Case report : congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patientHarlander, Matevž (Avtor) Badovinac, Maja (Avtor) Markoska, Frosina (Avtor) Salobir, Barbara (Avtor) Štupnik, Tomaž (Avtor) Dolenšek, Marija (Avtor) Kern, Izidor (Avtor) Gorjup, Vojka (Avtor) Galiè, Nazzareno (Avtor) pulmonary hypertensionpregnancycongenital extrahepatic portocaval shuntAbernethy malformationpulmonary arterial hypertensionCongenital 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.Wiley20222022-01-10 13:26:06Neznano14709sl© 2021 The Authors