%0 Case Reports
%T Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient.
%A Harlander M
%A Badovinac M
%A Markoska F
%A Salobir B
%A Štupnik T
%A Dolenšek MI
%A Kern I
%A Gorjup V
%A Galiè N
%J Pulm Circ
%V 12
%N 1
%D Jan 2022
%M 35506097
%F 2.886
%R 10.1002/pul2.12008
%X 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.