Also referred to as "a hole in the heart," septal defects are diagnosed when blood is able to flow between the heart's left and right chambers due to an opening in the wall that separates the two sides (the septum).

Types of Septal Defects

• Atrial septal defect - The opening is between the two upper chambers of the heart, the left and the right atrium. This defect allows blood to flow back into the lungs.
• Ventricular septal defect (VSD) - The opening is between the two lower chambers of the heart, the left and the right ventricle. This defect allows blood to flow back into the right ventricle instead of into the aorta.
• Eisenmenger's complex - Ventricular septal defect coupled with high blood pressure in the lungs, that may also include a malpositioned aorta that receives blood from both ventricles.
• Atrioventricular canal defect - The opening is between the upper and lower chambers of the heart. Blood is allowed to flow from the lungs, to the left side of the heart, to the right side of the heart, then back to the lungs (instead of the rest of the body).

Symptoms of Septal Defects

Children born with atrial septal defect may not have any symptoms early in life, but may have complications later. In the other defects, symptoms may include (depending upon the defect):

• Slow growth
• High blood pressure in the lungs
• Cyanosis (a bluish color to the skin)
• Heart murmur

Treatment Options

Treatment depends upon the severity of the defect. If treatment is required, surgical repair of the opening is typically successful in restoring normal circulation.

What is an ASD?

The septum is a wall that separates the heart's left and right sides. Septal defects are sometimes called a "hole" in the heart. A defect between the heart's two upper chambers (the atria) is called an atrial septal defect (ASD).

When there is a large defect between the atria, a large amount of oxygen-rich (red) blood leaks from the heart's left side back to the right side. Then this blood is pumped back to the lungs, despite already having been refreshed with oxygen. This is inefficient, because already-oxygenated blood displaces blood that needs oxygen. Many people with this defect have few, if any, symptoms.

Surgical Therapy

Closing an atrial septal defect in childhood can prevent serious problems later in life. The long-term outlook is excellent. If atrial septal defects are diagnosed in adulthood, the defect is also repaired. Rarely, the defect is left unrepaired if there's pulmonary hypertension (high blood pressure in the lungs). Your cardiologist can determine if the defect should be closed.

Ongoing Care

• Medical
  After an ASD is closed, patients need follow-up with a cardiologist. Only rarely will they need to take medicine. Your cardiologist can monitor you with noninvasive tests if needed. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms. They will help show if more procedures, such as a cardiac catheterization, are needed.
• Activity Restrictions
  Activity restrictions are almost never needed unless there are associated problems that you and your doctor have discussed.
• Endocarditis Prevention
  Not needed.

Problems You May Have

People with repaired atrial septal defects rarely have any problems. Those who have palpitations or faint need to be reevaluated by their cardiologist and may need medical therapy. Also, if the ASD is diagnosed late in life, the heart may be less able to pump. This can require diuretics, drugs to help the heart pump better and drugs to control blood pressure. If pulmonary hypertension develops (which is rare), some people may need more medications.

Will You Need More Surgery?

Once an ASD has been closed, it's unlikely that more surgery will be needed. Rarely, a patient may have a residual hole. Whether it will need to be closed depends upon its size.

VENTRICULAR SEPTAL DEFECT

What is a VSD? 

The septum is a wall that separates the heart's left and right sides. Septal defects are sometimes called a "ho le" in the heart. A defect between the heart's two lower chambers (the ventricles) is called a ventricular septal defect (VSD).

When there is a large opening between the ventricles, a large amount of oxygen-rich (red) blood from the heart's left side is forced through the defect into the right side. Then it's pumped back to the lungs, even though it's already been refreshed with oxygen. This is inefficient, because already-oxygenated blood displaces blood that needs oxygen. This means the heart, which must pump more blood, may enlarge from the added work. High blood pressure may occur in the lungs' blood vessels because more blood is there. Over time, this increased pulmonary hypertension may permanently damage the blood vessel walls.

