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Ductus venosus agenesis with extrahepatic drainage and liver bypass

MedGen UID:
1054002
Concept ID:
CN376966
Finding
HPO: HP:6000370

Definition

A form of ductus venosus agenesis in which umbilical vein does not connect to the portal venous system. The liver is bypassed and the umbilical vein drains into a systemic vein such as the inferior vena cava. This variant has been reported to have an unfavorable prognosis due to a presumed association with congestive heart failure and hydrops. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDuctus venosus agenesis with extrahepatic drainage and liver bypass

Recent clinical studies

Etiology

Berg C, Kamil D, Geipel A, Kohl T, Knöpfle G, Hansmann M, Gembruch U
Ultrasound Obstet Gynecol 2006 Sep;28(3):275-81. doi: 10.1002/uog.2811. PMID: 16826563

Diagnosis

Chan ES, Suchet I, Yu W, Somerset D, Soliman N, Kuret V, Chadha R
Pediatr Dev Pathol 2024 Mar-Apr;27(2):139-147. Epub 2023 Dec 14 doi: 10.1177/10935266231211760. PMID: 38098271Free PMC Article
Berg C, Kamil D, Geipel A, Kohl T, Knöpfle G, Hansmann M, Gembruch U
Ultrasound Obstet Gynecol 2006 Sep;28(3):275-81. doi: 10.1002/uog.2811. PMID: 16826563

Prognosis

Berg C, Kamil D, Geipel A, Kohl T, Knöpfle G, Hansmann M, Gembruch U
Ultrasound Obstet Gynecol 2006 Sep;28(3):275-81. doi: 10.1002/uog.2811. PMID: 16826563

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