Cardiac Structure and Function.

Understanding the effects of congenital and acquired heart defects requires knowledge of the
normal hearts structure and function, including embryologic development, fetal circulation, and
the changes that occur with postnatal growth. In addition to the following general overview, the
specific embryologic changes resulting in each major heart defect are discussed later.

CARDIAC DEVELOPMENT.

The heart is a muscular four-chambered organ whose primary purpose is to pump blood
throughout the body. It is located slightly to the left of the sternum in the space between the two
pleural cavities, called the mediastinum. The main mass of the heart is formed by the muscular
tissue, the myocardium. Lining the inner surface of the myocardium is endocardium, a thin layer
of endothelial tissue. The heart is also has its own special covering, a double-walled membrane
called pericardium. The outer membrane is the fibrous pericardium. The inner membrane, the
serous pericardium, also consists of two layers, the parietal layer, which lines the inside of the
fibrous pericardium, and the visceral layer (epicardium), which lines the heart muscle. Between
these two layers, is a slight space (pericardial space), which is filled with a few drops of serous
fluid (pericardial fluid). These layers provide for frictionless movement of the heart muscle.
The interior of the heart is divided into four chambers. The two upper chambers are
called atria and include a right atrium (RA) and a left atrium (LA). The two bottom chambers
are ventricles, a right ventricle (RV) and a left ventricle (LV). The chambers are separated by
walls called the atrial septum and ventricular septum. Located within the heart chambers are four
valves, whose main function is to prevent the backflow of blood. The valves are attached to the
heart muscle by several cordlike structures called chordae tendinae. The tricuspid valve, so
named because it has three flaps or cusps of endocardial tissue projecting into the ventricles, is
located between the right atrium and ventricle. The bicuspid, or mitral, valve ahs two flaps and is
located between the left atrium and ventricle. Together these two valves are often termed
atrioventricular valves. The semilunar valves are located in the pulmonary artery (pulmonic
valve) and the aorta (aortic valve), Heart sounds (S1 and S2) are thought to be related to the
vibrations that result during closing of these valves.

Embryologic development

The heart and other components of the circulatory system (blood, blood vessels, lymph) develop
from the mesoderm beginning during the fourth week of gestation and are completed by the eight
week. Cardiac development parallels the embryos increasing nutritional needs, which initially
were supplied by diffusion.
During the first 3 weeks the lateral mesoderm splits to form two layers, the somatic and
the splanchnic mesoderm. The somatic mesoderm eventually gives rise to limb muscle, while the
splanchnic mesoderm forms two endocardial tubes that fuse to become the heart tube. AS the
tube elongates ir begins to coil to the right. This looping occurs by approximately the twenty-
eight day when the heart begins to beat. The mesodermal tissue surrounding the heart tube
differentiates into two layers: the endocardium and myoepicardium. Concentration of
mesenchymal cells enlarge and cause the endocardium to bulge into the lumen of the heart.
These internal bulges are called endocardial cushions and eventually merge to divide the heart
chambers.

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 The developing heart tube bulges until it finally lies in the pericardial cavity. The tube
remains attached to the pericardium at its cephalic and caudal ends but is free at the midsection.
During the fifth week the midcardiac tube grows rapidly and assumes a characteristic convoluted
shape with identifiable structures. These structures ultimately give rise to the chambers and
vessels of the heart and include (1) a common atrium, (3) a common ventricle, (3) the bulbus
cordis, which eventually helps form the outflow tracts of the ventricles, (4) the sinus venosus,
which develops into the inferior and superior vena cava and coronary sinus, and (5) the truncus
arteriosus, which divides into the pulmonary artery and aorta.
The formation of the internal structures of the heart, particularly the cardiac septa, takes
place almost simultaneously. During this partitioning process, congenital defects may result if
the formation of various structures is distributed; the various pathologic changes are discussed
later in this chapter for each mahor defect. The structure of the fetal heart provides for a pattern
of circulation that is markedly different from that required during postnatal life. During prenatal
life I distributes oxygen and nutrients, supplied via the placenta, to the developing fetus through
an efficient system of shunts that partially bypass the nonfunctioning lungs.
Fetal circulation. The normal growth and development of the fetus rely on an active,
independent metabolism, but they also require an efficient circulation. During fetal life the lungs
are essentially nonfunctional and the liver only partially functional; therefore less blood is
needed in these organs than is required after birth. The fetal brain requires the highest oxygen
concentration, and the heart must pump a large amount of blood through the placenta. The
characteristics of fetal circulation ensure that the most vital organs and tissues receive the
maximum concentration of vital materials for growth.
Blood carrying oxygen and nutritive materials from the placenta enters the fetal system
through the umbilicus via the large umbilicum vein. The blood then travels upward to the
underside of the liver where it separates – part of the blod enters the portal and hepatic
circulation of the liver and the remainder travels directly to the inferior vena cava by way of the
ductus vensus. Because of the higher pressure of blood entering the right atrium from the inferior
vena cava, it is directed posteriorly in a straight pathway across the right atrium and through the
foramen ovale to the left atrium. In this way the better-oxygenated blood enters the left atrium
and ventricle to be pumped through the aorta to the head and upper extremities. Blood from the
head and upper extremities entering the right atrium from the superior vena cava is directed
downward through the tricuspid valve into the right ventricle. From here it is pumped through
the pulmonary artery, where the major portion is shunted to the descending aorta via ductus
arteriosus. A small amount flows to and from the nonfunctioning fetal lungs. Blood is returned to
the placenta from the descending aorta through the two umbilical arteries.
Before birth yje hight pulmonary vascular resistance created by the collapsed fetal lung
causes greater pressures in the right side of the heart and the pulmonary arteries. T the same time
the free-flowing placental circulation and the ductus arteriosus produce a low systemic vascular
resistance in the remainder of the fetal vascular system. With the clamping of the umbilical cord
and the expansion of the lungs at birth, the hemodynamics of the fetal vascular system undergo
pronounced and abrupt changes. These changes are the direct result of cessation of the placental
blood flow and the beginning of the lung respiration.

