Agenesis of the ductus venosus—A case with favorable outcome after early signs of cardiac failure

Abstract

Absence of the ductus venosus (ADV) is a rare vascular anomaly. Its prognosis depends on the pathway of the umbilical flow to the systemic venous circulation, and the presence or absence of associated structural or chromosomal anomalies, sometimes resulting in hydrops fetalis. In cases with isolated ADV in the absence of associated anomalies, survival rates are as high as 85%, depending on the shunt situation. Here, we report a patient with ADV and extrahepatic umbilical vein drainage with favorable outcome after intrauterine reversal of early signs of cardiac failure. Diagnosis was made after the appearance of moderate cardiomegaly in the 25th gestational week. Thus, in the case of cardiomegaly with or without further signs of cardiac failure, ultrasound imaging of the venous duct should be considered.

Umbilical Vein Insertion into the Inferior Vena Cava

“…Similarly, Staboulidou et al 8 reported that an absent DV as an isolated finding in fetuses with normal nuchal translucency resulted in healthy live births. Likewise, Hofmann et al 1 recently reported a fetus with a favorable outcome of agenesis of the DV. That fetus had communication of the UV to the right atrium, with subsequent cardiomegaly, which resolved at the age of 4 months.…”

“…The variant with extrahepatic shunts includes drainage of the UV into the internal iliac vein, the IVC (as in our cases), the renal vein, or directly into the right atrium, bypassing the liver completely. This variant may result in reduced perfusion and oxygenation of the liver, cardiomegaly, congestive heart failure, and intrauterine fetal death 1 , 6 , 10 – 13 . We have presented cases with extrahepatic shunts of the UV to the IVC.…”

“…This variant may result in reduced perfusion and oxygenation of the liver, cardiomegaly, congestive heart failure, and intrauterine fetal death. 1,6,[10][11][12][13] We have presented cases with extrahepatic shunts of the UV to the IVC. The 5 cases with additional anatomic and chromosomal anomalies terminated pregnancies before intrauterine fetal death could occur.…”

“…Abnormal drainage of the fetal umbilical vein (UV) with absence of the ductus venosus (DV) is a rare condition with a prevalence of 1 per 2500 fetuses 1 , 2 . Previous reports described an association with additional congenital malformations.…”

“…The incorporation of modern high‐resolution ultrasound machines combined with color Doppler sonography improved the detection of such anomalies to as early as the first trimester 1 , 2 . The objective of this series was to describe the sonographic characteristics and the pregnancy outcomes of 6 cases with abnormal insertion of the UV into the IVC as well as to emphasize the role of this finding with regard to chromosomal abnormalities.…”