CONGENITAL HEART DISEASE

I. INTRODUCTION

A congenital heart defect (CHD), also known as a congenital heart

anomaly and congenital heart disease, is a defect in the structure of the heart or great
vessels that is present at birth. Signs and symptoms depend on the specific type of defect.
Symptoms can vary from none to life-threatening. When present, symptoms may include
rapid breathing, bluish skin (cyanosis), poor weight gain, and feeling tired. CHD does not
cause chest pain. Most congenital heart problems are not associated with other diseases. A
complication of CHD is heart failure.

The cause of a congenital heart defect is often unknown. Certain cases may be due to
infections during pregnancy such as rubella, use of certain medications or drugs such
as alcohol or tobacco, parents being closely related, or poor nutritional status or obesity in
the mother. Having a parent with a congenital heart defect is also a risk factor. A number of
genetic conditions are associated with heart defects, including Down syndrome, Turner
syndrome, and Marfan syndrome. Congenital heart defects are divided into two main
groups: cyanotic heart defects and non-cyanotic heart defects, depending on whether the
child has the potential to turn bluish in colour. The problems may involve the interior walls
of the heart, the heart valves, or the large blood vessels that lead to and from the heart.

Congenital heart defects are partly preventable through rubella vaccination, the adding
of iodine to salt, and the adding of folic acid to certain food products. Some defects do not
need treatment. Others may be effectively treated with catheter based procedures or heart
surgery. Occasionally a number of operations may be needed, or a heart transplant may be
required. With appropriate treatment, outcomes are generally good, even with complex
problems. Congenital heart defects are the most common birth defect. In 2015, they were
present in 48.9 million people globally. They affect between 4 and 75 per 1,000 live births,
depending upon how they are diagnosed. In about 6 to 19 per 1,000 they cause a moderate to

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 severe degree of problems. Congenital heart defects are the leading cause of birth defect-
related deaths: in 2015, they resulted in 303,300 deaths, down from 366,000 deaths in 1990.

II. ANATOMY AND PHYSIOLOGY OF HEART

INTRODUCTION

The cardiovascular system is a closed system if the heart and blood vessels. The heart
pumps blood through a closed system of blood vessels. Blood vessels allow blood to circulate
to all parts of the body. Arteries usually colored red because oxygen rich, carry blood away
from the heart to capillaries within the tissues. Veins usually colored blue because oxygen
poor, carry blood to the heart from the capillaries. Capillaries are the smallest vessels within
the tissues where gas exchange takes place. The function of the cardiovascular system is to
deliver oxygen and nutrients to the body tissues and remove carbon dioxide and wastes
products. The vital importance of the heart is obvious. If one assumes an average rate of
contraction of 75 contractions per minute, a human heart would contract approximately
108,000 times in one day, more than 39 million times in one year, and nearly 3 billion times
during a 75-year lifespan. Each of the major pumping chambers of the heart ejects
approximately 70 ml blood per contraction in a resting adult. This would be equal to 5.25 liters
of fluid per minute and approximately 14,000 liters per day. Over one year, that would equal
10,000,000 liters or 2.6 million gallons of blood sent through roughly 60,000 miles of vessels.
In order to understand how that happens, it is necessary to understand the anatomy and
physiology of the heart.

LOCATION OF THE HEART

The human heart is located within the thoracic cavity, medially between the lungs in the
space known as the mediastinum. Figure 1 shows the position of the heart within the thoracic
cavity. Within the mediastinum, the heart is separated from the other mediastinal structures by
a tough membrane known as the pericardium, or pericardial sac, and sits in its own space called
the pericardial cavity. The dorsal surface of the heart lies near the bodies of the vertebrae, and
its anterior surface sits deep to the sternum and costal cartilages. The great veins, the superior

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and inferior venae cavae, and the great arteries, the aorta and pulmonary trunk, are attached to
the superior surface of the heart, called the base. The base of the heart is located at the level of
the third costal cartilage, as seen in Figure 1. The inferior tip of the heart, the apex, lies just to
the left of the sternum between the junction of the fourth and fifth ribs near their articulation
with the costal cartilages. The right side of the heart is deflected anteriorly, and the left side is
deflected posteriorly. It is important to remember the position and orientation of the heart when
placing a stethoscope on the chest of a patient and listening for heart sounds, and also when
looking at images taken from a midsagittal perspective. The slight deviation of the apex to the
left is reflected in a depression in the medial surface of the inferior lobe of the left lung, called
the cardiac notch.

Figure 1. The heart is located within the thoracic cavity, medially between the
lungs in the mediastinum. It is about the size of a fist, is broad at the top, and tapers
toward the base.

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CHAMBERS AND CIRCULATION THROUGH THE HEART

The human heart consists of four chambers: The left side and the right side each have
one atrium and one ventricle. Each of the upper chambers, the right atrium (plural = atria) and
the left atrium, acts as a receiving chamber and contracts to push blood into the lower
chambers, the right ventricle and the left ventricle. The ventricles serve as the primary pumping
chambers of the heart, propelling blood to the lungs or to the rest of the body.

There are two distinct but linked circuits in the human circulation called the pulmonary
and systemic circuits. Although both circuits transport blood and everything it carries, we can
initially view the circuits from the point of view of gases. The pulmonary circuit transports
blood to and from the lungs, where it picks up oxygen and delivers carbon dioxide for
exhalation. The systemic circuit transports oxygenated blood to virtually all of the tissues of the
body and returns relatively deoxygenated blood and carbon dioxide to the heart to be sent back
to the pulmonary circulation.

The right ventricle pumps deoxygenated blood into the pulmonary trunk, which leads
toward the lungs and bifurcates into the left and right pulmonary arteries. These vessels in turn
branch many times before reaching the pulmonary capillaries, where gas exchange occurs:
Carbon dioxide exits the blood and oxygen enters. The pulmonary trunk arteries and their
branches are the only arteries in the post-natal body that carry relatively deoxygenated blood.
Highly oxygenated blood returning from the pulmonary capillaries in the lungs passes through
a series of vessels that join together to form the pulmonary veins—the only post-natal veins in
the body that carry highly oxygenated blood. The pulmonary veins conduct blood into the left
atrium, which pumps the blood into the left ventricle, which in turn pumps oxygenated blood
into the aorta and on to the many branches of the systemic circuit. Eventually, these vessels
will lead to the systemic capillaries, where exchange with the tissue fluid and cells of the body
occurs. In this case, oxygen and nutrients exit the systemic capillaries to be used by the cells in
their metabolic processes, and carbon dioxide and waste products will enter the blood.

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 The blood exiting the systemic capillaries is lower in oxygen concentration than when it
entered. The capillaries will ultimately unite to form venules, joining to form ever-larger veins,
eventually flowing into the two major systemic veins, the superior vena cava and the inferior
vena cava, which return blood to the right atrium. The blood in the superior and inferior venae
cavae flows into the right atrium, which pumps blood into the right ventricle. This process of
blood circulation continues as long as the individual remains alive. Understanding the flow of
blood through the pulmonary and systemic circuits is critical to all health professions.

Figure 3. Blood flows from the right atrium to the right ventricle, where it is
pumped into the pulmonary circuit.

The blood in the pulmonary artery branches is low in oxygen but relatively high in
carbon dioxide. Gas exchange occurs in the pulmonary capillaries (oxygen into the blood,
carbon dioxide out), and blood high in oxygen and low in carbon dioxide is returned to the
left atrium. From here, blood enters the left ventricle, which pumps it into the systemic
circuit. Following exchange in the systemic capillaries (oxygen and nutrients out of the

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 capillaries and carbon dioxide and wastes in), blood returns to the right atrium and the
cycle is repeated.

MEMBRANES, SURFACE FEATURES, AND LAYERS

The exploration of more in-depth heart structures begins by examining the membrane
that surrounds the heart, the prominent surface features of the heart, and the layers that form
the wall of the heart. Each of these components plays its own unique role in terms of function.

Membranes

Figure 4. The pericardial membrane that surrounds the heart consists of three layers and
the pericardial cavity. The heart wall also consists of three layers. The pericardial
membrane and the heart wall share the epicardium.

The membrane that directly surrounds the heart and defines the pericardial cavity is
called the pericardium or pericardial sac. It also surrounds the “roots” of the major vessels,
or the areas of closest proximity to the heart. The pericardium, which literally translates as
“around the heart,” consists of two distinct sublayers: the sturdy outer fibrous pericardium
and the inner serous pericardium. The fibrous pericardium is made of tough, dense

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 connective tissue that protects the heart and maintains its position in the thorax. The more
delicate serous pericardium consists of two layers: the parietal pericardium, which is fused
to the fibrous pericardium, and an inner visceral pericardium, or epicardium, which is
fused to the heart and is part of the heart wall. The pericardial cavity, filled with
lubricating serous fluid, lies between the epicardium and the pericardium.

