Congenital heart diseases

The Normal Heart

The heart is the organ responsible for pumping blood throughout the body. The normal heart
has a right side and left side, separated by a wall (septum). Each side is further divided into
an upper collecting chamber (atrium) and lower pumping chamber (ventricle). A series of
valves located in the heart help regulate blood flow in the right direction. Arteries are the large
blood vessels, which take blood away from the heart; veins are blood vessels returning blood
to the heart.

Normally, the right atrium receives blood returning from the body. This blood is low in oxygen,
giving it a bluish color. It flows to the right ventricle, and is then pumped out of the pulmonary
artery to the lungs. In the lungs it picks up fresh oxygen and becomes bright red. This "red
blood" then flows through the pulmonary veins to the left atrium and into the left ventricle. It is
then pumped out of the aorta to the rest of the body, delivering oxygen and nutrients. Once
oxygen is delivered to and extracted by the body tissues, the "blue blood" returns through the
veins to the right atrium, beginning the cycle again.

In congenital heart disease any of these veins, valves, chambers, or arteries can be
malformed, absent, or abnormally placed.
 Atrioventricular Septal Defect

The heart is the organ responsible for pumping blood throughout the body. The normal heart
has a right side and left side, separated by a wall (septum). Each side is further divided into
an upper collecting chamber (atrium) and lower pumping chamber (ventricle). A series of
valves located in the heart help regulate blood flow in the right direction. Arteries are the large
blood vessels, which take blood away from the heart; veins are blood vessels returning blood
to the heart.

Normally, the right atrium receives blood returning from the body. This blood is low in oxygen,
giving it a bluish color. It flows to the right ventricle, and is then pumped out of the pulmonary
artery to the lungs. In the lungs it picks up fresh oxygen and becomes bright red. This "red
blood" then flows through the pulmonary veins to the left atrium and into the left ventricle. It is
then pumped out of the aorta to the rest of the body, delivering oxygen and nutrients. Once
oxygen is delivered to and extracted by the body tissues, the "blue blood" returns through the
veins to the right atrium, beginning the cycle again.

In congenital heart disease any of these veins, valves, chambers, or arteries can be
malformed, absent, or abnormally placed.
 Coarctation of the Aorta

Coarctation of the aorta is a constriction (narrowing) of a part of the aorta, the main artery
carrying "red blood" to the body. This generally occurs close to the region where arteries to
the head and neck arise. The constriction obstructs blood flow to the lower parts of the body.
It causes blood pressure to increase above the coarctation, resulting in higher blood pressure
in the upper part of the body compared with the lower part of the body.

The left side of the heart works hard as it tries to pump blood past the coarctation. This may
cause the left ventricle to enlarge. If the coarctation is very severe, an infant's heart may fail,
resulting in rapid heart rate, rapid breathing and poor feeding. In less severe narrowing, the
child may have no noticeable side effects except increased blood pressure. If the high blood
pressure continues, damage results to other blood vessels in the body.

The age when the coarctation is repaired depends on the severity of the constriction and the
patient's symptoms. Usually, surgery is needed very early in infancy. Children with coarctation
require penicillin (also called SBE prophylaxis) prior to any dental work or surgery on the
mouth, bowel, or bladder. This helps prevent the uncommon, but possible, occurrence of
bacterial infection affecting the heart near the defect or the heart valves
 Patent Ductus Arteriosus

A patent ductus arteriosus (PDA) is a blood vessel, which connects the aorta to the
pulmonary artery. This is normally present in the fetus and allows blood to bypass the lungs.
The PDA usually closes within a few days in a full term infant. In premature infants, the
closure may take longer.

When a PDA fails to close, "red blood" from the aorta mixes with "blue blood" in the
pulmonary artery. This extra blood in the pulmonary artery travels to the lungs and left side of
the heart. Over time, this extra blood may stress and damage the blood vessels in the lungs.
In addition, the left side of the heart may enlarge as it works harder. A PDA may vary in size
from small to large. Smaller PDA's will generally cause no symptoms or ill effects in the young
child. A child with a large PDA might have poor growth and frequent respiratory infections.
Surgical repair is recommended if the PDA remains open by three months of age. If the PDA
causes significant problems, repair at a younger age may be required.

