Open AccessCase report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
Author(s) -
Harlander Matevž,
Badovinac Maja,
Markoska Frosina,
Salobir Barbara,
Štupnik Tomaž,
Dolenšek Marija Iča,
Kern Izidor,
Gorjup Vojka,
Galiè Nazzareno
Publication year - 2022
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1002/pul2.12008
Subject(s) - medicine , pulmonary hypertension , asymptomatic , shunt (medical) , pregnancy , persistent pulmonary hypertension , portal hypertension , cardiology , portopulmonary hypertension , surgery , lung , transplantation , liver transplantation , cirrhosis , genetics , biology
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.