Ebstein's Anomaly

What It Is

Ebstein's anomaly, also called Ebstein's malformation, is a heart defect in which the tricuspid valve is abnormally formed. The tricuspid valve normally has three "flaps" or leaflets. In Ebstein's anomaly, one or two of the three leaflets are stuck to the wall of the heart and don't move normally. Often there's also a hole in the wall between the atria, the heart's two upper chambers. This hole is called an atrial septal defect or ASD. Because the tricuspid valve is malformed in Ebstein's anomaly, it often doesn't work properly and may leak. If the valve leaks, some of the blood pumped by the right ventricle goes backwards through the valve with each heartbeat.
Surgical Treatment
Ebstein's anomaly is mild in most adults who have it, so they don't need surgery. However sometimes the tricuspid valve leaks severely enough to result in heart failure or cyanosis (see the Glossary). Then surgery may be required.
Several different operations have been used in patients with Ebstein's anomaly. The most common involves a repair of the tricuspid valve. The valve can't be made normal, but often surgery significantly reduces the amount of leaking. If there's an ASD, it's usually closed at the same time. In some cases the tricuspid valve can't be adequately repaired. Then it's replaced with an artificial valve.
Ongoing Care

Medical

People with Ebstein's anomaly should receive continued care from a cardiologist with expertise in congenital heart defects. Besides getting information from routine exams, the cardiologist may use tests such as electrocardiograms, Holter monitor and echocardiograms.
Activity Restrictions
Being physically active is good for your cardiovascular system, so stay active. If valve leakage is mild and tests show no abnormal heart rhythms, you can usually participate in most sports. Your cardiologist may recommend avoiding certain intense competitive sports. Ask your cardiologist which activities are right for you.
Pregnancy
Pregnancy puts many increased demands on the heart. In most cases women with mild Ebstein's anomaly will safely deliver normal children. Still, each woman should be evaluated individually. Consult with your cardiologist about the safety of pregnancy.
Problems You May Have

Heart Rhythm Disturbances

People with Ebstein's anomaly may have a rapid heart rhythm called supraventricular tachycardia (SVT). An episode of SVT may cause palpitations. (You feel your heart racing.) Sometimes this is associated with fainting, dizziness, lightheadedness or chest discomfort. If you have these symptoms, contact your doctor. If your symptoms persist, seek immediate attention. Recurrent SVT may be prevented with medicines. In many cases, the source of the abnormal heart rhythm may be removed by a catheter procedure called radiofrequency ablation.
Other Problems
If the valve abnormality is especially severe, you may have decreased stamina, fatigue, cyanosis, and sometimes fluid retention. These problems usually develop because the valve has become leakier. If you have these symptoms, contact your cardiologist. The symptoms may respond to medicines such as diuretics, which cause you to lose excess fluid. In some instances surgery (described above) may be recommended.

Tags: congenital heart defects,heart diseases,Ebstein's Anomaly

Transposition of the Great Arteries

What It Is
In transposition of the great arteries, the aorta and pulmonary artery are reversed. The aorta receives the oxygen-poor blood from the right ventricle, but it's carried back to the body without receiving more oxygen. Likewise, the pulmonary artery receives the oxygen-rich blood from the left ventricle but carries it back to the lungs.

Surgical Treatment
All patients with transposition of the great arteries require surgery early in life to survive. Many infants undergo a procedure in the catheterization laboratory to "buy time" and delay the surgery until they can handle it better. The procedure enlarges a naturally occurring connection between the right and left upper chambers (the atria). This lets the blood mix so some oxygen-rich and oxygen-poor blood can be pumped to the correct side. Two major types of surgery can correct the transposition. The first creates a tunnel (a baffle) between the atria. This redirects the oxygen-rich blood to the right ventricle and aorta and the oxygen-poor blood to the left ventricle and the pulmonary artery. This operation is called an atrial or venous switch. It's also called the Mustard procedure or the Senning procedure. The second type is called the arterial switch operation. The aorta and pulmonary artery are switched back to their normal positions. The aorta is connected to the left ventricle, and the pulmonary artery is connected to the right ventricle. The coronary arteries, which carry the oxygen-rich blood that nourishes the heart muscle, also need to be re-attached to the new aorta.

Medical
Patients with transposition will require lifelong follow-up with a cardiologist trained to care for patients with congenital heart disease. You may need to take medications to improve how your heart works. The cardiologist will track you with a variety of non-invasive tests. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms.

