Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient.
Abernethy syndrome
portopulmonary hypertension
pregnancy
Journal
Pulmonary circulation
ISSN: 2045-8932
Titre abrégé: Pulm Circ
Pays: United States
ID NLM: 101557243
Informations de publication
Date de publication:
Jan 2022
Jan 2022
Historique:
received:
01
10
2021
revised:
20
11
2021
accepted:
22
11
2021
entrez:
4
5
2022
pubmed:
5
5
2022
medline:
5
5
2022
Statut:
epublish
Résumé
Congenital extrahepatic portocaval shunt (CEPS) is a rare condition in which a rare congenital vascular anomaly of the portal system is present. CEPS may manifest as pulmonary arterial hypertension (PAH). When diagnosed and treated early, PAH can be reversible. We report a case of a previously asymptomatic woman, who manifested with severe pulmonary hypertension during pregnancy and was consequently diagnosed with CEPS. After unsuccessful medical treatment, urgent lung transplantation was done.
Identifiants
pubmed: 35506097
doi: 10.1002/pul2.12008
pii: PUL212008
pmc: PMC9052963
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e12008Informations de copyright
© 2021 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.
Déclaration de conflit d'intérêts
The authors declare that there are no conflict of interests.
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