Disease: Septal Ventral Defects

Background:
Ventricular septal defect describes one or more holes in the wall that separates 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.
The most frequent types of congenital malformations affect the heart. It is estimated that approximately eight in 1,000
newborns have CHD. A VSD is the most frequent of the various types of CHD (25%-30% of all CHD). Approximately one infant
in 500 will be born with a VSD.
Before a baby is born, the right and left ventricles of its heart are not separate. As the fetus grows, a wall forms to
separate these two ventricles. If the wall does not completely form, a hole remains. This hole is known as a ventricular septal
defect, or a VSD. The baby may have no symptoms, and the hole can eventually close as the wall continues to grow after birth. If
the hole is large, too much blood will be pumped to the lungs, leading to heart failure.
The cause of VSD is not yet known. This defect often occurs along with other congenital heart defects. The heart is
forming during the first 8 weeks of fetal development. It begins as a hollow tube, then partitions within the tube develop that
eventually become the septa (or walls) dividing the right side of the heart from the left. Ventricular septal defects occur when
the partitioning process does not occur completely, leaving an opening in the ventricular septum.
Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or
environmental exposure, causing heart problems to occur more often in certain families. Most ventricular septal defects occur
sporadically (by chance), with no clear reason for their development.
In adults, ventricular septal defects are a rare but serious complication of heart attacks. These holes do not result from a
birth defect.
Normally, unoxygenated blood from the body returns to the right half of the heart, that is the right atrium, then
the right ventricle, which pumps the blood to the lungsto absorb oxygen. After leaving the lungs, the oxygenated blood returns

to the left half of the heart, that is the left atrium, then the left ventricle, where it is pumped out to provide oxygen to all the
tissues of the body.

A ventricular septal defect can allow newly oxygenated blood to flow from the left ventricle, where the pressures are
higher, to the right ventricle, where the pressures are lower, and mix with unoxygenated blood. The mixed blood in the
right ventricle flows back or recirculates into the lungs. This means that the right and left ventricles are working
harder, pumping a greater volume of blood than they normally would.
Eventually, the left ventricle can work so hard that it starts to fail. It can no longer pump blood as well as it did. Blood
returning to the heart from the blood vessels backs up into the lungs, causing pulmonary congestion, and further
backup into the body, causing weight gain and fluid retention. Overall, this is called congestive heart failure.
If the VSD is large and surgically uncorrected, pressure can build excessively in the lungs, called pulmonary
hypertension. The higher the lung or pulmonary pressure, the greater the chance of blood flowing from the right
ventricle to the left ventricle, backwards, causing unoxygenated blood to be pumped to the body and cyanosis (blue
skin).
The risk for these problems depends on the size of the hole in the septum and how well the infants lungs function.

The ventricular septal defect may not be heard with a stethoscope until several days after birth. This is because a
newborn's circulatory system changes during the first week with drop in the lung or pulmonary pressure, creating the
greater pressure differential between the 2 ventricles, thus greater left-to-right shunt and audible murmur. Ventricular
septal defects are the most common congenital heart defects in infants (that is, defects that a person is born with).

The condition occurs in about 25% of all infants born with a heart defect.
These defects are more common in premature infants.
No one knows what causes ventricular septal defects, but they probably come from amalformation of the heart that
occurs while theinfant is developing in the womb.
There may be just one hole or several holes in the septum.
The septum itself is divided into multiple areas, including the membranous part, themuscular part, and other areas
called the inlet and outlet. Any or all of these parts can have a hole.
The location of the hole depends on where the malformation takes place during fetal development.

The most common type of ventricular septal defect is the membranous variant. In this type, the hole is located below the aortic
valve, which controls flow of blood from the left ventricle into the main artery of the body, the aorta.
Small holes in the ventricular septum usually produce no symptoms but are often recognized by the child's health care
provider when a loudheart murmur along the left side of the lowerbreast bone or sternum is heard. Large holes typically
produce symptoms 1-6 months after an infants birth.

Risk Factors:
Ventricular septal defect appears to run in families and sometimes occurs with other genetic problems, such as Down
syndrome. If you already have a child with a heart defect, a genetic counselor can predict the approximate odds that your next
child will have one.
Having the following conditions during pregnancy can increase your risk of having a baby with a heart defect.

Rubella infection. Becoming infected with German measles (rubella) while pregnant can increase the risk of fetal
heart defects. The rubella virus crosses the placenta and spreads through the fetus' circulatory system damaging blood vessels
and organs, including the heart.

Poorly controlled diabetes. Uncontrolled diabetes in the mother in turn affects the fetus' blood sugar, causing
damaging effects to the developing fetus.

Drug or alcohol use or exposure to certain substances. Use of certain medications, alcohol or drugs or
exposure to chemicals or radiation during pregnancy can harm the developing fetus.

Poor nutrition of pregnant women.

Maternal age over 40 years.

