Ebstein's Anomaly

Treatment

Treatment of Ebstein's anomaly depends on the severity of the defect and the patient's symptoms. The goal of treatment is to minimize symptoms and avoid future complications, such as progressive heart enlargement and arrhythmias.

Observation and monitoring

For people without symptoms or heart rhythm disturbances, observation and monitoring requires regular evaluation by a cardiologist. A typical evaluation would generally include a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram, and an exercise test. A Holter monitor or event recorder may also be used to check for irregular heart rhythms.

Medications

Medication may be prescribed to enhance heart contraction and control abnormal heart rhythm. Medication may be indicated if symptoms such as shortness of breath, decreased activity tolerance, edema, and palpitations occur, or if the heart begins to enlarge.

Endocarditis prevention is always indicated in patients with Ebstein's anomaly.

Surgery

Surgical repair is recommended when symptoms are troublesome or when the heart begins to enlarge and overall heart function begins to decrease. Surgery may be for repair or replacement of the tricuspid valve and/or for correction of any associated defect such as closure of an atrial septal defect.

Evaluation of rhythm problems and abnormal conduction pathways

Rhythm problems are common in patients with Ebstein's anomaly. These should be evaluated prior to surgery to avoid postoperative complications. Often, abnormal rhythms can be controlled by medication, surgery, catheter-based intervention or a combination of treatments.

The patient's doctor may decide to order an electrophysiology study to identify the presence of abnormal electrical conduction pathways. If a pathway is present, it can be interrupted during the surgical repair. If surgery is not planned, catheter-based intervention may be possible.

Pregnancy and family planning

Pregnancy plans should be discussed with the doctor before pregnancy occurs. Close monitoring by an adult congenital cardiologist during pregnancy, labor, delivery and for a period after the pregnancy is recommended.