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Ductus venosus agenesis

MedGen UID:
811050
•Concept ID:
C2363934
•
Congenital Abnormality
Synonym: Agenesis of ductus venosus
 
HPO: HP:0034196

Definition

A developmental defect characterized by lack of formation of the ductus venosus (a shunt that allows oxygenated blood in the umbilical vein to bypass the liver in the fetal circulation). [from HPO]

Professional guidelines

PubMed

Prenatal diagnosis of abnormality of the umbilical portal DV complex: difficulty in universal classification due to various alternative routes in hepatic circulation for placental drainage.
Demirci O, Akay HÖ
J Matern Fetal Neonatal Med 2022 Oct;35(20):3872-3884. Epub 2020 Nov 2 doi: 10.1080/14767058.2020.1842870. PMID: 33138669
Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies.
Volpe P, Marasini M, Caruso G, Lituania M, Marzullo A, Volpe G, Gentile M
Prenat Diagn 2002 Nov;22(11):995-1000. doi: 10.1002/pd.456. PMID: 12424763

Recent clinical studies

Etiology

Prenatal diagnosis of abnormality of the umbilical portal DV complex: difficulty in universal classification due to various alternative routes in hepatic circulation for placental drainage.
Demirci O, Akay HÖ
J Matern Fetal Neonatal Med 2022 Oct;35(20):3872-3884. Epub 2020 Nov 2 doi: 10.1080/14767058.2020.1842870. PMID: 33138669
Ductus venosus-systemic shunt. Report of six cases and systematic review of the literature.
Erenel H, Karsli MF, Ozel A, Korkmaz SO, Sen C
J Matern Fetal Neonatal Med 2020 Mar;33(6):1015-1023. Epub 2019 Jan 28 doi: 10.1080/14767058.2019.1569611. PMID: 30691333
Absent ductus venosus: case series from two tertiary centres.
Maruotti GM, Saccone G, Ciardulli A, Mazzarelli LL, Berghella V, Martinelli P
J Matern Fetal Neonatal Med 2018 Sep;31(18):2478-2483. Epub 2017 Jul 12 doi: 10.1080/14767058.2017.1344637. PMID: 28629280
Absent ductus venosus associated with persistent truncus arteriosus: prenatal diagnosis.
Tutar E, Fitoz S
Cardiol Young 2010 Jun;20(3):345-8. Epub 2010 Apr 26 doi: 10.1017/S1047951110000338. PMID: 20420741

Diagnosis

Ductus Venosus Agenesis as a Marker of Pallister-Killian Syndrome.
Lapresa Alcalde MV, Cubo AM, Martín Seisdedos MC, Cortejoso Hernández J, Doyague Sanchez MJ, Sayagués JM
Medicina (Kaunas) 2019 Jul 15;55(7) doi: 10.3390/medicina55070374. PMID: 31311125Free PMC Article
Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.
Pacheco D, Brandão O, Montenegro N, Matias A
J Perinat Med 2018 Dec 19;47(1):1-11. doi: 10.1515/jpm-2018-0163. PMID: 29949516
Absent ductus venosus: case series from two tertiary centres.
Maruotti GM, Saccone G, Ciardulli A, Mazzarelli LL, Berghella V, Martinelli P
J Matern Fetal Neonatal Med 2018 Sep;31(18):2478-2483. Epub 2017 Jul 12 doi: 10.1080/14767058.2017.1344637. PMID: 28629280
Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies.
Volpe P, Marasini M, Caruso G, Lituania M, Marzullo A, Volpe G, Gentile M
Prenat Diagn 2002 Nov;22(11):995-1000. doi: 10.1002/pd.456. PMID: 12424763
Prenatal diagnosis of ductus venosus agenesis: a report of two cases and review of the literature.
Gembruch U, Baschat AA, Caliebe A, Gortner L
Ultrasound Obstet Gynecol 1998 Mar;11(3):185-9. doi: 10.1046/j.1469-0705.1998.11030185.x. PMID: 9589141

Prognosis

Ductus venosus-systemic shunt. Report of six cases and systematic review of the literature.
Erenel H, Karsli MF, Ozel A, Korkmaz SO, Sen C
J Matern Fetal Neonatal Med 2020 Mar;33(6):1015-1023. Epub 2019 Jan 28 doi: 10.1080/14767058.2019.1569611. PMID: 30691333
Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.
Pacheco D, Brandão O, Montenegro N, Matias A
J Perinat Med 2018 Dec 19;47(1):1-11. doi: 10.1515/jpm-2018-0163. PMID: 29949516
Absent ductus venosus: case series from two tertiary centres.
Maruotti GM, Saccone G, Ciardulli A, Mazzarelli LL, Berghella V, Martinelli P
J Matern Fetal Neonatal Med 2018 Sep;31(18):2478-2483. Epub 2017 Jul 12 doi: 10.1080/14767058.2017.1344637. PMID: 28629280
Prenatal diagnosis of abnormal course of umbilical vein and ductus venosus agenesis: report of three cases.
Corbacioglu A, Aslan H, Dagdeviren H, Ceylan Y
J Clin Ultrasound 2012 Nov-Dec;40(9):590-3. Epub 2012 Feb 24 doi: 10.1002/jcu.21883. PMID: 22362124
Agenesis of the ductus venosus that is associated with extrahepatic umbilical vein drainage: prenatal features and clinical outcome.
Jaeggi ET, Fouron JC, Hornberger LK, Proulx F, Oberhänsli I, Yoo SJ, Fermont L
Am J Obstet Gynecol 2002 Oct;187(4):1031-7. doi: 10.1067/mob.2002.126292. PMID: 12389001

Clinical prediction guides

Ductus venosus-systemic shunt. Report of six cases and systematic review of the literature.
Erenel H, Karsli MF, Ozel A, Korkmaz SO, Sen C
J Matern Fetal Neonatal Med 2020 Mar;33(6):1015-1023. Epub 2019 Jan 28 doi: 10.1080/14767058.2019.1569611. PMID: 30691333
Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies.
Volpe P, Marasini M, Caruso G, Lituania M, Marzullo A, Volpe G, Gentile M
Prenat Diagn 2002 Nov;22(11):995-1000. doi: 10.1002/pd.456. PMID: 12424763
Agenesis of the ductus venosus that is associated with extrahepatic umbilical vein drainage: prenatal features and clinical outcome.
Jaeggi ET, Fouron JC, Hornberger LK, Proulx F, Oberhänsli I, Yoo SJ, Fermont L
Am J Obstet Gynecol 2002 Oct;187(4):1031-7. doi: 10.1067/mob.2002.126292. PMID: 12389001
Prenatal diagnosis of ductus venosus agenesis: a report of two cases and review of the literature.
Gembruch U, Baschat AA, Caliebe A, Gortner L
Ultrasound Obstet Gynecol 1998 Mar;11(3):185-9. doi: 10.1046/j.1469-0705.1998.11030185.x. PMID: 9589141

Recent systematic reviews

Ductus venosus-systemic shunt. Report of six cases and systematic review of the literature.
Erenel H, Karsli MF, Ozel A, Korkmaz SO, Sen C
J Matern Fetal Neonatal Med 2020 Mar;33(6):1015-1023. Epub 2019 Jan 28 doi: 10.1080/14767058.2019.1569611. PMID: 30691333
Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.
Pacheco D, Brandão O, Montenegro N, Matias A
J Perinat Med 2018 Dec 19;47(1):1-11. doi: 10.1515/jpm-2018-0163. PMID: 29949516

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