Activity Restrictions
Most cardiologists recommend that patients limit their physical activities to their endurance. They don't recommend competitive sports for high school and college students. Your cardiologist will help determine the proper level of activity restriction for you.

Problems You May Have

Heart Function
Patients who've had an atrial switch (e.g., Mustard or Senning operation) may have a serious decline in heart muscle or heart valve function. This is because the right ventricle is pumping blood to the entire body instead of just the lungs. Medications to help the heart pump better, control fluid accumulation (diuretics) and control blood pressure may help. Patients who've had the arterial switch operation don't seem to have as great a risk of heart muscle decline. They may have valve leakage or coronary artery problems, however.

Heart Rhythm Disturbances (Arrhythmias)
People with repaired transposition, especially those who've had the Mustard or Senning operation, are at risk of developing heart rhythm abnormalities. These are called arrhythmias. These arrhythmias often arise in the heart's upper chambers. Your heart rate may be too slow or too fast. If your heart rate is too slow, an artificial pacemaker can speed it up. If your heart rate is too fast, medication can slow it down. At times, you may need a cardiac catheterization to study and treat these rhythm disturbances.

Pregnancy Women with repaired transposition may have successful pregnancies. The risk of pregnancy to the mother increases if there are serious heart muscle problems or arrhythmias. It's important to consult with a cardiologist experienced in caring for patients with congenital heart defects before pregnancy to find out your health risks.

Tags: congenital heart defects,heart defects,transposition of the great arteries

Truncus Arteriosus

What It Is
Truncus arteriosus is characterized by a large ventricular septal defect over which a large, single great vessel (truncus) arises. This single great vessel carries blood both to the body and to the lungs.

Surgical Treatment
Surgery is required to close the ventricular septal defect and separate blood flow to the body from blood flow to the lungs. This is generally done early in infancy to prevent high blood pressure from damaging the lungs' arteries. A patch is used to close the ventricular defect. The pulmonary arteries are then disconnected from the single great vessel and a tube (a conduit or tunnel) is placed from the right ventricle to these pulmonary arteries. This is sometimes called a Rastelli repair.

Medical
Patients with truncus arteriosus need regular follow-up with a pediatric or adult cardiologist with special training in congenital heart disease. You may need to take medicine after your operation to help your heart pump better. Your cardiologist will track you with a variety of tests. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms to determine when another procedure such as cardiac catheterization may be needed.

Activity Restrictions
If you have decreased heart function or rhythm disturbances, you may need to limit your activity. Your cardiologist will help determine what is necessary.

Problems You May Have

Heart Function
In the long-term period after the operation, the heart muscle's ability to contract may decrease. You may need medication including diuretics, agents to help your heart pump better and drugs to control your blood pressure.

Heart Rhythm Disturbances
Patients with repaired truncus arteriosus have a higher risk for heart rhythm disturbances, called arrhythmias. These arrhythmias can originate from the atria (the heart's two upper chambers) or the ventricles (the two lower chambers). Sometimes they may cause dizziness or fainting. Medication may be required to control them. In rare cases, a procedure in the cardiac catheterization laboratory or the operating room may be required to eliminate these arrhythmias and control symptoms.

High Blood Pressure in the Lungs (Pulmonary Hypertension)
Sometimes, even when the defect is repaired early, the pulmonary hypertension becomes progressively worse. You may experience shortness of breath, decreased exercise endurance and sometimes headaches and dizziness.

Pregnancy
Women with repaired truncus arteriosus may handle pregnancy well. However, some may not.

Tags: congenital heart defects, Truncus Arteriosus, heart disease, congenital heart disease

Single-Ventricle Defects

What It Is
Complex heart defects that result in one of the heart's pumping chambers (ventricles) being underdeveloped are called single-ventricle defects. Each of these defects is relatively rare. They include such problems as tricuspid valve atresia, hypoplastic left-heart syndrome, hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum), mitral valve atresia, and double-inlet ventricle. Other types of heart defects, such as atrioventricular canal defects or double outlet right ventricle, may be complicated by an underdeveloped ventricle.

Surgical Treatment
Patients with single-ventricle defects often need multiple operations. These include shunts such as Blalock-Taussig (B-T) or Glenn, placing a band on the pulmonary artery, or the Fontan operation.