In most organs within the body, visceral serous membranes such as the epicardium
are microscopic. However, in the case of the heart, it is not a microscopic layer but rather a
macroscopic layer, consisting of a simple squamous epithelium called a mesothelium,
reinforced with loose, irregular, or areolar connective tissue that attaches to the
pericardium. This mesothelium secretes the lubricating serous fluid that fills the pericardial
cavity and reduces friction as the heart contracts.

SURFACE FEATURES OF THE HEART

Inside the pericardium, the surface features of the heart are visible, including the four
chambers. There is a superficial leaf-like extension of the atria near the superior surface of the
heart, one on each side, called an auricle—a name that means “ear like”—because its shape
resembles the external ear of a human (Figure 5). Auricles are relatively thin-walled structures
that can fill with blood and empty into the atria or upper chambers of the heart. You may also
hear them referred to as atrial appendages. Also prominent is a series of fat-filled grooves, each
of which is known as a sulcus (plural = sulci), along the superior surfaces of the heart. Major
coronary blood vessels are located in these sulci. The deep coronary sulcus is located between
the atria and ventricles. Located between the left and right ventricles are two additional sulci
that are not as deep as the coronary sulcus. The anterior interventricular sulcus is visible on the
anterior surface of the heart, whereas the posterior interventricular sulcus is visible on the
posterior surface of the heart. Figure 5 illustrates anterior and posterior views of the surface of
the heart.

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Figure 5. Inside the pericardium, the surface features of the heart are visible.

Layers

The wall of the heart is composed of three layers of unequal thickness. From
superficial to deep, these are the epicardium, the myocardium, and the
endocardium. The outermost layer of the wall of the heart is also the innermost
layer of the pericardium, the epicardium, or the visceral pericardium discussed
earlier.

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 Figure 6. The swirling pattern of cardiac muscle tissue contributes significantly to
the heart’s ability to pump blood effectively.

The middle and thickest layer is the myocardium, made largely of cardiac muscle cells.
It is built upon a framework of collagenous fibers, plus the blood vessels that supply the
myocardium and the nerve fibers that help regulate the heart. It is the contraction of the
myocardium that pumps blood through the heart and into the major arteries. The muscle pattern
is elegant and complex, as the muscle cells swirl and spiral around the chambers of the heart.
They form a figure 8 pattern around the atria and around the bases of the great vessels. Deeper
ventricular muscles also form a figure 8 around the two ventricles and proceed toward the

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apex. More superficial layers of ventricular muscle wrap around both ventricles. This complex
swirling pattern allows the heart to pump blood more effectively than a simple linear pattern
would. Figure 6 illustrates the arrangement of muscle cells.

Although the ventricles on the right and left sides pump the same amount of blood per
contraction, the muscle of the left ventricle is much thicker and better developed than that of
the right ventricle. In order to overcome the high resistance required to pump blood into the
long systemic circuit, the left ventricle must generate a great amount of pressure. The right
ventricle does not need to generate as much pressure, since the pulmonary circuit is shorter and
provides less resistance. The image below illustrates the differences in muscular thickness
needed for each of the ventricles.

Figure 7. The myocardium in the left ventricle is significantly thicker than that of
the right ventricle. Both ventricles pump the same amount of blood, but the left
ventricle must generate a much greater pressure to overcome greater resistance in
the systemic circuit.

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 The ventricles are shown in both relaxed and contracting states. Note the differences in
the relative size of the lumens, the region inside each ventricle where the blood is contained.

The innermost layer of the heart wall, the endocardium, is joined to the myocardium
with a thin layer of connective tissue. The endocardium lines the chambers where the blood
circulates and covers the heart valves. It is made of simple squamous epithelium
called endothelium, which is continuous with the endothelial lining of the blood vessels.

Once regarded as a simple lining layer, recent evidence indicates that the endothelium of the
endocardium and the coronary capillaries may play active roles in regulating the contraction of
the muscle within the myocardium. The endothelium may also regulate the growth patterns of
the cardiac muscle cells throughout life, and the endothelins it secretes create an environment
in the surrounding tissue fluids that regulates ionic concentrations and states of contractility.
Endothelins are potent vasoconstrictors and, in a normal individual, establish a homeostatic
balance with other vasoconstrictors and vasodilator

INTERNAL STRUCTURE OF THE HEART

Recall that the heart’s contraction cycle follows a dual pattern of circulation—the pulmonary
and systemic circuits—because of the pairs of chambers that pump blood into the circulation.
In order to develop a more precise understanding of cardiac function, it is first necessary to
explore the internal anatomical structures in more detail.

SEPTA OF THE HEART

The word septum is derived from the Latin for “something that encloses;” in this case,
a septum (plural = septa) refers to a wall or partition that divides the heart into chambers. The
septa are physical extensions of the myocardium lined with endocardium. Located between the
two atria is the interatrial septum. Normally in an adult heart, the interatrial septum bears an
oval-shaped depression known as the fossa ovalis, a remnant of an opening in the fetal heart
known as the foramen ovale. The foramen ovale allowed blood in the fetal heart to pass
directly from the right atrium to the left atrium, allowing some blood to bypass the pulmonary

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circuit. Within seconds after birth, a flap of tissue known as the septum primum that previously
acted as a valve closes the foramen ovale and establishes the typical cardiac circulation pattern.

Between the two ventricles is a second septum known as the interventricular septum. Unlike
the interatrial septum, the interventricular septum is normally intact after its formation during
fetal development. It is substantially thicker than the interatrial septum, since the ventricles
generate far greater pressure when they contract.

The septum between the atria and ventricles is known as the atrioventricular septum. It
is marked by the presence of four openings that allow blood to move from the atria into the
ventricles and from the ventricles into the pulmonary trunk and aorta. Located in each of these
openings between the atria and ventricles is a valve, a specialized structure that ensures one-
way flow of blood. The valves between the atria and ventricles are known generically
as atrioventricular valves. The valves at the openings that lead to the pulmonary trunk and aorta
are known generically as semilunar valves. The interventricular septum is visible in the image
below. In this figure, the atrioventricular septum has been removed to better show the bicupid
and tricuspid valves; the interatrial septum is not visible, since its location is covered by the
aorta and pulmonary trunk. Since these openings and valves structurally weaken the
atrioventricular septum, the remaining tissue is heavily reinforced with dense connective tissue
called the cardiac skeleton, or skeleton of the heart. It includes four rings that surround the
openings between the atria and ventricles, and the openings to the pulmonary trunk and aorta,
and serve as the point of attachment for the heart valves. The cardiac skeleton also provides an
important boundary in the heart electrical conduction system.

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Figure 8. This anterior view of the heart shows the four chambers, the major
vessels and their early branches, as well as the valves. The presence of the
pulmonary trunk and aorta covers the interatrial septum, and the atrioventricular
septum is cut away to show the atrioventricular valves.

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 III. DISORDERS OF THE HEART: HEART DEFECTS

One very common form of interatrial septum pathology is patent foramen ovale, which
occurs when the septum primum does not close at birth, and the fossa ovalis is unable to
fuse. The word patent is from the Latin root patens for “open.” It may be benign or
asymptomatic, perhaps never being diagnosed, or in extreme cases, it may require surgical
repair to close the opening permanently. As much as 20–25 percent of the general
population may have a patent foramen ovale, but fortunately, most have the benign,
asymptomatic version. Patent foramen ovale is normally detected by auscultation of a heart
murmur (an abnormal heart sound) and confirmed by imaging with an echocardiogram.
Despite its prevalence in the general population, the causes of patent ovale are unknown,
and there are no known risk factors. In nonlife-threatening cases, it is better to monitor the
condition than to risk heart surgery to repair and seal the opening.

Coarctation of the aorta is a congenital abnormal narrowing of the aorta that is

normally located at the insertion of the ligamentum arteriosum, the remnant of the fetal
shunt called the ductus arteriosus. If severe, this condition drastically restricts blood flow
through the primary systemic artery, which is life threatening. In some individuals, the
condition may be fairly benign and not detected until later in life. Detectable symptoms in
an infant include difficulty breathing, poor appetite, trouble feeding, or failure to thrive. In
older individuals, symptoms include dizziness, fainting, shortness of breath, chest pain,
fatigue, headache, and nosebleeds. Treatment involves surgery to resect (remove) the
affected region or angioplasty to open the abnormally narrow passageway. Studies have
shown that the earlier the surgery is performed, the better the chance of survival.

A patent ductus arteriosus is a congenital condition in which the ductus arteriosus

fails to close. The condition may range from severe to benign. Failure of the ductus
arteriosus to close results in blood flowing from the higher pressure aorta into the lower
pressure pulmonary trunk. This additional fluid moving toward the lungs increases
pulmonary pressure and makes respiration difficult. Symptoms include shortness of breath
(dyspnea), tachycardia, enlarged heart, a widened pulse pressure, and poor weight gain in
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infants. Treatments include surgical closure (ligation), manual closure using platinum coils
or specialized mesh inserted via the femoral artery or vein, or nonsteroidal anti-
inflammatory drugs to block the synthesis of prostaglandin E2, which maintains the vessel
in an open position. If untreated, the condition can result in congestive heart failure.

