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.
Pulmonary veno-occlusive disease
Alerts and Notices
Synopsis
Codes
ICD10CM:
I27.9 – Pulmonary heart disease, unspecified
SNOMEDCT:
89420002 – Pulmonary veno-occlusive disease
I27.9 – Pulmonary heart disease, unspecified
SNOMEDCT:
89420002 – Pulmonary veno-occlusive disease
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
- Pulmonary edema – Echocardiogram will show cardiac enlargement and right ventricular hypertrophy without evidence of pulmonary arterial hypertension.
- Chronic pulmonary thromboembolic disease
- Heart failure
- Mitral stenosis
- Fibrosing mediastinitis
- Sarcoidosis
- Interstitial lung disease
- Pneumoconioses (see pneumoconiosis)
- Pulmonary capillary hemangiomatosis
- Atrial myxoma
- Pulmonary arterial hypertension
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
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.
Subscription Required
References
Subscription Required
Last Reviewed:05/07/2019
Last Updated:01/23/2022
Last Updated:01/23/2022