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Case report : congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
Matevž Harlander, Maja Badovinac, Frosina Markoska, Barbara Salobir, Tomaž Štupnik, Marija Dolenšek, Izidor Kern, Vojka Gorjup, Nazzareno Galiè, 2022, other scientific articles

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: 857; Downloads: 491
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