If the opening between the ventricles is small, it doesn't strain the heart. In that case, the only abnormal finding is a loud murmur.

Surgical Therapy

Closing small ventricular septal defects may not be needed. They often close on their own in childhood or adolescence. But if the opening is large, even in patients with few symptoms, closing the hole in the first two years of life is recommended to prevent serious problems later. Usually the defect is closed with a patch. Over time the normal heart lining tissue covers the patch, so it becomes a permanent part of the heart. Some defects can be sewn closed without a patch. Repairing a VSD restores the blood circulation to normal. The long-term outlook is good.

Ongoing Care

• Medical
  After their VSD is closed, patients should be examined regularly by a cardiologist. He or she will make sure that the heart is working normally.
• Activity Restrictions
  Most patients won't need to limit their activity. However, if you have pulmonary hypertension or your heart doesn't pump as well as it used to, you may need to limit your activity to how much you can endure. Your cardiologist will help determine if you need to limit your activity.

Endocarditis Prevention
Unclosed VSDs require endocarditis prophylaxis. After the VSD is successfully closed, preventive treatment is needed only during a six-month healing period. (See section on Endocarditis.)

Problems You May Have
Most people whose uncomplicated ventricular septal defects are repaired early in life don't have any significant long-term problems. In some people, the heart muscle may be less able to contract. This requires diuretics, agents to help the heart pump better and drugs to control blood pressure. Also, if pulmonary hypertension develops (it's uncommon), you may need more medical therapy.

Will You Need More Surgery?
People whose ventricular septal defects are repaired rarely need more surgery unless residual defects are seen afterwards. If this occurs, whether you'll need surgery depends upon the size of the residual defects.

COMPLETE AV CANAL(CAVC)

What is CAVC?

Many terms are used to describe this heart defect. They include atrioventricular (AV) canal, complete AV canal, complete common AV canal, and endocardial cushion defect.

Essentially, they all describe a similar heart problem. It's a combination of:
a hole in the wall dividing the heart's upper chambers (atrial septal defect),
a hole in the wall separating the heart's lower chambers (ventricular septal defect) and
abnormalities of the tricuspid and mitral valves inside the heart.

T hese combined defects cause extra blood to circulate through the lungs. This results in symptoms of congestive heart failure in infancy. Sometimes there's leakiness (regurgitation) of the abnormal single valve. This may add to the heart failure symptoms.

Surgical Therapy

CAVC is treated surgically. Surgery is done in infancy to close the atrial and ventricular septal defects and reconstruct two atrioventricular valves from the common, single valve. Some patients may have had a temporary procedure to limit blood flow to the lungs (pulmonary artery banding) before a complete repair. The reparative operation produces more normal circulation, but the reconstructed valves often work in a mildly abnormal way.

Ongoing Care

• Medical
  If you have a CAVC, a cardiologist with expertise in congenital heart defects should provide care. Besides giving you routine exams, a cardiologist will often obtain one or more tests. These can include an electrocardiogram, echocardiogram or chest X-ray.
  Some patients with residual defects, such as a leaky valve (valvar regurgitation), may need medications to help their heart pump better and/or lower blood pressure.

Being physically active is healthy for the cardiovascular system, but some patients may need to limit their activity. Discuss your situation with your cardiologist.

• Endocarditis Prevention
  Lifelong endocarditis preventive treatment is recommended. (See the section on Endocarditis.)

Problems You May Have

Some people with CAVC may be lethargic or have less stamina after the repair. This may be due to a valve working abnormally. Your cardiologist should check this. Occasional palpitations (skipped heartbeats) also may occur. Some patients may need a pacemaker after the repair, but that's rare.

Pregnancy

You may handle pregnancy well if you have no significant residual problems after your repair. Consult with your cardiologist before you decide to get pregnant. You need to find out the exact risks to you and your child.

Will You Need More Surgery?

The function of the reconstructed valves is a long-term concern. A few patients will need their valve replaced with a mechanical one when they get older.