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Pulm Circ. 2013 Apr;3(2):440-3. doi: 10.4103/2045-8932.113185.

Pulmonary arterial hypertension combined with a high cardiac output state: Three remarkable cases.

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  • 1Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands.


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.


abernethy malformation; congenital extrahepatic portosystemic shunt; congenital extrahepatic portosystemic venous shunt; pulmonary arterial hypertension

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