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Pulm Circ. 2012 Apr-Jun;2(2):250-5. doi: 10.4103/2045-8932.97637.

Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension.

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Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, Rhode Island, USA.


Partial anomalous pulmonary venous return (PAPVR) is a rare cause of adult onset pulmonary arterial hypertension (PAH) that can present with a wide spectrum of severity from early childhood throughout adult life. We present two patients with PAH secondary to PAPVR who reflect this range of disease. The diagnosis and treatment of PAPVR and its role in pulmonary vascular disease is discussed. Cardiac and pulmonary physicians should be aware of this entity and its diagnosis and management options.


congenital disease; pulmonary arterial hypertension; vascular abnormalities

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