Agenesis of the venous duct: two cases of extrahepatic drainage of the umbilical vein and extrahepatic portosystemic shunt with a review of the literature

Cardiol Young. 2015 Feb;25(2):208-17. doi: 10.1017/S1047951114000729. Epub 2014 May 22.

Abstract

Agenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.

Keywords: ductus venosus.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abnormalities, Multiple*
  • Adult
  • Angiography
  • Echocardiography
  • Female
  • Heart Atria / abnormalities*
  • Hepatic Veins / abnormalities
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / etiology
  • Infant, Newborn
  • Portal Vein / abnormalities*
  • Pregnancy
  • Ultrasonography, Prenatal
  • Umbilical Veins / abnormalities*
  • Vascular Malformations / complications
  • Vascular Malformations / diagnosis*
  • Vena Cava, Inferior / abnormalities*
  • Young Adult

Supplementary concepts

  • Patent Ductus Venosus