Septal defects are not uncommon in individuals and may be congenital or caused
by various disease processes. Tetralogy of Fallot is a congenital condition that may also
occur from exposure to unknown environmental factors; it occurs when there is an opening
in the interventricular septum caused by blockage of the pulmonary trunk, normally at the
pulmonary semilunar valve. This allows blood that is relatively low in oxygen from the
right ventricle to flow into the left ventricle and mix with the blood that is relatively high
in oxygen. Symptoms include a distinct heart murmur, low blood oxygen percent
saturation, dyspnea or difficulty in breathing, polycythemia, broadening (clubbing) of the
fingers and toes, and in children, difficulty in feeding or failure to grow and develop. It is
the most common cause of cyanosis following birth. The term “tetralogy” is derived from
the four components of the condition, although only three may be present in an individual
patient: pulmonary infundibular stenosis (rigidity of the pulmonary valve), overriding aorta
(the aorta is shifted above both ventricles), ventricular septal defect (opening), and right
ventricular hypertrophy (enlargement of the right ventricle). Other heart defects may also
accompany this condition, which is typically confirmed by echocardiography imaging.
Tetralogy of Fallot occurs in approximately 400 out of one million live births. Normal
treatment involves extensive surgical repair, including the use of stents to redirect blood
flow and replacement of valves and patches to repair the septal defect, but the condition
has a relatively high mortality. Survival rates are currently 75 percent during the first year
of life; 60 percent by 4 years of age; 30 percent by 10 years; and 5 percent by 40 years.

In the case of severe septal defects, including both tetralogy of Fallot and patent
foramen ovale, failure of the heart to develop properly can lead to a condition commonly
known as a “blue baby.” Regardless of normal skin pigmentation, individuals with this

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 condition have an insufficient supply of oxygenated blood, which leads to cyanosis, a blue
or purple coloration of the skin, especially when active.

Septal defects are commonly first detected through auscultation, listening to the
chest using a stethoscope. In this case, instead of hearing normal heart sounds attributed to
the flow of blood and closing of heart valves, unusual heart sounds may be detected. This
is often followed by medical imaging to confirm or rule out a diagnosis. In many cases,
treatment may not be needed. Some common congenital heart defects are illustrated in
Figure 9.

Figure 9. (a) A patent foramen ovale defect is an abnormal opening in the

interatrial septum, or more commonly, a failure of the foramen ovale to close. (b)
Coarctation of the aorta is an abnormal narrowing of the aorta. (c) A patent ductus
arteriosus is the failure of the ductus arteriosus to close. (d) Tetralogy of Fallot includes an
abnormal opening in the interventricular septum.

Right Atrium

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 The right atrium serves as the receiving chamber for blood returning to the heart from
the systemic circulation. The two major systemic veins, the superior and inferior venae cavae,
and the large coronary vein called the coronary sinus that drains the heart myocardium empty
into the right atrium. The superior vena cava drains blood from regions superior to the
diaphragm: the head, neck, upper limbs, and the thoracic region. It empties into the superior
and posterior portions of the right atrium. The inferior vena cava drains blood from areas
inferior to the diaphragm: the lower limbs and abdominopelvic region of the body. It, too,
empties into the posterior portion of the atria, but inferior to the opening of the superior vena
cava. Immediately superior and slightly medial to the opening of the inferior vena cava on the
posterior surface of the atrium is the opening of the coronary sinus. This thin-walled vessel
drains most of the coronary veins that return systemic blood from the heart. The majority of the
internal heart structures discussed in this and subsequent sections are illustrated in Figure 8.

While the bulk of the internal surface of the right atrium is smooth, the depression of the
fossa ovalis is medial, and the anterior surface demonstrates prominent ridges of muscle called
the pectinate muscles. The right auricle also has pectinate muscles. The left atrium does not
have pectinate muscles except in the auricle.

The atria receive venous blood on a nearly continuous basis, preventing venous flow
from stopping while the ventricles are contracting. While most ventricular filling occurs while
the atria are relaxed, they do demonstrate a contractile phase and actively pump blood into the
ventricles just prior to ventricular contraction. The opening between the atrium and ventricle is
guarded by the tricuspid valve.

Right Ventricle

The right ventricle receives blood from the right atrium through the tricuspid valve.
Each flap of the valve is attached to strong strands of connective tissue, the chordae tendineae,
literally “tendinous cords,” or sometimes more poetically referred to as “heart strings.” There
are several chordae tendineae associated with each of the flaps. They are composed of
approximately 80 percent collagenous fibers with the remainder consisting of elastic fibers and

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endothelium. They connect each of the flaps to a papillary muscle that extends from the
inferior ventricular surface. There are three papillary muscles in the right ventricle, called the
anterior, posterior, and septal muscles, which correspond to the three sections of the valves.

When the myocardium of the ventricle contracts, pressure within the ventricular
chamber rises. Blood, like any fluid, flows from higher pressure to lower pressure areas, in this
case, toward the pulmonary trunk and the atrium. To prevent any potential backflow, the
papillary muscles also contract, generating tension on the chordae tendineae. This prevents the
flaps of the valves from being forced into the atria and regurgitation of the blood back into the
atria during ventricular contraction.The image below shows papillary muscles and chordae
tendineae attached to the tricuspid valve.

Figure 10. In this frontal section, you can see papillary muscles attached to the
tricuspid valve on the right as well as the mitral valve on the left via chordae
tendineae. (credit: modification of work by “PV KS”/flickr.com)

The walls of the ventricle are lined with trabeculae carneae, ridges of cardiac muscle
covered by endocardium. In addition to these muscular ridges, a band of cardiac muscle, also
covered by endocardium, known as the moderator band reinforces the thin walls of the right
ventricle and plays a crucial role in cardiac conduction. It arises from the inferior portion of the
interventricular septum and crosses the interior space of the right ventricle to connect with the
inferior papillary muscle.

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 When the right ventricle contracts, it ejects blood into the pulmonary trunk, which
branches into the left and right pulmonary arteries that carry it to each lung. The superior
surface of the right ventricle begins to taper as it approaches the pulmonary trunk. At the base
of the pulmonary trunk is the pulmonary semilunar valve that prevents backflow from the
pulmonary trunk.

Left Atrium

After exchange of gases in the pulmonary capillaries, blood returns to the left atrium
high in oxygen via one of the four pulmonary veins. While the left atrium does not contain
pectinate muscles, it does have an auricle that includes these pectinate ridges. Blood flows
nearly continuously from the pulmonary veins back into the atrium, which acts as the receiving
chamber, and from here through an opening into the left ventricle. Most blood flows passively
into the heart while both the atria and ventricles are relaxed, but toward the end of the
ventricular relaxation period, the left atrium will contract, pumping blood into the ventricle.
This atrial contraction accounts for approximately 20 percent of ventricular filling. The
opening between the left atrium and ventricle is guarded by the mitral valve.

Left Ventricle

Recall that, although both sides of the heart will pump the same amount of blood, the
muscular layer is much thicker in the left ventricle compared to the right. Like the right
ventricle, the left also has trabeculae carneae, but there is no moderator band. The mitral valve
is connected to papillary muscles via chordae tendineae. There are two papillary muscles on the
left—the anterior and posterior—as opposed to three on the right.

The left ventricle is the major pumping chamber for the systemic circuit; it ejects blood into the
aorta through the aortic semilunar valve.

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 Figure 11. With the atria and major vessels removed, all four valves are clearly
visible, although it is difficult to distinguish the three separate cusps of the
tricuspid valve.

A transverse section through the heart slightly above the level of the atrioventricular
septum reveals all four heart valves along the same plane (Figure 11). The valves ensure
unidirectional blood flow through the heart. Between the right atrium and the right ventricle is
the right atrioventricular valve, or tricuspid valve. It typically consists of three flaps, or leaflets,
made of endocardium reinforced with additional connective tissue. The flaps are connected by
chordae tendineae to the papillary muscles, which control the opening and closing of the
valves.

Emerging from the right ventricle at the base of the pulmonary trunk is the pulmonary
semilunar valve, or the pulmonary valve; it is also known as the pulmonic valve or the right
semilunar valve. The pulmonary valve is comprised of three small flaps of endothelium

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reinforced with connective tissue. When the ventricle relaxes, the pressure differential causes
blood to flow back into the ventricle from the pulmonary trunk. This flow of blood fills the
pocket-like flaps of the pulmonary valve, causing the valve to close and producing an audible
sound. Unlike the atrio ventricular valves, there are no papillary muscles or chordae tendineae
associated with the pulmonary valve.

Located at the opening between the left atrium and left ventricle is the mitral valve, also
called the bicuspid valve or the left atrioventricular valve. Structurally, this valve consists of
two cusps, known as the anterior medial cusp and the posterior medial cusp, compared to the
three cusps of the tricuspid valve. In a clinical setting, the valve is referred to as the mitral
valve, rather than the bicuspid valve. The two cusps of the mitral valve are attached by chordae
tendineae to two papillary muscles that project from the wall of the ventricle.

