Double outlet right ventricle (DORV) with remote ventricular septal defect (VSD)

Since Kirklin described the first successful correction of double-outlet right ventricle (DORV), surgical repair has been extended to more complex forms of the malformation [1]. In 1972, Lev and Bharati introduced their classification according to the anatomic position of the VSD as related to the arterial trunks [2]. The term ‘non-committed’ (or remote) was used to define hearts in which the ventricular septal defect (VSD) was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. DORV with non-committed VSD has challenged surgeons throughout the modern era of congenital heart surgery [3–5]. This subset was known to have a poor outcome and higher risk for reoperation, and was frequently treated by an univentricular repair [6]. To our knowledge, this is the first serial report on the surgical management of this subset.