Surgery for Functional Single Ventricle Associated with Heterotaxy Syndrome: Common Atrioventricular Valve Repair

Abstract

Common atrioventricular valve regurgitation in functional single ventricle associated with heterotaxy syndrome and pulmonary venous obstruction should be repaired to achieve Fontan operation and improve life prognosis. Common atrioventricular valve repair consists mainly of bivalvation with edge-to-edge suturing of both bridging leaflets and bridging strip, combined with cleft suture, commissuroplasty, or partial annuloplasty. Valve replacement was occasionally performed, but the therapeutic effect remains controversial. Valve repair is preferred to be performed therapeutically or prophylactically at the timing of bidirectional Glenn. During the past decade, outcomes have improved along with the development of diagnostic technologies. However, the outcomes of valve repair before bidirectional Glenn are still dismal. Thus, the development of a small-caliber tissue-engineered heart valve with growth potential, increasing heart or heart/lung transplantation, and further improvement of repair technique per se are required.