Large intra-abdominal umbilical vein varix with absent ductus venosus: The undeniable etiology of fetal heart failure despite associated congenital heart disease

Abstract

Fetal intra-abdominal umbilical vein varix (IUVV) is one of the rare anomalies of the umbilical vessels that simulate a cystic structure but with a vascular nature. IUVV usually drains into IVC through ductus venosus (DV), with evidence of an increase in the cardiac preload in most cases. In the current report, we present a fetus with congenital heart disease; however, the association of large IUVV with DV agenesis and direct drainage into the heart resulted in a high output fetal heart failure.

Keywords: congenital heart disease; ductus venosus; intra-abdominal umbilical vein varix.