
and live normal lives. If corrective surgery is not performed the life expectancy
is month

NURSING MANAGEMENT OF THE CHILD WITH CONGENITAL

HEART DISEASE

The child with a congenital heart defect has multiple needs and requires
comprehensive multidisciplinary care. Nurses play a key role in helping the
child and family during this intensely stressful time.Nursing care focuses on
improving oxygenation promoting adequate nutrition assisting the child and
family with copying ,prviding postoperative nursing care, preventing infection
and providing patient and family education.An important component of
education involves prepairing the child and parents for discharge.In addition to
the nursing management presented below,refer to nursing process for additional
interventions appropriate for the child with CHD. Individualize nursing care
specific to the childs needs.
1. IMPROVING OXYGENATION

Due to the hemodynamic changes accompanying the underlying structural

defect,oxygenation is key. Provide frequent ongoing assessment of the childs
cardiopulmonary status.Assess airway patency and suction as needed.Position
the child in fowlers or semi-fowlers position to facilitate lung
expansion.Monitor vital signs especially heart and respiratory rates. Monitor the
childs color and oxygen saturation levels closely,using these to guide oxygen
administration.Observe for tachypnea and other signs of respiratory distress
such as nasal flaring grunting and retraction. Auscultate the lungs for
adventitious sounds. Provide humidified supplemental oxygen as
ordered,Warning it to prevent wide temperature fluctuation. Anticipate the need
for assisted ventilation if the child ha difficulty maintaining the airway or
experiences deterioration in oxygenation capacity.List of interventions related to
relief of hypercyanotic spells are given bellows

Relieving hypercyanotic spells

a. Use a calm comforting approach

b. Place the infant or child in a knee-to-chest position
c. Provide supplemental oxygen
d. Administer morphine sulfate(0.1mg/kgIV,IM or SC)
e. Supply IV fluids
f. Administer propranolol(0.1mg/kg IV)
2. PROMOTING ADEQUATE NUTRITION

Adequate nutrition is critical to foster growth and development as well as to

reduce the risk for infection. Children with congenitial heart defects typically
have increased nutritional needs due to the increased energy expenditure
associated with increased cardiac and respiratory workloads.In addition many of
the defects leads to heart failure,which may affect the childs fluid balance status
further increasing the childs energy expenditure.
Nutririons may be provided orally,enterally,orparenterally for example for the
newborn or infant nutrition via breast milk or formula may be provided orally or
via gavage feeding.Breastfeeding is usually associated with decreased energy
expenditure during the act of feeding,Yet some infant in intensive care are not
stable enough to breastfeed.Gavage with breast milk is possible and the use of
human milk fortifier adds additional calories that the infant requires.Formula-
fed infants may also require increased calorie formula which may be achieved
by more concentrated mixing of the formula or through the use of additives
such as polycose or vegetable oil.Consult the nutritionist to determine the
individual infants caloric needs and prescription of appropriate feeding.

Cutting a large hole in the nipple or cross-cutting the nipple decreases the work
of feeding for some infants.Generally nipple feedings should be limited to 20
minute duration as feeding for longer periods results in excess caloric
expenditure Many infants may feed orally for 20 minutes,receiving the
remainder ot that feeding via orogastric or nasogastric tube. Offer older
children small frequent feedings to reduce the amount of energy required to feed
or eat and to prevent overtiring the child.Whenneeded,administer and monitor
total parenteral nutrition as prescribed.

3. ASSISTING THE CHILD AND FAMILY TO COPE

The diagnosis of CHD is especially overwhelming for the child and the
parents.The numerous examination, diagnostic tests and procedures are sources
of stress for the infant or child regardless of age and for the parents.

4. PREVENTING INFECTIONS
Teach parents proper hand hygiene . Provide appropriate dental care . Make sure
the child receives prophylaxis for infective endocarditis as needed. Ensure that
children 24 months or younger who have hemodynamically significant heart
defects receive respiratory syncytial virus prophylaxis as recommended during
respiratory syncytial virus (RSV) season.

5. PROVIDING CARE FOR THE CHILD UNDERGOING CARDIAC

SURGERY

Cardiac surgery may be necessary to correct a congenitial defect or provide

symptomatic relief. The surgery may be planned as an elective procedure or
done as an emergency. Open heart surgery involves an incision ot the heart
muscle to repair the internl structures. This may require cardiopulmonary
bypass. Closed-heart surgery involves structures related to the heart but not the
heart muscle itself and may be performed with or without cardiopulmonary
bypass.

6. PROVIDING PREOPERATIVE CARE

The preoperative nursing assessment complements the history and physical

examination and provides important baseline information for comparison during
the postoperative period. Establish a relationship with the child and parents.

The preoperative physical assessment includes

 Temperature and weight measurement

 Examination of extermities
 Respiratory assessment including respiratory rates work of breathing and
auscultation of the lungs for breath sound obtain any necessary laboratory
and diagnostic tests to establish a baseline. In addition review the results
of any test done previously.Testing may include complete blood count
 (CBC) electrolyte levels clotting studies urinalysis cultures of blood and
other body secreation renal and hepatic function tests chest X ray
electrocardiogram,echocardiogram and cardiac catherization.

