Tricuspid annuloplasty and ventricular plication for Ebstein's malformation.

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.

Bidirectional cavopulmonary shunt in patients with multiple risk factors.

BACKGROUND: Twenty-five patients in whom a modified Fontan operation was deferred because of multiple risk factors underwent a bidirectional cavopulmonary shunt. Two or more of the following risk factors were present in all: age less than 1 year, severe pulmonary artery distortion, impaired left ventricular function, subaortic obstruction, anomalous systemic-pulmonary venous connection, atrioventricular valve incompetence, and increased mean pulmonary artery pressure. METHODS: Additional procedures included take-down of systemic-pulmonary artery shunt, atrial septectomy, pulmonary artery reconstruction, bulboventricular foramen enlargement, and atrioventricular valve repair. RESULTS: There were three hospital deaths (12%). Mean follow-up is 21 months. There was no late mortality. Mean oxygen saturation increased from 71% to 83%. Results obtained from pulmonary artery reconstruction, enlargement of bulboventricular foramen, and atrioventricular valve repair were satisfactory for the patients in whom these risk factors were present preoperatively. Ventricular function also improved in the survivors in whom it was previously deteriorated, this being related to the suppression of the sources of ventricular volume overload. CONCLUSIONS: In this risk group of patients for a modified Fontan operation, a bidirectional cavopulmonary shunt provided adequate palliation at reasonable low risk. Early bidirectional cavopulmonary shunt would minimize complications originating from systemic-pulmonary shunts such as pulmonary artery distortion and the potential harm of chronic ventricular volume overload.