Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient | Zendy

Harlander Matevž | Zendy; Badovinac Maja | Zendy; Markoska Frosina | Zendy; Salobir Barbara | Zendy; Štupnik Tomaž | Zendy; Dolenšek Marija Iča | Zendy; Kern Izidor | Zendy; Gorjup Vojka | Zendy; Galiè Nazzareno | Zendy

Open Access

Case 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.

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