Surgical management for functional single ventricle with coarctation and interruption of the aortic arch

Abstract

Objective: To evaluate the surgical outcomes of patients with a functional single ventricle accompanied by coarctation of the aorta or an interrupted aortic arch (CoA/IAA) after staged palliation.
Methods: We compared surgical outcomes and cardiac functions between patients with a functional single ventricle and CoA/IAA after bilateral pulmonary artery banding as the first palliation (n = 13) or aortic arch repair with pulmonary trunk banding (n = 11).
Results: One patient in the bilateral pulmonary artery banding group with asplenia syndrome and severe common atrioventricular valve regurgitation died three months after palliation, seven completed the Fontan procedure as the 3^rd stage of repair and five others who underwent the Norwood-Glenn operation as the interim procedure after the initial palliation await the completion of the Fontan operation. A good end-diastolic volume index and ejection fraction were maintained in these patients while waiting for the Fontan operation.
Three patients in the aortic arch repair with pulmonary trunk banding group died and two others dropped out from BDG/Fontan track because subaortic stenosis progressed, and the first palliation exacerbated atrioventricular valve regurgitation. End-diastolic volumes increased over time in the remaining six patients during BDG/Fontan track.
Conclusions: The surgical outcomes for patients with a functional single ventricle and CoA/IAA in whom subaortic stenosis progressed after aortic arch repair with pulmonary trunk banding were unsatisfactory. Bilateral pulmonary artery banding as the first palliation followed by the Norwood-Glenn operation reduced the risk of subaortic stenosis and atrioventricular valve regurgitation, and promoted hemodynamic stability and candidacy for the Fontan operation.