Case report : congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patientMatevž Harlander
, Maja Badovinac
, Frosina Markoska
, Barbara Salobir
, Tomaž Štupnik
, Marija Dolenšek
, Izidor Kern
, Vojka Gorjup
, Nazzareno Galiè
, 2022, short scientific article
Abstract: 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.
Keywords: pulmonary hypertension, pregnancy, congenital extrahepatic portocaval shunt, Abernethy malformation, pulmonary arterial hypertension
Published in DiRROS: 10.01.2022; Views: 438; Downloads: 249
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