Postnatal Development

In infancy the size of the heart in relaton to total body size is larger, and it occupies a larger
space within the lung enclosure. It lies at a transverse angle, but with growth and the enlarging
lungs it comes to lie lower and more obliquely at maturity.

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The ventricle walls are more or less equal in thickness at birth (some believe that the right
ventricle may be somewhat larger). With the increased demand of the postnatal peripheral
circulation, the left site becomes thicker than the right. During the first years the weight of the
heart is doubled; by 5 years, it is increased fourfold, and by 8 years, six-fold. An increase in
heart size accompanies the adolescent growth spurt with a resulting increase in blood pressure
and decrease in heart rate. The heart rate at any age shows an inverse relationship to body size.
The arteries and veins elongate to keep pace with expanding body dimensions, and the
vessel walls thicken to cope with the increased pressure. The systolic blood pressure after birth is
low, reflecting the weaker left ventricle of the neonate. With the developing strength and power
of the left side of the heart, the systolic blood pressure rises rather sharply during the first 6
weeks and continues to rise but at a much slower rate until shortly before puberty, at which point
it rises rapidly to adult levels.
Postnatal circulation. Once the cardiorespiratory system adjusts to the changes necessary to
support extrauterine life, the circulation through the heart assumes a pathway that allows for
oxygenation of venous blood by the lungs and delivery of saturated blood to the systemic
circulation. Blood returning from the body via superior vena cava (SVC) and inferior vena cava
(IVC) is received in the RA. It is then pumped to the RV through the tricuspid valve. The LV
pumps the blood through the pulmonic valve into the pulmonary artery where the blood becomes
saturated with oxygen in the lungs. The blood is then returned from the lungs via the pulmonary
veins into the LA, where it is pumped through the bicuspid valve to the LV, and the finally
through the aortic valve to the aorta into the systemic circulation.
Arteries are blood vessels that carry blood away from the heart and serve primarily the
function of distributing highly oxygenated blood to the capillaries. Veins are blood vessels that
carry poorly oxygenated blood to the heart and function as both collectors and reservoirs. Their
function as a reservoir helps maintain normal circulation. For example, if there is increased
resistance to blood flow through the right side of the heart (e.g., as the result of severe
pulmonary valvular stenosis), right ventricular end-diastolic and right atrial pressures may rise.
As result, central venous pressure rises and hepatomegaly may occur. The function of the
arterioles is mainly to provide resistance to blood flow to maintain blood pressure and
circulation.
Although major blood vessels enter and leave the heart, the heart muscles receives its
own coronary blood supply. The right and left coronary arteries, which arise above the aortic
valve, supply all of the myocardium. Coronary veins collect the blood and return it directly to
the right atrium or through a common venous channel called the coronary sinus, which drains
into the right atrium.
Conduction system. To maintain an orderly and effective pumping action, the heart has a
specialized electrical conduction system – electrical impulses generated within the heart initiate
the mechanical contraction leading to circulation of blood. Although all myocardial cells are
capable of developing an action potential and depolarizing without external stimulation, certain
specialized cells exist that make up the hearts normal pacemaker. This structure include:

1. The sinoatrial (SA) node, located within the right atrial wall near the opening of the
superior vana cava.
2. The atrioventricular (AV) node, also located within the right atrium but near the lower
end of septum

