[Definitive repair for double inlet ventricle and common atrio-ventricular valve]

From 1982 through 1990, 13 patients with double inlet ventricle and common atrio-ventricular valve underwent definitive cardiac surgery. Nine patients simultaneously had anomalous systemic venous connection and 4 had anomalous pulmonary venous connection. One patient with double inlet left ventricle underwent a successful partition of ventricle and atrio-ventricular valve. All other 12 patients underwent a Fontan operation, which utilized different techniques to deal with various forms of anomalous systemic and pulmonary venous connection. Four patients with common atrioventricular valve regurgitation underwent a Fontan operation combined with a concomitant circular annuloplasty of atrio-ventricular valve. In all 4 patients, the degree of regurgitation decreased postoperatively. There was one operative death (mortality 7.7%). Most of patients with double inlet ventricle and common atrio-ventricular valve now are considered to be suitable not for partition but for the Fontan operation. Because of the complexity of anatomic variables, however, the repair of anomalous systemic or pulmonary venous connections in conjunction with the Fontan operation requires an individualised plan in each patient to provide unobstructed systemic and pulmonary venous pathways. We think that a circular annuloplasty could effectively decrease the degree of atrio-ventricular valve regurgitation in most cases.