title: Pulmonary arterial hypertension combined with a high cardiac output state: Three remarkable cases.
abstract: A congenital extrahepatic portosystemic venous shunt (CEPVS), also known as an Abernethy malformation, is a rare cause of pulmonary arterial hypertension (PAH). In this case series, we describe three male patients of 30, 23, and 27 years of age with PAH due to a CEPVS. In all three patients, a right heart catheterization revealed a high cardiac output. The aim of this case series is to make pulmonary hypertension physicians aware of the possibility of a CEPVS when PAH is accompanied with a high cardiac output state.
authors.affiliation: Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Onno A
authors.initials: OA
authors.lastname: Spruijt
authors.forename: Harm-Jan
authors.initials: HJ
authors.lastname: Bogaard
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk-Noordegraaf
keywords: abernethy malformation; congenital extrahepatic portosystemic shunt; congenital extrahepatic portosystemic venous shunt; pulmonary arterial hypertension