Modifications of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve.

Two modifications of the Fontan operation are proposed for prevention of pulmonary venous obstruction in patients with left atrioventricular valve atresia or single atrioventricular valve. In the first technique, a bipedicled flap of right atrial wall is used to separate systemic and pulmonary venous drainages; an atriopulmonary connection is constructed by use of the right atrial appendage, and the defect in the right atrial wall is repaired with a pericardial patch. In the second technique, the superior vena cava is transected and the cardiac and cephalad ends are anastomosed separately to the pulmonary arterial confluence. A patch is positioned inside the right atrium to separate the systemic from the pulmonary venous drainage. Both techniques achieve unrestricted systemic and pulmonary venous channels. Severe atrial arrhythmias, present in the four patients operated on with the first procedure, were not seen in 12 patients who had repair with the second procedure.