Ebstein's Anomaly: Editorial Comments and Personal Observations

Abstract

Renewed interest in the natural history of Ebstein's anomaly has been created by recent surgical innovations in its management. Tricuspid valve replacement and various types of plastic reconstruction are the two methods of treating Ebstein's anomaly. Replacement of the tricuspid valve has been more successful in older children and adults than in infants, but overall, less successful than procedures involving a mitral or aortic valve prosthesis. At the Mayo Clinic, 42 patients with Ebstein's anomaly, aged 11 months to 62 years, underwent surgical repair. Plastic repair was accomplished in 34 of these patients, and right ventricular plication and valve replacement in 6 others. Two patients had valves that could not be reconstructed, and underwent a Fontan procedure instead. Three hospital deaths and two late deaths occurred. There have been no deaths in the 25 patients seen last in the series. Long-term follow-up was obtained in the first 22 survivors. The majority were in New York Heart Association (NYHA) Class III or IV preoperatively; all but 3 had improved to NHYA Class I or II at follow-up. Because of the low mortality rate and good long-term results as well as the fact that the majority of patients can be repaired with a plastic procedure, the operation is advisable for patients who have deteriorated into NYHA Class III or IV. Those patients with moderate to severe cyanosis, paradoxical emboli, or progressive increase in cardiac size are also candidates for operation.