The Fontan operation largely separates the heart into two circulations. This lets oxygen-poor blood go to the lungs and oxygen-rich blood go to the body. The Fontan operation substantially reduces the mixing of blue and red blood and produces a normal or near-normal oxygen supply to the body. It also reduces the risk of a stroke or other complications, and decreases the workload on the single ventricle. A Fontan operation can't be done if you have pulmonary hypertension (high blood pressure in the lungs)

Medical
Single-ventricle defects are among the most complex congenital heart problems known. If you have this defect, you'll need regular checkups and ongoing care all your life. Many people with single-ventricle defects require daily or multiple medications. This care is best given by a cardiologist who's very familiar with the anatomical complexities and complications that these patients have. This requires the expertise of a pediatric or an adult cardiologist specifically trained in congenital heart disease.

You may need yearly checkups to monitor your health. This may mean that you require such tests as electrocardiogram (ECG), echocardiogram (ultrasound of the heart, including transesophageal echocardiograms), cardiac catheterization, Holter and arrhythmia event monitoring, and exercise stress-testing.

Activity Restrictions
You may need to limit your activity, particularly competitive sports. If you have decreased heart function or rhythm disturbances, you may need to limit your activity more. Your cardiologist will help you determine if you must limit activities.

Problems You May Have
Most patients with single-ventricle defects may have health problems. These include cyanosis (lower oxygen levels, causing blueness), lower energy and a higher risk of infections such as brain abscess or endocarditis (infection of the heart). These problems shorten the lives of some people.

If you've had surgery for a single-ventricle defect, you can live a relatively normal life. However, your ability to exercise vigorously will probably be reduced.

Several basic types of problems are most common in this group of people. These problems may relate to the person's age at the time of the operation and the type of surgery done. Potential problems include:

1. Rhythm problems, generally fast heart rate (tachycardia, supraventricular tachycardia, atrial flutter) or slow heart rate.
2. Fluid retention, particularly in the abdomen and lower extremities. Some adults may develop varicose veins after the operation.
3. More risk of a weakening and failing heart muscle when there's only one ventricle.
4. Blood clots inside the heart that may require anticoagulation therapy.

F ew reports exist, but some women have been able to conceive and carry a pregnancy to term after surgery. Be sure to consult your cardiologist and obstetrician before considering pregnancy.

congenital heart defects

Pulmonary Valve Stenosis

What It Is
Oxygen-depleted blood is pumped through the pulmonary valve to the lungs to pick up oxygen. Normally the pulmonary valve has three leaflets. If these leaflets are malformed, the valve may become narrowed (stenotic) or leaky (insufficient). The stenosis, insufficiency or both can be mild to severe.

Surgical Treatment
If the stenosis is severe, the pulmonary valve must be opened to increase blood flow to the lungs. A balloon-tipped catheter is used to do this. It dilates (widens) the valve when the balloon is inflated. The valve also may be opened during open-heart surgery.

If the insufficiency is severe, an operation is required to repair or replace the pulmonary valve.

Medical
If your pulmonary stenosis or insufficiency was severe, you'll need ongoing care to check for restenosis or increasing insufficiency. If it's mild or moderate, you probably won't need surgical treatment at first. But you will need ongoing follow-up so your cardiologist can check for arrhythmias or any signs of strain or dilation of your right ventricle. (The right ventricle is the heart chamber that pumps blood through the pulmonary valve.)

Activity Restrictions
If you have mild to moderate pulmonary stenosis or insufficiency, you probably won't need to limit your physical activity. If it's severe, you may need to limit your activity because of strain or dilation of the right ventricle or arrhythmias. Ask your cardiologist about exercise recommendations.

Problems You May Have

Symptoms
If you have mild pulmonary stenosis or insufficiency, you probably won't have any symptoms. If it's moderate or severe, you may not tolerate exercise well and may have shortness of breath or palpitations.

Pregnancy
You'll likely handle pregnancy well if you have mild or moderate stenosis or regurgitation. If your lesion is severe, pregnancy may be higher risk, particularly if you have an artificial valve. Your cardiologist should be involved in your care during pregnancy and delivery.

congenital heart defects

Coarctation of the Aorta

What It Is
The aorta is the body's main artery. It distributes oxygen-rich blood to all parts of the body except the lungs. The first branches of the aorta go to the upper body (arms and head). After that, blood goes to the lower body (abdomen and legs). Coarctation of the aorta is a narrowing of the aorta between the upper-body artery branches and the branches to the lower body. This blockage can increase blood pressure in your arms and head, reduce pressure in your legs and seriously strain your heart. Aortic valve abnormalities often accompany coarctation.

