We present a case of Aortic valve atresia (AVA) with type C interrupted aortic arch (IAA) in a neonate with absent left ventricle and mitral valve atresia. The hemodynamics of the lesion has been discussed with an illustrative cartoon.

First described in 1965 by Johnson and Wilcox, partial absence of the tricuspid valve (TV) associated with a ventricular septal defect(VSD) is a very rare malformation.(1) Heart failure secondary to severe TV regurgitation results in-utero fetal compromise. (2) We present here a fetus with partial absence of the tricuspid valve with conoventricular septal defect.

Aortic atresia is uncommonly associated with atrioventricular and ventriculoarterial discordance.(1) Presence of severe regurgitation of Ebsteinoid malformation of the tricuspid valve in this subset results in reduced aortic blood flow in-utero. The hemodynamic explanation of this anomaly was reported by Celermajer and colleagues.(2) We report here a term neonate with this anomaly detect antenatally.