Clinical manifestation:
Patients with ventricular septal defects may not have symptoms. However, if the hole is large, the baby often has
symptoms related to heart failure.
The most common symptoms include:

Shortness of breath

Fast breathing

Hard breathing

Paleness

Failure to gain weight

Fast heart rate

Sweating while feeding

Frequent respiratory infections

Poor weight gain

When a ventricular septal defect is not detected early in life, it can cause more severe problems and more severe symptoms as
time goes on. The biggest concern is development of high pressure in the lungs (pulmonary hypertension). If the ventricular
septal defect is not surgically closed, irreversible pulmonary hypertension can develop, and the child is no longer operable and
has a poor prognosis. The following are typical symptoms of pulmonary hypertension:
Fainting
Shortness of breath
Chest pain
Bluish discoloration of the skin (cyanosis)
The skin turns faintly bluish when the tissues are not receiving quite enough oxygen. This condition is often termed
"hypoxemia" or "hypoxia."

Assessment and Diagnostic Methods:

Listening with a stethoscope usually reveals a heart murmur (the sound of the blood crossing the hole). The loudness of
the murmur is related to the size of the defect and amount of blood crossing the defect.
Tests may include:

Cardiac catheterization (rarely needed, unless there are concerns of high blood pressure in the lungs)

Chest x-ray -- looks to see if there is a large heart with fluid in the lungs

ECG -- shows signs of an enlarged left ventricle

Echocardiogram -- used to make a definite diagnosis

MRI of the heart -- used to find out how much blood is getting to the lungs

Medical Management:
Some children have no symptoms, and require no medication. However, many children may need to take medications to help
the heart work more efficiently, due to the strain from the extra blood passing through the VSD. Medications that may be
prescribed include the following:

digoxin - a medication that helps strengthen the heart muscle, enabling it to pump more efficiently.

diuretics - the body's water balance can be affected when the heart is not working as well as it could. These
medications help the kidneys remove excess fluid from the lungs and the body.

ACE inhibitors - medications that lower the blood pressue in the body, making it easier for the blood to be pumped
from the left ventricle into the body (because of its lowered blood pressure) rather than that blood being pumped from
th0 e left ventricle across the VSD into the right ventricle then into the lungs .

Prevention:
Except for VSD that is caused by a heart attack, this condition is always present at birth.
Drinking alcohol and using the antiseizure medicines depakote and dilantin during pregnancy may increase the risk of
VSDs. Other than avoiding these things during pregnancy, there is no known way to prevent a VSD.

Pathophysiology:
During ventricular contraction, or systole, some of the blood from the left ventricle leaks into the right ventricle, passes through
the lungs and reenters the left ventricle via the pulmonary veins and left atrium. This has two net effects. First, the circuitous
refluxing of blood causes volume overload on the left ventricle. Second, because the left ventricle normally has a much higher
systolic pressure (~120 mm Hg) than the right ventricle (~20 mm Hg), the leakage of blood into the right ventricle therefore
elevates right ventricular pressure and volume, causing pulmonary hypertension with its associated symptoms.

In serious cases, the pulmonary arterial pressure can reach levels that equal the systemic pressure. This reverses the left to right
shunt, so that blood then flows from the right ventricle into the left ventricle, resulting in cyanosis, as blood is by-passing the
lungs for oxygenation.
This effect is more noticeable in patients with larger defects, who may present with breathlessness, poor feeding and failure to
thrive in infancy. Patients with smaller defects may be asymptomatic. Four different septal defects exist, with perimembranous
most common, outlet, atrioventricular, and muscular less commonly.

Possible Complications:

Aortic insufficiency (leaking of the valve that separates the left ventricle from the aorta)

Damage to the electrical conduction system of the heart during surgery (causing an irregular heart rhythm)

Delayed growth and development (failure to thrive in infancy)

Heart failure

Infective endocarditis (bacterial infection of the heart)

Pulmonary hypertension (high blood pressure in the lungs) leading to failure of the right side of the heart

Nursing Intervention:
a. Pre-surgical Care

Explain to the child with age-appropriate manner, before surgery

1. Monitor the child's basic status:

Vital signs

The color of mucous membranes

The quality and intensity of the peripheral arteries

When the capillary

The temperature of the extremities

2. Help and support children during the performance of laboratory tests and diagnostic tests

A complete blood count, urinalysis, serum glucose, and blood urea nitrogen

Serum Electrolytes - Na, K, and Cl

Blood type and cross-examination

Radiographic examinations

ECG
b. Post-surgical Care
1. Monitor the child's postoperative status every 15 minutes during the first 24 to 48 hours.

Vital signs

The color of mucous membranes

The quality and intensity of the peripheral arteries

When the capillary

Edema periorbital

Pleural effusions
Pulsus paradoxus or a decrease in pulse pressure
Arterial pressure
Heart rhythm

2. Monitor the bleeding

Measure the chest tube output per hour

Assess the presence of a clot in the chest tube

Assess the presence of lesions and ecchymoses petekia

Assess the bleeding from somewhere else

Record the output of blood for diagnostic studies

Monitor intake and output strictly

Give fluids as much as 50% to 75% volume maintenance during the first 24 hours

Provide the necessary blood products

3. Monitor the child's hydration status

Skin turgor

Humidity mucous membranes

Density

Weight daily

The output of urine

References

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Bonow RO, Mann DL, Zipes DP,
Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders
Elsevier; 2011:chap 65.

http://www.nlm.nih.gov/medlineplus/ency/article/001099.htm
http://www.mayoclinic.com/health/ventricular-septal-defect/DS00614/DSECTION=preparing-for-your-appointment
http://bestpractice.bmj.com/best-practice/monograph/1100/basics/pathophysiology.html
cmbi.bjmu.edu
http://www.mayoclinic.com