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