Surgical Treatment
The narrowing can be removed by surgery or sometimes by a nonsurgical balloon dilation in the cardiac catheterization lab. Aortic coarctation may return even after successful surgery or balloon dilation. This isn't uncommon if your repair was done when you were a newborn. (It's uncommon if it was repaired when you were a child.) If you've reached your full adult size and have no blood pressure difference between your arms and legs, it's highly unlikely that your aorta will become obstructed again.

Recurrent coarctation is usually treated with nonsurgical balloon dilation or by implanting a stent using cardiac catheterization.

Medical
After the coarctation is repaired, you'll need your blood pressure checked every 1-2 years. The reason is that you're at higher risk of developing generalized high blood pressure or problems with your aortic valve. Both of these can be checked for during your routine cardiology visits.

Activity Restrictions
Depending on your blood pressure at rest or during exercise, you may be advised to avoid some forms of strenuous exercise. Heavy isometric exercise, such as power weightlifting, may be a particular concern if your pressure is elevated. In general, you don't need to restrict activity if your arm and leg blood pressures are normal. Ask your cardiologist if you should limit any activity.

Pregnancy
Most women with repaired coarctation shouldn't have any difficulties, unless there's residual aortic obstruction or generalized high blood pressure. However, if you have persistent coarctation or any associated problems that might affect you or your baby, check with your physician before considering getting pregnant.

Problems You May Have

Symptoms
Coarctation of the aorta usually doesn't have symptoms. However, if the obstruction becomes severe, you may not tolerate exercise well. You could have a headache or leg pains after exertion. You also might have chest pain or palpitations. Tell your cardiologist promptly about any activity-related symptoms.

congenital heart defects

Aortic Valve Stenosis and Insufficiency

What It Is

The aortic valve lets blood flow from the heart's left ventricle into the aorta. (The aorta is the main artery and distributes oxygen-rich blood throughout the body.) The aortic valve closes after each heartbeat, to keep blood from leaking back into the left ventricle. A normal aortic valve has three leaflets or cusps (tricuspid). Aortic valves abnormal from birth usually have two leaflets (bicuspid), but other variations occur. Only about 2 percent of the population have biscuspid valves. Most bicuspid valves work normally for a long time — sometimes a lifetime. But some can become thick and narrowed (stenotic) or curled at the edges and leaky (insufficient).

Surgical Treatment
If the valve becomes very stenotic, severe pressure builds up in the left ventricle, which can injure the heart. When this occurs, the valve must be opened either 1) in the catheterization lab with a balloon valvotomy catheter or 2) in the operating room by open-heart surgery. You may have had one or both of these procedures as an infant or child.

Valvotomy by either technique doesn't repair the valve. Instead, it helps to lessen the stenosis and pressure in the left ventricle. Some valve leakage is likely to develop after balloon or surgical valvotomy. If your aortic valve no longer responds to valvotomy or has become severely insufficient (leaky), it will probably need to be replaced.

Your aortic valve can be surgically replaced in three ways:

1. The Ross procedure, a surgery in which your aortic valve is removed and replaced by your pulmonary valve. Then your pulmonary valve is replaced with a preserved donor pulmonary valve.
2. Aortic valve replacement with a preserved donor valve.
3. Aortic valve replacement with a mechanical valve.

your cardiologist, cardiac surgeon or both.

When serious stenosis persists by itself or with valve leakage, a more complex surgery may be needed. It will enlarge the part of the left ventricle that leads to the aortic valve (left ventricular outflow tract) when the valve is replaced. This surgery is often called the Konno procedure.

Medical
Everyone with aortic valve disease needs routine follow-up. The severity of your valve problem will dictate how often you'll need to visit the doctor. Stenosis and insufficiency can change as you age, whether or not you've had surgery or catheterization. Your doctor can check for this and other problems that sometimes coexist with aortic valve abnormalities. Medical therapy usually doesn't help people with aortic stenosis. However, medications can help reduce leakage and maintain left ventricle health in some patients with serious aortic valve insufficiency.

Activity Restrictions
If you haven't required aortic valve replacement but have ongoing valve stenosis or insufficiency, your cardiologist may tell you to limit your activity. For instance, heavy weightlifting and other extreme "isometric" exercises excessively strain your aortic valve and left ventricle. Depending on the state of your aortic valve, you

Each option has advantages and disadvantages. Discuss them with may need to limit such exercise. Ask your cardiologist about your exercise limits.

Endocarditis Prevention
People with even mildly abnormal aortic valves are at risk for bacterial endocarditis.

