Ebstein's malformation. Surgical experience at the Mayo Clinic

At the Mayo Clinic, 13 patients with Ebstein's malformation have undergone surgical repair since 1963. Their ages ranged from 18 months to 51 years (median 13 years). Ten patients were in Functional Class III or IV. Marked cardiomegaly, cyanosis, paradoxic emboli, and dysrhythmias secondary to Wolff-Parkinson-White syndrome were indications for operation in the remaining 3 patients. A wide range of anatomic variations was encountered. All 5 patients who underwent tricuspid annuloplasty with plication of the atrialized segment of the right ventricle survived operation. Of 5 patients who underwent prosthetic valve replacement, only one survived. Other procedures included atrial septal defect closure alone in one patient, atrial septal defect closure and relief of pulmonary stenosis in one patient, and tricuspid annuloplasty alone in one patient. One patient had concomitant mapping and division of anomalous conduction pathways. Functional classification improved in 8 of 10 operative survivors. There were two late sudden deaths; both patients had had preoperative dysrhythmias. The data suggest that results are improved when the atrialized segment of the right ventricle is dealt with during repair of the tricuspid valve. A combined ventricular plication and tricuspid annuloplasty yielded better early and late results than did valve replacement.