What is pulmonary veno-occlusive disease (PVOD)?

Updated: Oct 16, 2018
  • Author: Hakim Azfar Ali, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Pulmonary veno-occlusive disease (PVOD) is one of the less commonly encountered causes of pulmonary hypertension. Some reports suggest that PVOD accounts for 5-20% of cases classified as idiopathic pulmonary arterial hypertension (PAH). [1, 2, 3] PAH remains an incurable disease that results in significant morbidity and mortality. (See Epidemiology, Treatment, and Medication.)

In the past, PVOD has been described by various terms, such as pulmonary venous sclerosis, obstructive disease of the pulmonary veins, or the venous form of primary pulmonary hypertension. As the name suggests, PVOD is a clinicopathologic entity characterized by occlusion or narrowing of the pulmonary veins and venules by sometimes loose, sometimes more dense and collagen-rich, fibrous tissue, [4] leading to clinical manifestations that are, in many ways, similar to PAH. (See Pathophysiology, Etiology, Presentation, and Workup.) However, owing to the differences in pathology and response to PAH-specific therapy, it was classified in a unique group 1 in the pulmonary hypertension classification in 2009.

Although the term pulmonary veno-occlusive disease was first used in the 1960s, the first case was described by Dr J. Hora in 1934 in a 48-year-old patient who died within one year of diagnosis with symptoms of right-sided heart failure. [5] Historically, the disease has been underdiagnosed, possibly because of lack of awareness by clinicians.

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