Pulmonary Arterial Hypertension Combined with a High Cardiac Output State: Three Remarkable Cases


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.

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Congenital Heart Disease


Onno A. Spruijt, Harm-Jan Bogaard, Anton Vonk-Noordegraaf

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Pulmonary Circulation Vol 3: No 2 cover image

June 2013

Pulmonary Circulation Vol 3: No 2

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