At the base of the aorta is the aortic semilunar valve, or the aortic valve, which prevents
backflow from the aorta. It normally is composed of three flaps. When the ventricle relaxes and
blood attempts to flow back into the ventricle from the aorta, blood will fill the cusps of the
valve, causing it to close and producing an audible sound.

In the image above, the two atrioventricular valves are open and the two semilunar
valves are closed. This occurs when both atria and ventricles are relaxed and when the atria
contract to pump blood into the ventricles. The image below shows a frontal view. Although
only the left side of the heart is illustrated, the process is virtually identical on the right.

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 Figure 12. (a) A transverse section through the heart illustrates the four heart valves.
The two atrioventricular valves are open; the two semilunar valves are closed. The atria and
vessels have been removed. (b) A frontal section through the heart illustrates blood flow
through the mitral valve. When the mitral valve is open, it allows blood to move from the left
atrium to the left ventricle. The aortic semilunar valve is closed to prevent backflow of blood
from the aorta to the left ventricle.

Image above shows the atrioventricular valves closed while the two semilunar valves
are open. This occurs when the ventricles contract to eject blood into the pulmonary trunk and
aorta. Closure of the two atrioventricular valves prevents blood from being forced back into the
atria. This stage can be seen from a frontal view in image b above.

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 Figure 13. (a) A transverse section through the heart illustrates the four heart valves
during ventricular contraction. The two atrioventricular valves are closed, but the two
semilunar valves are open. The atria and vessels have been removed. (b) A frontal view shows
the closed mitral (bicuspid) valve that prevents backflow of blood into the left atrium. The
aortic semilunar valve is open to allow blood to be ejected into the aorta.

When the ventricles begin to contract, pressure within the ventricles rises and blood
flows toward the area of lowest pressure, which is initially in the atria. This backflow causes
the cusps of the tricuspid and mitral (bicuspid) valves to close. These valves are tied down to
the papillary muscles by chordae tendineae. During the relaxation phase of the cardiac cycle,
the papillary muscles are also relaxed and the tension on the chordae tendineae is slight (image
b above). However, as the myocardium of the ventricle contracts, so do the papillary muscles.
This creates tension on the chordae tendineae (image b above), helping to hold the cusps of the
atrioventricular valves in place and preventing them from being blown back into the atria.

The aortic and pulmonary semilunar valves lack the chordae tendineae and papillary
muscles associated with the atrioventricular valves. Instead, they consist of pocket-like folds of
endocardium reinforced with additional connective tissue. When the ventricles relax and the

23
change in pressure forces the blood toward the ventricles, the blood presses against these cusps
and seals the openings

IV. 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.

24
 V. FETAL CIRCULATION

The blood that flows through the fetus is actually more complicated than after the baby
is born (normal heart). This is because the mother (the placenta) is doing the work that the
baby’s lungs will do after birth.

25
 The placenta accepts the bluest blood (blood without oxygen) from the fetus through
blood vessels that leave the fetus through the umbilical cord (umbilical arteries, there are two
of them). When blood goes through the placenta it picks up oxygen and becomes red. The red
blood then returns to the fetus via the third vessel in the umbilical cord (umbilical vein). The
red blood that enters the fetus passes through the fetal liver and enters the right side of the
heart. The red blood goes through one of the two extra connections in the fetal heart that will
close after the baby is born. The hole between the top two heart chambers (right and left
atrium) is called a patent foramen ovale (PFO). This hole allows the reddest blood to go from
the right atrium to left atrium and then to the left ventricle and out the aorta. As a result the
blood with the most oxygen gets to the brain.Blood coming back from the foetus’s body also
enters the right atrium, but the fetus is able to send this blue blood from the right atrium to the
right ventricle (the chamber that normally pumps blood to the lungs). Most of the blood that
leaves the right ventricle in the fetus bypasses the lungs through the second of the two extra
fetal connections known as the ductus arteriosus.

The ductus arteriosus sends the bluer blood to the organs in the lower half of the fetal
body. This also allows for the bluest blood to leave the fetus through the umbilical arteries and
get back to the placenta to pick up oxygen.Since the patent foramen ovale and ductus arteriosus
are normal findings in the fetus, it is impossible to predict whether or not these connections
will close normally after birth in a normal fetal heart. These two bypass pathways in the fetal
circulation make it possible for most fetuses to survive pregnancy even when there are complex
heart problems and not be affected until after birth when these pathways begin to close.

VI. DISORDERS OF CARDIOVASCULAR SYSTEM

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

26
I. 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.

27
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.

6. PATHOPHYSIOLOGY
 The exact cause is unknown. However, true 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

28
 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

29
 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

30
 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

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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 muscular 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

32
c) Mother who is over 40 years old
d) Poor nutrition during pregnancy
e) Rubella or other viral illnesses during pregnancy

Pathophysiology

Normally the blood (deoxygenated) flows in right side of heart and oxygenated.
blood (bright red in colour) flows in left side of the heart, but in the condition tetralogy of
fallot. The deoxygenated blood (blue in colour) flows towards the left ventricle through the
ventricle septal defect and due to the pulmonary stenosis. This results in mixing of blood
and cause cyanosis, It represents the right to left shunt. This mix blood enters in the aorta
and leads cyanosis. The haemo dynamic (pressure) condition depends on the degree of
pulmonary stenosis, size of the ventricular septal defect (VSD) and pulmonary and
systemic resistance to flow. Because the VSD is usually large, pressure may be equal in the
right and left ventricles. So shunt direction depends on the difference between pulmonary
and systemic vascular resistance. If pulmonary vascular resistance is higher than systemic
resistance, the shunt is from right to left. If systemic resistance is higher than pulmonary
resistance, the shunt is from left to right. Pulmonary stenosis decreases blood flow to the
lungs
and it decreases the amount of oxygenated blood which return to the left side of the heart.
According to the position of aorta, blood from both ventricles may be distributed to
systemic circulation and leads cyanosis.
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 mucus membrane 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
33
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) History collection
ii) Physical examination: It involves the listening of heart and lungs
iii) Chest X ray: it shows enlarged ventricle on the right side, the large aorta and the
decreased in the size of pulmonary artery
iv) Complete blood count: it shows polycythemia and arterial oxygen instaurations
v) Echocardiogram: It shows evidence of aortic override, thick anterior right
ventricular wall and large aorta
vi) Electrocardiogram: Right ventricular hypertrophy
vii) MRI of the heart
viii) Cardiac catheterisation: It reveals systolic hypertension in the right
ventricle, with a rapid fall in pressure as the catheter goes into pulmonary artery
Management
a. Medical Management : The child should be treated for cyanosis and hypoxic spells.
i) Treat dehydration, anemia.
ii) Oxygen therapy.
iii) Place the baby in knee chest position to treat hypoxic spells. Provide I.V. fluids.

34
 iv) Provide intravenous administration of vasopressors such as methoxamine.
v) Prostaglamdin E, intravenously for severe tetralogy of fallot which produce
dilatation of the ductus and increase pulmonary blood flow.
vi) Treatment of acidosis.

b. Surgical Management of TOF

(i) Palliative shunt : This is used for infants who cannot undergo primary
repair. In this, a palliative procedure to increase pulmonary blood flow and increase
oxygen saturation may be performed. The preferred procedure is
Blalock Taussing or Modified Blalock Taussing Shunt : This shunt provides blood
flow to the pulmonary arteries from the left or right subclavin artery.
(ii) Complete repair (elective surgery) : It is usually performed in the first year of
life. Indications for repair include increasing cyanosis and the development of hyper
cyanotic spells. Complete repair involves closure of the VSD and resection of the
infundibular stenosis, with a pericardial patch to enlarge the right ventricular out flow
tract. The procedure requires a median sternotomy and the use of cardio-pulmonary
bypass.
Complications

a) Delayed growth and development

b) Irregular heart rhythm
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

35
 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

36
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

Management

37
a. Medical management
In the neonate whose pulmonary blood flow depends on the patency of the ductus
arteriosus, a continuous infusion of prostaglandin E, started at 0.1 mg/kg of body
weight/ min until surgical treatment can be followed.
b. Surgical management: Palliative treatment is the placement of a shunt (pulmonary to
systemic artery anastomosis) to increase blood flow to the lungs. If the ASD is small, an
atrial septostomy is done during cardiac catheterization. Some children have increased
pulmonary blood flow and require pulmonary artery banding to lessen the volume of
blood to the lungs. A bidirectional Glenn shunt (cavopulmonry anastomosis) may be
performed at 6 to 9 months as a second stage.
c. Modified fontan procedure : In this procedure systemic venous return is directed to the
lungs without a ventricular pump through surgical connections between the right atrium
and pulmonary artery. A fenestrations (opening) in the right atrial baffle is some times
done to relieve pressure. The patient must have normal ventricular functions and a low
pulmonary vascular resistance for the procedure to be successful. The modified fontan
procedure separate oxygenated and unoxygenated blood inside the heart and eliminates
the excess volume load on the ventricle but does not restore normal anatomy.
d. Supportive management :Provide psychological support to the child and parents
before and after the surgery. Educate the parents about the care of child and involve
them in care of child or during any procedure.

