Ebstein's anomaly: unusual presentation and surgical repair

Ebstein's anomaly is the most significant congenital anomaly involving the tricuspid valve. This lesion may present in a simple form or may be complicated by other lesions. Patients with this anomaly may vary from mildly symptomatic to severely debilitated. The role of surgical treatment is not uniformly agreed upon, and the optimal procedure for correction or palliation is not clearly defined. Because of the wide spectrum of morphologic abnormalities which may be present in Ebstein's anomaly, no one particular operation may always be satisfactory. We and other groups continue to search for optimal procedures and feel strongly that individualization must be carried out to optimize the result in each patient. We encountered a patient with Ebstein's anomaly in an almost atretic tricuspid valve. In addition, there was severe right ventricular outflow tract obstruction and pulmonary valve stenosis. In this patient the "Fontan Principle" was successfully applied in his management.