Pregnancy
You should handle pregnancy well if you have mild aortic stenosis or insufficiency. Your risk level depends on the severity of the valve obstruction or leakage. Women who have artificial or mechanical aortic valves, who receive warfarin (Coumadin) anticoagulation, or who receive blood vessel dilators (vasodilators such as Vasotec, Zestril, etc.) to treat severe aortic valve leakage must consult their physician
before considering pregnancy. These medications may injure the developing fetus.

Problems You May Have

Symptoms
Aortic stenosis and insufficiency usually don't have symptoms. However, when either becomes severe, activity may bring shortness of breath, exercise

arrhythmias (such as ventricular tachycardia). These can produce the symptoms described above. If you have any of these symptoms, report them to your physician.

congenital heart defects

Complete Atrioventricular Canal (CAVC)

What It Is
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:

1. a hole in the wall dividing the heart's upper chambers (atrial septal defect),
2. a hole in the wall separating the heart's lower chambers (ventricular septal defect) and
3. 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.

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.

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.

congenital heart defects

Ventricular Septal Defect (VSD)

What It Is
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 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.

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.

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.
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.

congenital heart defects

Atrial Septal Defect (ASD)

What It Is
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.

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.

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.

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.

congenital heart defects

How the heart works

To understand congenital heart defects, it's helpful to know how the normal heart works.

Your child's heart is a muscle about the size of his or her fist. It works like a pump and beats 100,000 times a day.

The heart has two sides, separated by an inner wall called the septum. The right side of the heart pumps blood to the lungs to pick up oxygen. Then, oxygen-rich blood returns from the lungs to the left side of the heart, and the left side pumps it to the body.

The heart has four chambers and four valves and is connected to various blood vessels. Veins are the blood vessels that carry blood from the body to the heart. Arteries are the blood vessels that carry blood away from the heart to the body.

The illustration shows a cross-section of a healthy heart and its inside structures. The blue arrow shows the direction in which oxygen-poor blood flows from the body to the lungs. The red arrow shows the direction in which oxygen-rich blood flows from the lungs to the rest of the body.

Heart Chambers

The heart has four chambers or "rooms."

• The atria (AY-tree-uh) are the two upper chambers that collect blood as it comes into the heart.
• The ventricles (VEN-trih-kuls) are the two lower chambers that pump blood out of the heart to the lungs or other parts of the body.

Heart Valves

Four valves control the flow of blood from the atria to the ventricles and from the ventricles into the two large arteries connected to the heart.

• The tricuspid (tri-CUSS-pid) valve is in the right side of the heart, between the right atrium and the right ventricle.
• The pulmonary (PULL-mun-ary) valve is in the right side of the heart, between the right ventricle and the entrance to the pulmonary artery, which carries blood to the lungs.
• The mitral (MI-trul) valve is in the left side of the heart, between the left atrium and the left ventricle.
• The aortic (ay-OR-tik) valve is in the left side of the heart, between the left ventricle and the entrance to the aorta, the artery that carries blood to the body.

Valves are like doors that open and close. They open to allow blood to flow through to the next chamber or to one of the arteries, and then they shut to keep blood from flowing backward.

When the heart's valves open and close, they make a "lub-DUB" sound that a doctor can hear using a stethoscope.

• The first sound - the "lub" - is made by the mitral and tricuspid valves closing at the beginning of systole (SIS-toe-lee). Systole is when the ventricles contract, or squeeze, and pump blood out of the heart.
• The second sound - the "DUB" - is made by the aortic and pulmonary valves closing at beginning of diastole (di-AS-toe-lee). Diastole is when the ventricles relax and fill with blood pumped into them by the atria.

Arteries

The arteries are major blood vessels connected to your heart.

• The pulmonary artery carries blood pumped from the right side of the heart to the lungs to pick up a fresh supply of oxygen.
• The aorta is the main artery that carries oxygen-rich blood pumped from the left side of the heart out to the body.
• The coronary arteries are the other important arteries attached to the heart. They carry oxygen-rich blood from the aorta to the heart muscle, which must have its own blood supply to function.

Veins

The veins are also major blood vessels connected to your heart.

• The pulmonary veins carry oxygen-rich blood from the lungs to the left side of the heart so it can be pumped out to the body.
• The vena cava is a large vein that carries oxygen-poor blood from the body back to the hear

congenital heart defects

What are congenital heart defects?

Congenital (kon-JEN-i-tal) heart defects are problems with the heart's structure that are present at birth. These defects can involve the interior walls of the heart, valves inside the heart, or the arteries and veins that carry blood to the heart or out to the body. Congenital heart defects change the normal flow of blood through the heart.

