BACKGROUND: Seven patients with the diagnosis of Ebstein's malformation of the tricuspid valve were operated on. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present. METHODS: Surgical repair included tricuspid annuloplasty associated with longitudinal plication of the atrialized portion of the right ventricle. This was attained by approximating the anterior-posterior commissure with either the posterior-septal commissure or the septal leaflet remnant. The thin atrialized ventricular wall thus excluded remained as a cul du sac and was plicated by suturing along the longitudinal axis of the heart. When present, the dysplastic posterior leaflet was included in the plication. In essence, a monocuspid right atrioventricular valve was fashioned out of the anterior leaflet. The remaining septal leaflet played a minimal functional role. No additional procedures for treatment of arrhythmia were associated with the technique described. RESULTS: The postoperative course was uneventful in all patients. Mean follow-up is 4.3 years (range, 1 to 10 years). Doppler echocardiographic studies reveal satisfactory monocusp valve function in all patients, with adequate coaptation of the anterior leaflet and the septal structures. CONCLUSIONS: This technique seems applicable to most forms of Ebstein's malformation and is reproducible. The technique relies on the adequate mobilization of the anterior leaflet. Occasionally it is necessary to free fibrous adhesions of the leaflet to the underlying ventricular surface.
BACKGROUND: Seven patients with the diagnosis of Ebstein's malformation of the tricuspid valve were operated on. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present. METHODS: Surgical repair included tricuspid annuloplasty associated with longitudinal plication of the atrialized portion of the right ventricle. This was attained by approximating the anterior-posterior commissure with either the posterior-septal commissure or the septal leaflet remnant. The thin atrialized ventricular wall thus excluded remained as a cul du sac and was plicated by suturing along the longitudinal axis of the heart. When present, the dysplastic posterior leaflet was included in the plication. In essence, a monocuspid right atrioventricular valve was fashioned out of the anterior leaflet. The remaining septal leaflet played a minimal functional role. No additional procedures for treatment of arrhythmia were associated with the technique described. RESULTS: The postoperative course was uneventful in all patients. Mean follow-up is 4.3 years (range, 1 to 10 years). Doppler echocardiographic studies reveal satisfactory monocusp valve function in all patients, with adequate coaptation of the anterior leaflet and the septal structures. CONCLUSIONS: This technique seems applicable to most forms of Ebstein's malformation and is reproducible. The technique relies on the adequate mobilization of the anterior leaflet. Occasionally it is necessary to free fibrous adhesions of the leaflet to the underlying ventricular surface.