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

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 asymp...

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Detalles Bibliográficos
Autores principales: Harlander, Matevž, Badovinac, Maja, Markoska, Frosina, Salobir, Barbara, Štupnik, Tomaž, Dolenšek, Marija Iča, Kern, Izidor, Gorjup, Vojka, Galiè, Nazzareno
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Case Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9052963/
https://www.ncbi.nlm.nih.gov/pubmed/35506097
http://dx.doi.org/10.1002/pul2.12008
Descripción
Sumario: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.

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