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Pulmonary veno-occlusive disease
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Pulmonary veno-occlusive disease

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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.

For more information on the autosomal dominant form, see OMIM.

For more information on the autosomal recessive form, see OMIM.

Codes

ICD10CM:
I27.9 – Pulmonary heart disease, unspecified

SNOMEDCT:
89420002 – Pulmonary veno-occlusive disease

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 05/08/2019
Last Updated: 05/08/2019
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Pulmonary veno-occlusive disease
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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 © 2019 VisualDx®. All rights reserved.