| Author/Editor | Harlander, Matevž; Badovinac, Maja; Markoska, Frosina; Salobir, Barbara; Štupnik, Tomaž; Dolenšek, Marija; Kern, Izidor; Gorjup, Vojka; Galiè, Nazzareno | |
| Title | Case report | |
| Type | članek | |
| Vol. and No. | Letnik 12, št. 1 | |
| Publication year | 2022 | |
| Volume | str. 1-4 | |
| ISSN | 2045-8940 - Pulmonary circulation | |
| Language | eng | |
| 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. | |
| Descriptors | Hypertension, pulmonary Pregnancy Pljučna hipertenzija Nosečnost | |
| Keywords | prirojeni ekstrahepatični portokavalni šant Abernethyjev sindrom pljučna arterijska hipertenzija congenital extrahepatic portocaval shunt Abernethy malformation pulmonary arterial hypertension |