Atrioventricular valve repair for patient with heterotaxy syndrome and a functional single ventricle

Heterotaxy syndrome is a mortality risk factor for patients with complex heart disease. These patients often have common atrioventricular valve (CAVV) morphology and significant atrioventricular valve regurgitation - yet another mortality risk factor in congenital heart disease. Thus, patients with both heterotaxy syndrome and atrioventricular valve regurgitation are at highest risk of mortality. A large number of patients with heterotaxy syndrome have CAVV and a functional single ventricle. These patients are more difficult to operate than patients with biventricular morphology because in the former, the CAVV is often less adapted to systemic afterload than the mitral valve. Herein, we present the results of atrioventricular valve repair surgeries performed at the Okayama University Hospital on patients with heterotaxy syndrome, atrial isomerism, and a functional single ventricle. In addition, we review the current literature in this typically challenging patient population. In-depth understanding of reasonable management strategies and resolution of technical issues will help guide surgeons during this procedure.