Modified Fontan procedure for severe Ebstein's malformation with predominant tricuspid stenosis.

Two children, aged 7 and 6 years, had severe Ebstein's malformation of the tricuspid valve and underwent operation because of progressive cyanosis (saturation, 70%). Preoperative angiography demonstrated linear attachment of the distal edges of the displaced anterosuperior and mural leaflets, leaving only a "keyhole" communication between the atrialized and functional portions of the right ventricle. Operation was directed toward completely excising these dysplastic leaflets, which were obstructing the flow of blood. The continuity between the functional right ventricle and pulmonary trunk was left intact. In addition, an atriopulmonary truncal connection was established. Postoperatively, both children were in functional class I with arterial saturation of 95%. Angiography and Doppler echocardiography demonstrated that there was a dominant phase of systolic flow of blood up the pulmonary trunk from the right ventricle, and also diastolic filling of the pulmonary arteries through the atriopulmonary truncal connection. The filling patterns did not change over time for the follow-up period of 4 and 1.5 years, respectively. This modified Fontan procedure appears to be an effective surgical alternative for some patients with severe Ebstein's malformation and predominant tricuspid stenosis.