Complications

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

38
 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.

39
1. ATRIAL SEPTAL DEFECT(ASD)

Definition
Atrial septal defect (ASD) is an congenital (birth it self) heart defect in
Which abnormal opening between the right and left atrium, which allowing the blood to
flow from higher pressure left atrium to lower pressure right atrium, due to this it is
also known as left-right shunt.
The opening can be three types:
A) Ostium primum (ASDL) :When opening at lower end of septum, it can be associated
with mitral valve abnormalities.
B)Ostium Secondum (ASD 2) :When opening near center of septum.
C) Sinus venosus defect : When opening near junction of superior vena cava and right
atrium.
Incidence :It is about 9% of all CHD's in children. 90% of ASD are due to persistent of
ostium secundum.
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

40
 In heart left atrial pressure is little more than right atrial pressure and blood flow from
the left to right atrium and cause oxygenated blood flow into the right side of heart.
Blood enter in right atrium because it also have great distensibility power. Blood
volume is well tolerated by the right ventricle because it is delivered under much lower
pressure than a ventricular septal defect, as there is right atrial and ventricular
enlargement, cardiac failure is unusual in ASD.

Clinical features

The child may be asymptomatic

i) Congestive heart failure

ii) Heart murmur
iii) Risk of dysrhythmias
iv) Fatigue
v) Poor growth and weight gain
vi) Cardiac enlargement
vii) dyspnoea

Diagnostic evaluation

a. Closed physical examination : Listen the heart and lung sound which shows murmur
sound.
b. Chest-x-ray: It shows right atrium and right ventricle dilation because of more amount
of blood from left side.
c. Electrocardiogram (ECG) : It shows electrical activity of heart and present
dysrhythmias or abnormal rhythm of heart.
d. Echocardiogram : This procedure shows the anatomy and physiology of heart by
producing sound waves, total structure we can see on monitor like septal defect size,
position, flow of blood through the opening etc. Now a days, colour doppler studies are
available which gives more clear view.

41
 e. Cardiac catheterization : A catheter is passed in heart through the blood vessel in
groin and contrast dye is injected through the catheter to visualize the heart defects and
picture is taken to view the result.
Management
a. Medical Management : Congestive heart failure and arrhythmias or dysarhythmias
should be treated with medications such digoxin which strengthen the heart muscles for
regular pumping function. Antibiotics to prevent infections and diuretics for remove the
excess fluid by the kidney from the body.
b. Surgical Management :
i.Surgical management include-patch closure (pericardial patch or dacron patch) of
moderate to large defects similar to closure of ventricular septal defects.
ii.Open repair with cardio pulmonary bypass surgery is usually performed before
school age. The sinus venosus defect requires patch placement, so the anomalous right
pulmonary venous return is directed to the left atrium with a baffle.
iii.The ASD 1 (ostium primum) may require mitral valve repair of replacement of
mitral valve( rarely)
iv. The ASD2 ( ostium secondum) may also be closed using devices during cardiac
catheterization.
Complications

a) Arrhythmias
b) Heart failure
c) Pulmonary hypertension
d) Stroke

2.VENTRICULAR SEPTAL DEFECT

Alternative Names : Ventricular Septal Defect (VSD)

42
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
During the first 8 weeks of fetal development, there is formation of hollow tube
which laterly devides in two seperate parts right side and left side by a septum, If any
opening occur in septum during partition leads ventricle septal defects. Some other cause
like gene defect, chromosomal abnormalities and environmental factors are also have
influence to develop congenital heart defects.

Pathophysiology

In presence of a ventricular septal defect, a portion of oxygenated blood returning

from lungs into the left atrium and left ventricle crosses the ventricular septal defect and
enters the right ventricle, from where it returns to the pulmonary circulation. The shunt is
left to right. The magnitude of shunt is determined by the size of ventricular septal defect
and amount of pulmonary vascuiar resistance (PVR) present. High pulmonary vascular
resistance will elevate right ventricular pressure (making it approximate to left ventricular
pressure) and decrease shunting across ventricular septal defect. In the newborn period,
pulmonary vascular resistance is still high therefore little shunting may occur at this time
and the child may be asymptomatic. As pulmonary vascular resistance decreases over the

43
 first one to two months of life the child may become symptomatic. Due to increased blood
in right ventricle, right ventricular hypertrophy occurs.

Clinical features
The clinical features of ventricular septal defect depend upon the size of defect, the degree
of shunting, age of the child and pulmonary vascular resistance.
i. With small defects, there is little shunting and infant or child is usually asymptomatic.
ii. With large VSDs, large left to right shunt occurs and the infant or the child may
experience failure to thrive and congestive heart failure.
iii. Medium sized defects produce symptoms like dyspnea, tachypnea, slow
physical development, feeding difficulties and frequent pulmonary infections.

Diagnostic evaluation

The diagnosis of ventricular septal defect is made on the following basis:

i. Cardiac examination: Blood flows across the ventricular septal defect and produces a
system murmur that can be heard best at mid to lower left sternal border. In presence
of large left to right shunt, the increased regurgitation of blood across mitral valve
produces a diastolic rumble.
ii. Electrocardiogram: In presence of small ventricular septal defect, the ECG is normal
with moderate to large ventricular septal defects, right ventricular hypertrophy is
usually present.
iii. Chest radiograph: With moderate to large size ventricular septal defects, the heart
size and pulmonary vascular markings are increased.
iv. Echocardiogram: Color 2D echocardiography is helpful in determining the size and
location of ventricular septal defect. The degree of left to right shunting and PVR can
also be assessed.

Management

Management of ventricular septal defect is as follows:

44
i. 75-80% of small ventricular septal defects and 5-10% of large VSDs will
spontaneously usually during the first two years of life.
ii. Infants with small ventricular septal defects require no surgery, except antibiotics to
endocarditis.
iii. lnfants with moderate to large ventricular septal defects who are symptomatic, showing
signs of Congestive Heart Failure and failing to grow are usually medically managed
with a combination
of Digoxin and Diuretics. If the infant continues to show signs of Congestive Heart
Failure early surgical repair is indicated.
iv. Surgical repair of ventricular septal defects is an open heart procedure.
Ventricular defects of children weighing less than 7 kg are usually repaired with child
in deep hypothermia causing circulatory arrest. In older children cardiopulmonary
bypass is used. Moderate to small sized ventricular septal defects are closed by purse
string sutures, while for large defect, a synthetic Dacron patch is used to close the
defect. The cardiac tissue covers the patch completely within 6 months of surgery.
Large ventricular septal defects are closed through a median sternotomy and an
incision in the right atrium rather than an incision through the left or right ventricle.
Following surgical repair antibiotic prophylaxis for endocarditis is required with
cardiology follow -up.

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)

45
 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.

46
 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

47
 7) Poor growth

Diagnostic evaluation

i. Cardiac examination: Systolic murmur or continuous murmur may be present, which

is best heard in second to third left intercostals space. With large PDA, a diastolic
rumble and a gallop may be heard. Pulse is usually bounding in these Children
ii. Electrocardiogram: The ECG is usually normal; however, it may show left
ventricular hypertrophy and left atrial dilatation in older children.
iii. Chest radiograph: The chest radiograph shows increased pulmonary vascularity with
normal or increased heart size.
iv. Echocardiogram: A color flow 20 echo helps in visualization of patent ductus
arteriosus. With a Doppler, the amount of blood flow across the PDA can be
estimated.
MANAGEMENT
(A) Medical management : It include admihistration of indomethacin 0.1 to 0.25 mg/
kg/dose intra venously slowly, it is useful medication in closing of patent ductus in
premature infants and some newborn babies.
 Do the management of CCF with proper medications.
 PDA in small size may not require treatment or it may close with out treatment
treatment spontaneously.

(B) Surgical Management: It include surgical division or lagation of the patent

vessel via a left thoracotomy. A newer technique, Visual assisted thoracoscopic surgery
(VATS), by uses a thoracoscope and instruments placed through three small incisions on
the left side of the chest to place a clip on the ductus. It is used in some centers and
speeding the post operative recovery. In some centers PDA can be closed by using the
coils in catheterization laboratory.

Complications

48
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

49
  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

50
 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.

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 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.

Causes
The causes are genetic factors, chromosomal abnormalities, environmental factors and
gene defects.
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

52
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 after load 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.

