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 Iča Dolenšek; Izidor Kern; Vojka Gorjup; Nazzareno Galiè

doi:10.1002/pul2.12008

Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient

Pulm Circ. 2022 Jan 3;12(1):e12008. doi: 10.1002/pul2.12008. eCollection 2022 Jan.

Authors

Matevž Harlander^{1

2}, Maja Badovinac¹, Frosina Markoska¹, Barbara Salobir^{1

2}, Tomaž Štupnik^{2

3}, Marija Iča Dolenšek⁴, Izidor Kern⁵, Vojka Gorjup⁶, Nazzareno Galiè⁷

Affiliations

¹ Department of Pulmonary Diseases and Allergy University Medical Centre Ljubljana Ljubljana Slovenia.
² Faculty of Medicine Ljubljana University of Ljubljana Ljubljana Slovenia.
³ Department of Thoracic Surgery University Medical Centre Ljubljana Ljubljana Slovenia.
⁴ Institute of Radiology University Medical Centre Ljubljana Ljubljana Slovenia.
⁵ Cytology and Pathology Laboratory University Clinic of Respiratory and Allergic Diseases Golnik Golnik Slovenia.
⁶ Department of Intensive Internal Medicine University Medical Centre Ljubljana Ljubljana Slovenia.
⁷ DIMES S. Orsola University Hospital University of Bologna and IRCCS Bologna Italy.

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: Abernethy syndrome; portopulmonary hypertension; pregnancy.

Publication types

Case Reports