{Reference Type}: Case Reports
{Title}: Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient.
{Author}: Harlander M;Badovinac M;Markoska F;Salobir B;Štupnik T;Dolenšek MI;Kern I;Gorjup V;Galiè N;
{Journal}: Pulm Circ
{Volume}: 12
{Issue}: 1
{Year}: Jan 2022
{Factor}: 2.886
{DOI}: 10.1002/pul2.12008
{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.