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 3. The atrioventricular bundle (bundle of His), which extends from the atrioventricular
node along each side of the interventricular septum and then divides into right and left
bundle branches.
4. Purkinje fibers, which extend from the atrioventricular bundle into the walls of the
ventricles

The sinoatrial (SA) node initiates the hearts conduction system and normally is the hearts
pacemaker. The impulse spreads from the SA node throughout the atria to cause depolarization.
As the atria depolarize, impulses spread to the atrioventricular node to stimulate the ventricles.
The atrioventricular node is the major pathway by which the impulses from the atria can be
transmitted to the ventricles. The impulses then spread to the atrioventricular bundle and
Purkinje fibers to cause simultaneous depolarization of the ventricles.
A cardiac cycle is composed of sequential contraction (systole) and relaxation (diastole)
of both the atria and the ventricles. Then, as the atria relax, the ventricles contract to eject blood
into the pulmonary artery and aorta. During the period of atrial diastole blood enters the atria
from the systemic and pulmonary veins, thus completing one cardiac cycle.

ASSESSMENT OF CARDIAC FUNCTION

Diagnosis of congenital or acquired heart desesases is aided by a comprehensive history and

physical examination. In addition several specialized diagnostic procedures are available to help
confirm the diagnosis. The following discussion is an overwiew of the major techniques.
Specific positive findings are included under the discussion of the heart defect.

History and Physical Assessment

A complete history is essential regardless of the type of heart condition. The major categories to
investigate include a history of:
1. Poor weight gain, poor feeding habits, and fatigue during feeding
2. Frequent respiratory infections and difficulties (tachypnea, dyspnea, shortness of
breath)
3. Cyanosis with or without clubbing of fingers
4. Evidence of exercise intolerance

In taking a history the student should elicit the concerns of the parents. Often they have
vague, nonspecific complaints such as “baby doesn’t feed well” or “ baby is too quite” that offer
clues to less obvious cardiac defect. In addition, a history of previous defects in a sibling,
maternal rubella infection during pregnany, the use of medications or chemicals during
pregnancy, and a chronic illness in the mother can be important clues to diagnosis. Children with
chromosomal abnormalities, such as Turner or Down syndrome, are especially likely to have
associated heart defects and the history I essential in evaluating their overall health. A history of
a viral infection or toxic exposure is important if myocarditis is suspected, and a history of a
previous streptococcal infection is of primary importance in rheumatic fever.
Several aspects of the physical examination may yield evidence of heart disease. The
most important is auscultation to:

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 1.Asses heart rate and rhythm, heart sounds, and adventitious sounds, such as friction rubs or
murmurs
2.Determine physiologic splitting of the second heart sound
3.Locate sounds, including murmurs
4.Assess approximate anatomic size from the point of maximum impulse
5.Determine blood pressure

Palpation and percussion are used to:

1. Assess the quality and symmetry of all pulses

2. Locate the cardiac, hepatic and splenic borders for evidence of organ enlargement
3. Determine thrills

Inspection of nutritional status, skin color (especially signs of cyanosis), respiratory rate and
rhythm, posturing, chest deformities or asymmetry, distended veins, and abnormal cardiac
pulsations may help substantiate evidence of cardiac defect.

Murmurs. Although most murmurs are benign, they can also be an important sign of
cardiac defects.
Overview of the types of murmurs heard in heard defects:

The most common cause of murmurs is an abnormal shunting of blood between two heart
chambers or between vessels. However, murmurs can also be produced by disturbing the flow of
fluid through a vessel as a result of
1) increasing the rate of flow
2) constricting or dilating the lumen
3) creating some type of irregularity on the vessel wall, such as an aneurysm, which vibrates
as fluid flows past.

Murmurs are classified according to their timing within the cardiac cycle

- Systolic: between S1 and S2

- Diastolic: between S2 and S1
- Systolic ejection: begin after the S1, attain a peak during midsystole, and terminate
before the S2
- Pansystlolic or holosystolic: during all of systole
- Pandyastolic or holodyastolic: during all of diastole
- Prodiastlic: early diastolic
- Presystolic: late diastolic
- Continous: continue through all of systole and all or part of diastole.

Murmurs caused by congenital defects involving the septum or great vessels are usually heard
best near the sternal borders or over the base of the heart. Those of valvular origin are typically
loudest over the respective auscultating valvular area, in the direction of blood flow. Murmurs
that originate on the right side of the heart are subject to change during respiration as a result of
intrathoracic pressure that prolongs right ventricular filling. Thus murmurs originating on the
right side of the heart increase during inspiration.

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