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Ebstein's anomaly
Depending upon individual circumstances, treatment and prognosis can vary widely with Ebstein's anomaly, an abnormal tricuspid valve. Some people with Ebstein's anomaly live into their 60s and 70s with no treatment despite significant abnormalities in their tricuspid valves; others die in infancy. For people without symptoms, monitoring and observation including a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram, and an exercise test at regular intervals is necessary. A Holter monitor or event recorder might also be used to check for irregular heart rhythms. More detail about these tests can be found in the Testing section.
If symptoms develop, treatment is recommended to avoid future complications, in particular an enlarged heart, as well as to help control symptoms. Medication is usually the first line of treatment and may include diuretics to decrease swelling, minimize shortness of breath, and improve activity levels.
Abnormal heart rhythms are common in people with Ebstein's anomaly and may require electrophysiology studies to map out abnormal electrical conduction pathways. Most often these problems can be controlled by antiarrhythmic medication, heart surgery, catheter-based intervention, or a combination of treatments.
Surgery may be necessary to repair or replace a faulty tricuspid valve that is regurgitating blood or close the atrial septal defect, if present. More information is available in the section on Treatment for Valve Regurgitation or the section on Atrial Septal Defects. Surgery is usually postponed until the heart becomes enlarged, but should be performed before the right ventricle becomes severely enlarged and poorly functioning and before patients develop severe symptoms of shortness of breath. Heart rhythm problems should be evaluated prior to surgery, to identify abnormal electrical pathways that may be affected by the procedure.
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