Clinical features

There are two groups of patients with coarctation:

i. Those who are symptomatic in infancy.
ii. Those who remain asymptomatic and are diagnosed during routine physical
examination years.
Symptomatic children present with the following features:
i. Increased blood pressure in the upper part of body, resulting in headache, dizziness,
nose bleed (epistaxis) and later cerebrovascular accident.
ii. In lower extremities the blood pressure is relatively low, resulting in absent or
diminished femoral and pedal pulse.
iii. Occasionally the child may present with complaints of weakness or pain in their
legs on exercise. Their legs may be cooler than arms.
iv. Infants usually present with congestive heart failure and failure-to-thrive.
Symptoms includes respiratory distress, poor weight gain, feeding problems,
irritability and tachycardia.
v. Mottling is often seen in lower extremities. Infants with coarctation and a PDA may
have adequate blood flow to the lower extremities, good pedal pulse and no
difference in pressure in upper and lower extremities. Once PDA closes, symptoms
develop.
Diagnostic evaluation

53
i. Cardiac examination: The first and second heart sounds are usually normal. There
may be no murmurs present or a systolic murmur may be heard along the left mid to
upper sternal border that radiates to the back.
ii. Electrocardiogram: Left or right ventricular hypertrophy is seen on ECG in the infant
with coarctation. The older child may have left ventricular hypertrophy or a normal
ECG.
iii. Echocardiogram: The presence of a coarctation and the degree of narrowing as well
as presence of other cardiac defects may be determined by the echocardiogram.
iv. MRI and cardiac catheterization are useful in clearly defining the area and extent of
narrowing.

Therapeutic Management

Coarctation may be surgically corrected using numerous approaches which are as

follows:

i. End-to-end anastomosis: In this procedure, the narrowed portion of aorta is

removed, and two normal parts are joined.
ii. Subclavian flap aortoplasty: In this procedure a longitudinal incision is made in the
aorta across the coarctated site and continued to the end of distally divided left
subclavian artery. The left
subclavian artery is used as a patch or flap to increase the diameter of the aorta.
iii. Patch aortoplasty: In this procedure, coarctated area is excised and a patch graft is
placed on aorta to widen it.
iv. Balloon aortoplasty: It is a special procedure in which a balloon catheter is
introduced into the aorta during cardiac catheterization and inflated at the site of
coarctation to relieve the

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 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.

55
 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

i.Cardiac Examination: On auscultation a systolic ejection murmur is heard best

at left upper sternal border. An ejection click may precede the murmur.
ii.Electrocardiogram: With mild to moderate pulmonary stenosis, the ECG may be
normal or show right ventricular hypertrophy. In severe pulmonary stenosis the ECG
shows right ventricular hypertrophy and right atrial enlargement.
iii. Chest radiograph: Chest X-ray shows right ventricular hypertrophy and post stenotic
dilatation of pulmonary artery.

iv Echocardiogram: Color flow 20 Echo can demonstrate the size of right

ventricle and its outflow' tract. The level of obstruction can be visualized.

Therapeutic Management

56
 Children with mild to moderate pulmonary stenosis need no treatment. In severe
stenosis pulmonary balloon valvuloplasty or surgical repair is required. When right
ventricular pressure exceeds left ventricular pressure, pulmonary stenosis is considered
severe or critical. In newborn infants PGE1 infusion is given to maintain patency of ductus
arteriosus, allowing adequate pulmonary blood flow until relief of obstruction is achieved.
 If the valve annulus (the ring of tissue to which the leaflets of pulmonary valve are
attached) is small, an incision into the annulus is made and a patch is used to enlarge the
annulus.
 When infundibular stenosis is present resection of hypertrophied muscles is performed and
if necessary, a pericardial or Dacron patch is used to widen the outflow tract.
 For supravalvular stenosis the area of obstruction is incised and a patch graft is inserted to
widen
Complication:

 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 vessels
2) Total anomalous pulmonary venous return

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 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

58
 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 ductus arteriosus 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

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 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

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 VII. 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, providing
postoperative nursing care, preventing infection and providing patient and family
education. An important component of education involves preparing 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
i) Due to the hemodynamic changes accompanying the underlying structural defect,
oxygenation is key.
ii) Provide frequent ongoing assessment of the childs cardiopulmonary status.
iii) Assess airway patency and suction as needed. Position the child in fowlers or semi-
fowlers position to facilitate lung expansion.
iv) Monitor vital signs especially heart and respiratory rates.
v) Monitor the childs colour 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.
vi) Provide humidified supplemental oxygen as ordered, Warning it to prevent wide
temperature fluctuation.
vii) Anticipate the need for assisted ventilation if the child having difficulty maintaining the
airway or experiences deterioration in oxygenation capacity.

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 viii) 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
i) Adequate nutrition is critical to foster growth and development as well as to reduce the
risk for infection. Children with congenital heart defects typically have increased
nutritional needs due to the increased energy expenditure associated with increased
cardiac and respiratory workloads.
ii) In addition many of the defects leads to heart failure, which may affect the childs fluid
balance status further increasing the childs energy expenditure. Nutritious may be
provided orally, enterally, or parenterally for example for the newborn or infant
nutrition via breast milk or formula may be provided orally or via gavage feeding.
iii) 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.
iv) 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 of that feeding via orogastric or

62
 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. When needed,
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

63
 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.

64
  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

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 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 aspiration
xiii) Monitor intake and output hourly
xiv) Maintain accurate intravenous infusion rate, restrict fluids as ordered to 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

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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.

II. 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
VIII. RESEARCH ABSTRACT
Study of congenital heart disease in neonates: clinical profile, diagnosis, immediate
outcome and short-term follow-up

Abstract

Background: Congenital heart disease (CHD) occurs in approximately 0.8% of live births.
Early recognition of CHD is important for appropriate management and decision making
regarding referral. The purpose of this study was to document the common presenting

67
 symptoms and signs in the neonates with CHD, definitive diagnosis and short-term follow-
up for six months.

Methods: Sixty full term neonates with suspected CHD admitted in neonatal intensive
care unit (NICU) at Niloufer Hospital, Hyderabad during the period December 2016 to
May 2017 were included in the study.

Results: Of the 60 neonates, most common age of presentation was for first week (45%, n
= 27). Of the 60 neonates, 32 (54%) were males and 28 (46%) were females. The
commonest presentation was hurried respiration (68%), followed by feeding problem
(63%) and only eight neonates were asymptomatic with clinically significant murmur. 40%
(n = 24) of the babies were born of consanguineous marriage. 72% (n = 43) of babies
presented with murmur and 6 babies had extra-cardiac manifestations. Babies with
acyanotic CHD were 38 (63%) of which ventricular septal defect (VSD) was the
commonest. Cyanotic CHD were 22 (37%) of which transposition of great arteries (TGA)
was the commonest. 25 babies (42%) expired during neonatal period. Of the remaining
babies during follow-up, 29 % of babies thrived well, 35% presented with repeated
respiratory tract infections, 21% with failure to thrive and 15% with congestive heart
failure (CHF).

Conclusions: Neonates with CHD have a unique presentation and they carry poor outcome
unless diagnosed early and managed appropriately. Babies presenting with multiple
anomalies should be screened for any underlying structural heart disease.

2. The Care of Children With Congenital Heart Disease in Their Primary Medical Home

Abstract

Congenital heart disease (CHD) is the most common birth anomaly. With advances in repair
and palliation of these complex lesions, more and more patients are surviving and are
discharged from the hospital to return to their families. Patients with CHD have complex health
care needs that often must be provided for or coordinated for by the primary care provider

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(PCP) and medical home. This policy statement aims to provide the PCP with general
guidelines for the care of the child with congenital heart defects and outlines anticipated
problems, serving as a repository of current knowledge in a practical, readily accessible format.
A timeline approach is used, emphasizing the role of the PCP and medical home in the
management of patients with CHD in their various life stages.

Primary care providers (PCPs; physicians, physician assistants, and nurse practitioners)
working in medical homes (MHs) are tasked with providing and coordinating the multiple
health care needs of patients with congenital heart disease (CHD). With the goals of improving
patient outcomes and influencing the care of these children, the American Academy of
Pediatrics (AAP) and the American College of Cardiology (ACC) assembled a team of experts,
including representation from the AAP Committee on Practice and Ambulatory Medicine, to
review current literature and to develop this policy statement. A representative from the AAP’s
Family Partnership Network was requested to review this document. The role of the PCP and
family engagement across the life span is emphasized.

Background

Advances in treatment and palliation of CHD result in more patients surviving and being
discharged from the hospital with their families. Previously unrecognized familial psychosocial
stressors, racial and socioeconomic outcome disparities, other organ system involvement,
complications of therapy, feeding challenges, neurodevelopmental concerns, and other special
needs have been garnering more attention. Recommendations regarding newborn pulse
oximetry or critical CHD (CCHD) screening and infection prevention strategies in children
with CHD have been introduced or updated. Patients with CHD have complex health care
needs that are to be provided for or coordinated for by their PCP. This policy statement aims to
be a repository of available new information for the care of children with CHD, highlighting
anticipated problems. A chronologic timeline approach is used, emphasizing the role of the
PCP and/or MH in the management of patients with CHD and their families in various life
stages. Frequent communication among all care providers is recommended.

