Absent Ductus venosus in the Fetus: Review of the Literature and First Report of Direct Umbilical Venous Drainage to the Coronary Sinus

Abstract

The ductus venosus connects the portal and umbilical veins with the inferior vena cava and acts as a sphincter to protect the fetus from placental overcirculation. Its absence usually causes hydrops fetalis and is associated with high mortality rate, chromosomal anomalies and congenital malformations. In this condition, the umbilical vein almost always drains directly into right-sided structures such as inferior vena cava or right atrium. We reviewed the literature and describe the first case of a fetus with absent ductus venosus and direct connection of the umbilical vein to the coronary sinus.

Fetal umbilical–portal–systemic venous shunt: in‐utero classification and clinical significance

“…Most of the prenatal literature consists of case reports or case series of fetuses with absence of the DV, in which the shunt is mentioned as a cofeature and not the focal point, . Doing so prevents us from seeing the complete picture.…”

“…In adult life, any communication between the portal system and the systemic veins is known as a ‘congenital portal–systemic venous shunt’. Prenatally this entity has been investigated specifically in only three studies; in other in‐utero studies, cases have been reported within the context of diagnosing absence of the ductus venosus (DV) or ‘abnormalities of the fetal venous system’. Most of the existing information regarding its diagnosis, clinical symptoms and significance comes from pediatric and adult studies.…”

Shunt diameter in agenesis of the ductus venosus with extrahepatic portosystemic shunt impacts on prognosis

“…However, some of these cases are clinically silent; in the absence of other anomalies, it is possible that many such cases remain undetected. Prognosis may also depend on the existence of a shunt draining from the umbilical vein to the systemic venous circulation9, 10, 14–21.…”

Absent Ductus Venosus With Single Umbilical Artery: A Rare Association

“…This might be due to volume overload because of loss of regulatory mechanism by ductus venosus. 3,7,8,9,10 Single umbilical artery has not shown any co-relation with absent ductus venosus in the literature; however multiple aneuplodies have been reported. 11 Here we are presenting a case of absent ductus venosus with single umbilical artery which is extremely rare and other anomalies in form of mal-aligned spine, hydronephrotic kidneys.…”