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Epidemiology, pathogenesis, clinical evaluation, and diagnosis of pulmonary veno-occlusive disease in adults

Jess Mandel, MD
Barbara LeVarge, MD
Section Editor
Scott Manaker, MD, PhD
Deputy Editor
Geraldine Finlay, MD


Pulmonary veno-occlusive disease (PVOD) is a rare condition that represents a small subgroup of adult patients with pulmonary hypertension (PH) (table 1) [1-3]. The terms "isolated pulmonary venous sclerosis," "obstructive disease of the pulmonary veins," and "venous form of primary pulmonary hypertension" were previously used to describe the syndrome [4-7]. Compared with pulmonary arterial hypertension (eg, idiopathic or heritable PAH), PVOD-associated PH has a grim prognosis and serious adverse effects can occur when pulmonary hypertension-specific advanced therapy is administered. Thus, the suspicion for and early identification of PVOD is critical to its management.  

The epidemiology, pathogenesis, clinical features, and diagnosis of PVOD are reviewed here. The treatment of PVOD-associated PH as well as the clinical features and diagnosis of other forms of pulmonary hypertension are discussed separately. (See "Treatment and prognosis of pulmonary veno-occlusive disease in adults" and "Classification and prognosis of pulmonary hypertension in adults" and "Clinical features and diagnosis of pulmonary hypertension in adults".)


The World Health Organization (WHO) has classified pulmonary hypertension (PH) based upon etiology and mechanism into the five groups listed below (table 1) [8,9]. Pulmonary arterial hypertension (PAH) refers to group 1 PAH. Pulmonary hypertension (PH) refers to any of group 2 through group 5 PH, and is also used when referring to all five groups collectively.

Group 1 – PAH (PVOD is a subgroup of this type of PAH)

Group 2 – PH due to left heart disease

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Literature review current through: Nov 2017. | This topic last updated: Nov 22, 2017.
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