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General Issues Affecting Families of Children With CHD

Psychosocial Issues

In response to the stress of their child’s serious cardiac illness, some parents report an
increased incidence of emotional distress and psychosocial risk affecting optimal
parenting1 that may also affect the care of other children in the home. This may be exacerbated
by parental guilt, financial and marital stress, challenges in parental work-life balance, and
adversity because of poverty, neglect, environmental violence, or caregiver substance abuse or
mental illness.
Disparate outcomes have been observed across racial and socioeconomic classes. A PCP/MH
with long-standing relationships with the family may be the first to recognize danger signs and
be in a position to provide support and referrals to mental health professionals and groups that
can provide respite care and psychological help.

Need for Cardiopulmonary Resuscitation and Automated External Defibrillator Training for
Qualified Caregivers

Cardiopulmonary resuscitation (CPR) and automated external defibrillator (AED) training for
qualified caregivers and family members is important. Lay CPR-AED education may be
beneficial and may increase survival in out-of-hospital cardiac arrest.4 Many institutions,
therefore, advocate parental CPR instruction before an infant is discharged from the hospital.
AED availability and CPR training before high school graduation are mandated in certain
states.

Prenatal Period

CHD is often detected by fetal echocardiography, usually performed at ∼18 to 22 weeks’

gestation. However, both false-positive and false-negative diagnoses can occur, and certain
lesions may be not be detected in basic screening because some may be progressive and others
may develop later in gestation.5 Prenatal counseling is dependent on the gestational age,
disease natural history, presence of extra cardiac anomalies, and need for fetal intervention.6

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Barring obstetric complications, delivery at or close to term is preferred because of the critical
development of most organ systems, particularly the fetal brain and lungs, during the last 6
weeks’gestation.7 Improved outcomes are demonstrated in newborn infants with CCHD born at
39 to 40 weeks’ gestation compared with those born at 37 to 38 weeks’ gestation.8 The best
time for the family to establish relationships with primary and subspecialty care providers, and
to contact support groups if desired is before delivery.

Neonatal Period

Pulse Oximetry or CCHD Screening

CCHD screening, added to the US Neonatal Recommended Uniform Screening Panel in 2011,
may improve the timeliness of a diagnosis of CCHD that has eluded prenatal detection. 9 A
sample protocol has been published,10 but this may vary by state† or altitude. This screening
targets lesions with hypoxemia, but the test is imperfect, and a neonate who “passed” might
still have acyanotic, left-heart obstructive lesions.11,12 Thus, symptoms such as tachypnea, an
abnormally active precordium, a concerning heart murmur, or a diminution in lower extremity
pulses warrant taking a relevant history and conducting a physical examination. Depending on
findings, a pediatric cardiology consultation or other diagnostic tests, such as
electrocardiography or echocardiography, may be performed.

Genetic Screening

When aneuploidy is suspected, children with cardiac defects will benefit from genetic
evaluation to discuss prognosis and recurrence risks. Genetic testing is recommended in
particular for conotruncal abnormalities, such as d-transposition, tetralogy of Fallot, truncus
arteriosus, or interrupted aortic arch type B, to rule out 22q chromosome deletion
(velocardiofacial or DiGeorge syndrome), because the latter may be associated with calcium
metabolism, neurodevelopmental and psychiatric abnormalities, or other organ involvement.

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Infancy/Childhood

Care of the Patient With CHD After Hospital Discharge

For a coordinated transition of care from the hospital to the MH, information regarding the
patient’s cardiac diagnosis, completed or planned interventions, residual defects, organ system
involvement, and discharge physical examination findings, including weight, oxygen
requirements, and baseline saturations, must be available to the PCP. Home medications and
feeding regimens, in a schedule compatible with family home routines, including formula
concentration or expressed breast milk preparation and route of administration, are important to
note. The need for anticoagulation and thrombosis prophylaxis for patients dependent on
systemic to pulmonary artery shunts, or for those at high risk of thrombosis and stroke, 13 are
important to communicate. The target international normalized ratio and enoxaparin levels (for
patients on warfarin and enoxaparin, respectively), the reason for anticoagulation, and the
duration of therapy and frequency of monitoring should ideally be outlined in discharge
summaries.
Patients with heart failure, baseline cyanosis, shunt dependence for pulmonary blood flow, or
single-ventricle physiology have a limited cardiopulmonary reserve and are sensitive to
intravascular volume changes. These patients may decompensate rapidly during childhood
respiratory or gastrointestinal infections, when respiratory function and enteral intake are
impaired or fluid losses are magnified. Such patients need close follow-up with their PCP
and/or MH and may require hospitalization and intravenous fluids early in their course of
treatment. It would be ideal if they were identified and if recognition strategies and
interventions were discussed in advance of occurrence.

Nutrition and Feeding Challenges

Adequate nutrition, necessary for growth and brain development, may be challenging in certain
children with CHD. This is most apparent in infants with pulmonary overcirculation and in
those who underwent stage 1 Norwood palliation for a single ventricle, who may need 120 to
150 kcal/kg per day for adequate growth. Published nutritional algorithms may serve as

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reference. Some infants have limited ability for oral intake because of heart failure, suck and
swallow in coordination, postoperative vocal cord injury, and airway and structural or
functional neurologic abnormalities. Infants who cannot be safely orally fed may benefit from
nasogastric or gastrostomy tubes. Published growth charts are available15 but should be
consulted while keeping in mind that some infants with heart disease may follow their own
curve, and grow at a minimal rate of 5 g to 10 g per day. If growth velocity is suboptimal,
fortifying the caloric density of infant formula or expressed breast milk up to 30 kcal/oz may
be necessary. Potential complications in these patients may include gastroesophageal reflux,
aspiration risk, osmotic diarrhea, constipation, consequences of improperly mixed formula, and
in rare cases, necrotizing enterocolitis.

Special Immunization Needs and Infection Prevention

Preventing, identifying, and managing infections in children with CHD are primary roles of the
PCP. Current, recent, or upcoming anesthesia and surgery generally are not contraindications
for immunization. Efforts should be made to ensure vaccine administration during
hospitalization or at discharge, when indicated and age-appropriate. However, practice may
vary because of concerns for a potential febrile response that may mar the clinical picture or
the ability to mount an immune response after cardiopulmonary bypass. Because children with
CHD may have a lowered capacity to resist and fight infections, comprehensive routine
immunizations, including the recommended schedule for 13-valent conjugate pneumococcal
vaccine, are important. Patients with functional asplenia should receive subsequent doses of
23-valent polysaccharide vaccine. Some children, particularly those with heterotaxy and
asplenia or nonfunctional polysplenia, will be at risk for encapsulated bacteremia, which may
be prevented with daily antibiotic prophylaxis, at least until 5 years of age. Recommendations
for seasonal protection against respiratory syncytial virus (RSV) are available and updated
regularly. Ensuring herd immunity by vaccinating close contacts, especially against pertussis
and seasonal influenza, is recommended. Patients with DiGeorge syndrome would benefit from
immunologic assessment of their T lymphocyte function. If a patient is found to be
significantly immunocompromised, consultation with an infectious disease specialist may be

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considered to identify an appropriate vaccination strategy, because some patients should not
receive live-virus vaccines. Endocarditis prevention includes family education about risks and
adherence to current guidelines for dental procedures involving gingival manipulation and
other potentially bacteremic, high-risk interventions and surgeries. Conditions and procedures
in which endocarditis prophylaxis are recommended are summarized in Commitment to
excellent oral hygiene, a dental home capable of caring for children at high risk, application of
fluoride varnish and sealants, and oral fluoride intake are important.

Neurodevelopmental and Behavioral Concerns

Some children with CHD demonstrate a higher rate of adverse neuro developmental
outcomes25 and psychological maladjustment related to low self-esteem. This may be related to
the heart disease itself, injury during earlier episodes of shock or hypoxemia, genetic issues,
prematurity, prolonged hospitalization, cardiopulmonary bypass, intervention sequelae, or
other factors. The PCP/MH is tasked to provide careful developmental and behavioral
surveillance and screening throughout the child’s life as well as ongoing assessment of
academic progress. Early referrals to mental health providers and prompt treatment are
important.