In most non emergent cases preoperative assessmsnt is performed in an

outpatient setting and the patient is admitted to the hospital on the day of
surgery. Nursing care during this phase focuses on thorough patient and parent
education. If the surgery is an emergency patient teaching must be done quickly
emphasizing the most important elements of the child care.

Child and parent education typically includes the following topics

 Heart anatomy and its function Including what area is involved with the
defects that is to be corrected
 Events before surgery,Including any testing or preparation such as a skin
scrub
 Location of the child after surgery, Such as a pediatric intensive care unit
Which may include a visit to the unit if appropriate and explanation of the
sights and sound that may be present
 Appeaence of the child after surgery ( equipment or devices used for
monitoring such as O2 administration electrocardiogram
leads,Pulseoximeter chest tubes mechanical ventilation or intravenous
lines.)
 Approximate location of the incision and coverage with dressing
 Postoperative activity level including measures to reduce the risk of
complication, such as coughing and deep-breathing exercises incentive
spirometry early ambulation and leg exercises.
 Nutritional restrictions such as nothing by mouth far a specified time
before surgery and use of intravenous fluids.
 Medication such as anesthesia sedation and analgesics as well as
medication the child is taking now that need to be continued or withheld.
Instruct parents to stop food and liquids at the designated time, depending on
the childs age and to give all as directed. Some medications may be withheld
before surgery If the childs nutritional status is poor or questionable, nutritional
supplementation may be ordered for a period of time preoperatively to ensure
the the child has the best possible nutritional status before surgery When its time
for the child to be transported to the surgical area,allow the parents to
accompany the child as far as possible depending on the institution policy. Also
reinforce with the child that the parents will be present at the bedside when he
or she awaken from surgery.

7. PROVIDING POSTOPERATIVE CARE

The child will usually be transported from the operating room to the intensive
care unit .Depending on the age postoperative stability and type of surgery,the
child may stay in the intensive care unit for several hours up to several days .
Vigilant nursing care aids in the transition of the child and parents after surgery
and reduces the risk of complications.

During the postoperative period the nurse should do the following:

i) Assess vital signs frequently as often as every 1 hour until stable

ii) Assess the color of the skin and mucous membrance check capillary refill
and palpate peripheral pulses
iii) Observe cardiac rate and rhythm via electronic monitoring and ausculate
heart rate rhythm and sound frequently
iv) Monitory hemodynamic status via arterial and /or central venous lines
v) Provide site care and tubing changes according to the institution policy
vi) Ausculate lungs for adventitious diminished or absent breath sounds
vii) Assess oxygen saturation levels via pulse oxymetry and arterial
blood gases as well as work of breathing and level of consciousness
frequently
viii) Administer supplemental oxygen as needed
ix) Monitor mechanical ventilation and suction as ordered
 x) Inspect chest tube functioning noting amount color and character of
drainage
xi) Inspect the dressing (incision and chest tube) for drainage and intactness
reinforce or change the dressing as ordered
xii) Assess the incision for redness irritation,drainage or eparation
xiii) Monitor intake and output hourly
xiv) Maintain accurate intravenous infusion rate,restrict fluids as
ordered tp prevent hypervolemia
xv) Assess for changes in level of consciousness report
restlessness,irritability or seizures
xvi) Obtain ordered laboratory such as digoxin inotropic or vasopressor
agents as ordered watching the child closely for possible adverse effects
xvii) Encourage the child to turn cough deep breathe the incentive
spirometer and splint the incisional are with pillows
xviii) Assess the childs pain level and administer analgesics as ordered :
allow time for the child to rest and sleep
xix) Assist the child to get out of bed as soon as possible and as ordered
xx) Assess daily weights
xxi) Administer small frequent feeding or meals when oral intake is
allowed
xxii) Position the child in a comfortable position on that maximized
chest expansion change position frequently
xxiii) Assess the child for complications
xxiv) Provide emotional and physical support to the child and family
making appropriate referrals such as to social services for assistance
xxv) Prepare the patient and family for discharge

POSSIBLE COMPLICATIONS AFTER CARDIAC SURGERY

1. Atelectasis
2. Bacterial endocarditis
3. Cardiac arrhythmias
4. Cardiac tamponade
5. Cerebrovascular accident
6. Heart failure
 7. Hemorrhage
8. Pleural effusion
9. Pneumonia
10.Pneumothorax
11.Postcardiac surgery syndrome
12.Pulmonary edema
13.Seizures
14.Wound infection
8. PROVIDING PATIENT AND FAMILY EDUCATION

Provide patient and family education throughout the childs stay Initially
teaching focuses on the underlying defect and measures to treat or control the
problems. If the child requires surgery, Teaching shifts to preoperative and
postoperative events. Emphasize discharge teaching for each admission.

BIBLIOGRAPHY

A Padmaja. Text book of child health nursing. Jaypee publication. page no.326-
345