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
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