Exercise and Sports Participation

With normal biventricular function and the absence of hemodynamically significant residual
lesions, most patients with repaired CHD will benefit from physical activity and conditioning
(exercise prescription rather than restriction). Cardiovascular health will be enhanced in
virtually all children with CHD by avoiding a sedentary lifestyle, obesity, and hypertension.
Studies of rehabilitation and conditioning programs for patients with CHD have generally
revealed benefits.28 European recommendations support active lifestyles that include
recreational and competitive sports for most patients with CHD.
Some patients with CHD are at risk for cardiac decompensation and sudden death, depending
on specific lesions and their severity.30 Examples of conditions at highest risk during strenuous
activity include those with severe ventricular outflow obstruction, hypertrophic

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cardiomyopathy, congestive heart failure, coronary insufficiency, pulmonary hypertension,
severe untreated systemic hypertension, Marfan syndrome and aortic dilation, exercise-induced
arrhythmias, and long QT syndrome. Some other lesions of concern are listed in.
Recommendations vary by individual patient, because risk may be altered with intervention
and severity of residual lesions. Close collaboration between the cardiologist and the PCP is
essential, and care must be taken to avoid giving the family conflicting advice.
Noncardiac Surgery

Up to 40% of children with CHD require noncardiac surgery by 5 years of age.31 Severity of
heart disease, cyanosis, pulmonary hypertension, or congestive heart failure increase the risk of
perioperative morbidity. Those requiring inpatient noncardiac surgery were found to have a
two-fold higher mortality risk. In contrast, perioperative complications were fewer in
ambulatory noncardiac surgeries.32Patients with CHD requiring noncardiac surgery benefit
from careful evaluation and multidisciplinary planning, including a thorough understanding of
their anatomy and physiology, with input from their PCP, cardiologist, anesthesiologist, and
surgeon to minimize the risk of adverse events.

Late Childhood/Adolescence

Obesity

Patients with CHD are not immune to the growing trend of obesity and inactivity in North
America. In a study of more than 700 children with CHD, 28% were overweight and 17% had
at least 1 BMI calculation indicating obesity.35 The etiology of obesity is multifactorial and
includes poor nutritional choices and physical inactivity from perceived handicap by the child
or parent, possibly an offshoot of the “vulnerable child syndrome.” Overweight or obese
patients have a lower percent-predicted maximum oxygen consumption and a higher blood
pressure response to exertion. Those who exercise experience fewer complications.
Adolescents with greater risk knowledge may adopt a more favorable diet.35 Physical activity
and nutrition counseling are important because obesity may have unique and harmful
implications in children and adolescents with CHD.

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Risk Reduction and Transition to Adult Care

Fostering patient engagement by providing the knowledge and skills for care participation, self-
advocacy, career planning, and job suitability is an important role for the PCP. Substance
abuse, teen-aged pregnancy, and information regarding appropriate contraception and safe sex
practices are topics for discussion. Certain CHDs, listed in carry a high risk of maternal
morbidity and mortality. When developmentally appropriate, giving adolescents increasing
responsibility for their health and encouraging provider visits without a parent for older
teenagers are important goals.

Other Endeavors Important to Children With CHD

Electronic Health Records

Electronic health records (EHRs) may provide accurate, up-to-date, legible, and accessible
medical information important in the care of patients with CHD. Although cost and technology
deficiencies are barriers to universal acceptance, many centers are espousing the use of EHRs
in compliance with the US federal mandate that includes both incentives for implementation of
EHR systems and penalties for nonadapters. EHRs may prove useful to flag patients with CHD
who will benefit from the recommendations described in this statement. For example, single-
ventricle interstage patients may be flagged as being at a high risk for mortality and morbidity
and as susceptible to dehydration and intercurrent respiratory illnesses. This is particularly
important when patients present for care at new institutions. Caregivers may also be prompted
to provide RSV or subacute bacterial endocarditis prophylaxis during well-child visits if an
“advisory” is highlighted. EHR use facilitates growth and weight trending. Medical
information portability, interoperability, and multiinstitutional sharing are helpful for
continuity and test duplication avoidance. A regularly updated and accurate “cardiology
coordination note” is ideal for multidisciplinary care. This note may contain contact
information for care team members, the patient diagnosis, medications, baseline saturations,
interventions performed, information on implants or pacemakers, the need for infective
endocarditis prophylaxis and anticoagulation, exercise restrictions if applicable, and a care plan

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 describing the circumstances that might constitute an emergency, with specific guidance for
action. This cardiology coordination note would ideally be shared among all care providers and
with the MH and the family.

Advocacy and Legislation

The AAP, state AAP chapters, the ACC, and other advocacy groups aim to minimize the
impact of CHD. Through their efforts, some states have passed legislation that ensures that
newborn infants are screened for CCHD, that students are trained in lifesaving CPR before
high school graduation, that opportunities for physical activity to reduce obesity are available,
that tobacco use be prevented, that insurance not be denied for preexisting conditions, and that
public funding for CHD research be increased. Initiatives establishing pediatric patient-
centered MHs are likewise underway. The ACC Adult Congenital and Pediatric Cardiology
Section has advocated for congressional support of legislation important to the CHD
community, such as the Advancing Care for Exceptional Kids Act of 2015, ongoing funding of
the Children’s Health Insurance Program, and funding from the Centers for Disease Control
and Prevention to support surveillance and research in CHD. The AAP Division of State
Government Affairs provides state-specific information on CHD-related policies. Individual
states may enact legislation or adopt regulations or standard-of-practice guidance.

Summary

Patients with CHD and their families have multiple needs. Care and support provided by the
PCP/MH, as outlined in the recommendations below, are invaluable for improved outcomes
throughout the patient’s life span.

Recommendations

1. Promote care coordination and communication among the family, PCP, and subspecialists
at all times, but especially during the transition from the hospital to the home or from pediatric
to adult care;

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2. Advocate for infrastructure support for caregivers, and recognize stressors that can affect
care across the life span;

3. Facilitate patient access to pediatric subspecialty care and medications;

4. Be up to date on pediatric basic and advanced life support. Encourage caregivers to undergo
CPR training for patients at an increased risk of sudden death;

5. Augment a thorough newborn history and physical examination (including palpation of

lower extremity pulses) with neonatal pulse oximetry to improve the likelihood of recognizing
CCHD, acknowledging differing legal obligations to do so in various jurisdictions;

6. Facilitate nutritional support to encourage growth and development in infants;

7. Prescribe antibiotics for asplenia, seasonal RSV prophylaxis for high-risk patients, and
influenza vaccination for eligible patients and family members when indicated. Be cognizant of
the risks of live-virus vaccines in patients with DiGeorge syndrome. Encourage dental hygiene
and adhere to endocarditis prophylaxis regimens when applicable;
8. Anticipate other organ involvement, thrombosis risk, neurodevelopmental and learning
difficulties, complications of therapy, and susceptibility to childhood illnesses;

9. Assist in promoting a lifestyle of good nutrition and physical activity in children and
adolescents. In most cases, exercise prescription is appropriate;

10. Counsel patients against illicit drug, alcohol, and tobacco use, unprotected sex, and teen-
aged pregnancy;

11. Foster self-reliance and facilitate smooth transitioning to adult health care; and

12. Support accuracy in EHRs and use them to flag patients who may benefit from the above
recommendations and to help facilitate provider access to medical information, despite patient
geographic mobility.

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Implementation

This document aims to reinforce best-practice recommendations as they pertain to children

with CHD, for which diagnosis codes already exist. Time-based coding to value incremental
work may be applied to the processes outlined in this statement.

IX. CONCLUSION

Congenital heart disease refers to a problem with the heart’s structure and function
due to abnormal heart development before birth. Congenital means present at
birth. Congenital heart disease (CHD) can describe a number of different problems
affecting the heart. It is the most common type of birth defect. A congenital heart defect is
a problem with the structure of the heart. It is present at birth. Congenital heart defects are
the most common type of birth defect. The defects can involve the walls of the heart, the
valves of the heart, and the arteries and veins near the heart. They can disrupt the normal
flow of blood through the heart. The blood flow can slow down, go in the wrong direction
or to the wrong place, or be blocked completely. Congenital heart disease are very
common in our setup and early detection rate of CHD is increasing. Moreover the general
doctors and pediatrician diagnose CHD at an early age, and are becoming more aware of
the complications of CHD that may develop if they refer late. Thus they are referring the
CHD cases to cardiac centers for proper care at an early age. Ventricular septal defect
being the most frequent anomaly. Congenital heart diseases were more common in
females than males most common clinical feature of CHDs was increased breathing,
diaphoresis, tachypnoea and tachycardia and also most common heart murmur in CHDs
was harsh systolic murmur/pansystolic murmur. Echocardiography is a useful tool to
screen neonates. Echo can also help to distinguish pathological murmurs from innocent
murmurs and decrease the anxiety of the relatives. Many children with congenital heart
defects don't need treatment, but others do. Treatment can include medicines, catheter
procedures, surgery, and heart transplants. The treatment depends on the type of the defect,
how severe it is, and a child's age, size, and general health. CHD is responsible for more

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 deaths in the first year of life than any other birth defects. Many of these defects need to be
followed carefully. Some heal over time, others will require treatment.

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2. A Padmaja. Text book of child health nursing. NewDelhi:Jaypee publication.
3. Rimple Sharma. (2017). Essentias of pediatric Nursing. NewDelhi:Jaypee publication
4. A. Pathasarathy.(2013). IAP text book of paediatrics. NewDelhi:Jaypee publication
5. Marilyn .J. Hockenberry.(2009).Essentials of pediatric nursing. Elsevier publication
6. Vinod Kumar Ravilala, Sreenivas Kotla, Radhakishan T., Ranjeet Malava.International
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