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.

Atrial, sinoatrial node and atrioventricular node disease with preserved conductivity results in tachy-bradycardia syndrome.(1) Tachy-bradycardia syndrome has been reported in fetuses with the missense variant p.(Gly482Arg) in HCN4 gene.(2) Atrial ectopics and junction ectopic tachycardia were reported in fetuses with autoimmune atrioventricular block.(3,4) An unusual dominant involvement of sinoatrial node in a fetus presenting with tachy-bradycardia syndrome of Anti-SSA/SSB-Positive mother.

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.