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Pulmonary veno-occlusive disease
Other Resources UpToDate PubMed

Pulmonary veno-occlusive disease

Contributors: Casey Silver MD, Mary Anne Morgan MD, Michael W. Winter MD
Other Resources UpToDate PubMed

Synopsis

Pulmonary veno-occlusive disease (PVOD) is a rare disease of pulmonary vein occlusion due to the presence of abnormal fibrous tissue, resulting in pulmonary hypertension. Its etiology is not well understood. Some reports have shown a genetic basis, while others have found infectious associations. It occurs most frequently in children and young adults. PVOD is a very rare condition, estimated at about 0.1 cases per million individuals, and is the cause of pulmonary arterial hypertension in approximately 10% of patients.

Common findings include exertional dyspnea, lethargy, chest pain, pleural effusion, syncope, orthopnea, and nail clubbing. PVOD may be misdiagnosed as pulmonary hypertension.

There is no current effective treatment, and survival prognosis ranges from a few weeks to a few years.

Codes

ICD10CM:
I27.9 – Pulmonary heart disease, unspecified

SNOMEDCT:
89420002 – Pulmonary veno-occlusive disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

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References

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Last Reviewed:05/07/2019
Last Updated:01/23/2022
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Pulmonary veno-occlusive disease
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A medical illustration showing key findings of Pulmonary veno-occlusive disease : Chest pain, Cough, Fatigue, Hepatomegaly, Exertional dyspnea, Ground glass opacity, Neck vein distension, Pleural effusion, Syncope, Hemoptysis, RUQ pain, Orthopnea
Copyright © 2024 VisualDx®. All rights reserved.