Children with PDA's require penicillin (also called SBE prophylaxis) prior to any dental work or
surgery on the mouth, bowel, or bladder. This helps prevent the uncommon, but possible,
occurrence of bacterial infection affecting the heart near the defect or heart valves.
 Tetralogy of Fallot

Tetralogy of Fallot (TOF) consists of two major lesions and two minor lesions:

• Large ventricular septal defect (VSD): This is a hole in the wall separating the two
lower heart chambers which are the pumping chambers.
• Pulmonary stenosis: This is a narrowing at and below the pulmonary valve,
obstructing blood flow to the lungs. The pulmonary arteries may be small as well.
• Right ventricular hypertrophy.
• Overriding aorta.

These defects cause the child to become cyanotic (bluish color to the lips, nailbeds, and skin).
This is caused by "blue blood," low in oxygen, from the right ventricle passing through the
VSD directly into the aorta and then to the body. Reduced amounts of blood go to the lungs
because of the obstruction from the pulmonary stenosis and smaller size of the pulmonary
artery. The stress of pumping blood through these narrowed areas causes the right ventricle
to enlarge (hypertrophy). Some infants and children experience episodes called "tet spells"
caused by a sudden decrease in the amount of blood going to the lungs. During these spells
the child turns very blue, may breathe rapidly, and may pass out.

Children with these defects may grow poorly, tire easily, and have frequent respiratory
infections. The age of surgical repair will depend on the severity of the defects and the child's
symptoms. Surgery usually consists of total correction. Sometimes, it may be performed in
stages, allowing the child to grow. Children with surgically treated or untreated TOF require
penicillin (also called SBE prophylaxis) prior to any dental work or surgery on the mouth,
bowel, or bladder. This helps prevent the uncommon, but possible, occurrence of bacterial
infection affecting the heart near the defects or the heart valves.
 Transposition of the Great Vessels

In the normal heart, their are two "great vessels": the aorta which carries blood from the left
ventricle to the body and the pulmonary artery which carries blood from the right ventricle to
the lungs. In transposition, these two great vessels are switched (transposed) with the aorta
arising from the right ventricle, and pulmonary artery arising from the left ventricle. This
results in two separate circulations:

1) "Red blood" pumped continually through the left side of the heart to the lungs and back,
without entering the rest of the body.

2) "Blue blood" pumped continually through the right side of the heart to the body and back,
without entering the lungs.

Babies born with transposition are cyanotic (bluish color of skin, lips, and nail beds) shortly
after birth because of the low oxygen in their blood. Two normal connections in the newborn
heart and blood vessels help some "red blood" and "blue blood" to mix, keeping babies alive.
One connection, the foramen ovale, is an opening between the two atria (upper chambers). In
some patients, if the foramen ovale is not open enough for blood to mix, a balloon can be
passed through this opening, making it larger. In addition, medicine can be given to keep the
second connection (called a patent ductus arteriosus) open. This second connection is a
blood vessel between the aorta and pulmonary artery, which usually closes after the first few
days of life.

Ultimately, surgery is required in order for enough red blood to circulate through the body. The
type of surgery performed depends upon a number of factors including the age of the child,
the child's general state of health and the exact nature of the heart defect.

Children with transposition require penicillin (also called SBE prophylaxis) prior to any dental
work or surgery on the mouth, bowel, or bladder. This helps prevent the uncommon, but
possible, occurrence of bacterial infection affecting the heart near the defect or valves.
 Truncus Arteriosus

During the development of the heart in the fetus, a common large artery, called a truncus
arteriosus, carries blood from the two lower chambers (ventricles) to the body and lungs. This
truncus later divides into two separate arteries, the aorta and the pulmonary artery. In
addition, the wall between the ventricles grows, completely separating the arteries. When a
child is born with truncus arteriosus, these normal steps in development have failed to occur.
"Red blood" from the left side of the heart mixes with "blue blood" from the right side of the
heart at the opening between the ventricles. This mixed blood is then pumped out of the heart
through the truncus arteriosus. From there, some blood gets to the lungs through pulmonary
arteries, which branch off from the truncus, and the rest goes to the body by way of the aorta.

Babies with truncus arteriosus may be cyanotic (bluish color of the lips, skin, and nailbeds)
because the mixed blood is low in oxygen. They tend to be small, tire easily, and breathe
rapidly because their heart works harder than normal. Surgery to repair this defect is required
very early in life.

Following surgery, children require penicillin (also called SBE prophylaxis) prior to any dental
work or surgery on the mouth, bowel, or bladder. This helps prevent the uncommon, but
possible occurrence of bacterial infection affecting the heart near the defect of the heart
valves