There are many different types of congenital heart defects. They range from simple defects with no symptoms to complex defects with severe, life-threatening symptoms.

Congenital heart defects are the most common type of birth defect, affecting 8 of every 1,000 newborns. Each year, more than 35,000 babies in the United States are born with congenital heart defects. Most of these defects are simple conditions that are easily fixed or need no treatment.

A small number of babies are born with complex congenital heart defects that need special medical attention soon after birth. Over the past few decades, the diagnosis and treatment of these complex defects has greatly improved.

As a result, almost all children with complex heart defects grow to adulthood and can live active, productive lives because their heart defects have been effectively treated.

What are birth defects?

Birth defects are structural or functional abnormalities present at birth that cause physical or mental disability. Some may be fatal.

Researchers have identified thousands of different birth defects. Currently, birth defects are the leading cause of death for infants during the first year of life.

What causes birth defects?

Birth defects have a variety of causes, such as:

• Genetic problems caused when one or more genes doesn't work properly or part of a gene is missing
• Problems with chromosomes, such as having an extra chromosome or missing part of a chromosome
• Environmental factors that a woman is exposed to during pregnancy, such as rubella or German measles while pregnant, or using drug or alcohol during pregnancy.

What are the different types of birth defects?

There are two main types of birth defects: structural and functional/developmental.

Structural birth defects are related to a problem with body parts. Some physical problems include cleft lip or cleft palate, heart defects, such as missing or misshaped valves, and abnormal limbs, such as a club foot. They also include neural tube defects, such as spina bifida, problems that are related to the growth and development of the brain and spinal cord.

Functional birth defects are related to a problem with how a body part or body system works. These problems often lead to developmental disabilities and can include things such as:

• Nervous system or brain problems - such as learning disabilities, mental retardation, behavioral disorders, speech or language difficulties, convulsions, and movement trouble. Some examples of birth defects that affect the nervous system include Autism, Down sysdrome, Prader – Willi syndrome, and Fragile X syndrome.
• Sensory problems - such as blindness, cataracts and other visual problems, and varying degrees of hearing loss including deafness
  
  Metabolic disorders - involve a body process or chemical pathway or reaction, such as conditions that limit the body's ability to get rid of waste materials or harmful chemicals. Two common metabolic disorders are phenylketonuria (PKU) and hypothyroidism.

• Degenerative disorders--are conditions that might not be obvious at birth, but cause one or more aspects of health to steadily get worse. For example, X-linked adrenoleukodystrophy (X-ALD), which was the focus of the movie Lorenzo's Oil, Rett syndrome, muscular dystrophy, and lysosomal disorders are examples of degenerative disorders. In some cases, birth defects are caused by a combination of factors. Some recognized patterns of birth defects affect many parts or processes in the body, leading to both structural and functional problems.

In some cases, birth defects are caused by a combination of factors. Some recognized patterns of birth defects affect many parts or processes in the body, leading to both structural and functional problems.

Congenital heart defects can be classified into several categories according to the problems your child may experience. They include the following:

• Problems that cause too much blood to pass through the lungs -- These defects allow oxygen-rich (red) blood that should be traveling to the body to re-circulate through the lungs, causing increased pressure and stress in the lungs. Examples include the following:
• patent ductus arteriosus (PDA)
• atrial septal defect (ASD)
• ventricular septal defect (VSD)
• atrioventricular canal (AV canal or AVC)

• Problems that cause too little blood to pass through the lungs — These defects allow blood that has not been to the lungs to pick up oxygen (and, therefore, is oxygen-poor) to travel to the body. The body does not receive enough oxygen with these heart problems, and the baby will be cyanotic, or "blue." Examples include the following:
• tricuspid atresia (TA)
• pulmonary atresia (PA)
• transposition of the great arteries (TGA)
• Tetralogy of Fallot (TOF)

• Problems that cause too little blood to travel to the body -- These defects are a result of underdeveloped chambers of the heart or blockages in blood vessels that prevent the proper amount of blood from traveling to the body to meet its needs. Examples include:
• coarctation of the aorta
• aortic stenosis
• pulmonary stenosis

• A combination of several heart defects -- These combinations create a more complex problem that can fall into several of these categories. Complex combination of heart defects include:
• hypoplastic left heart syndrome (HLHS)
• truncus arteriosis
• total anomalous pulmonary venous return (TAPVR)

congenital heart defects