The prenatal diagnosis of a rare circular shunt with absent pulmonary valve syndrome.

Abstract

Absent pulmonary valve syndrome (APVS) with tricuspid atresia is very rare. We report a case of APVS in which the fetus died due to severe hydrops fetalis. In addition to weakened cardiac contraction due to left ventricular noncompaction cardiomyopathy, a circular shunt through a ventricular septal defect exacerbated heart failure. Although APVS with tricuspid valve atresia is not supposed to cause a circular shunt, we herein present the echocardiographic demonstration of a case of circular shunt.