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Pulmonary veno-occlusive disease Causes and Risks:
The cause is unknown, but it may be related to a thrombus ( clot ) formation after an injury, or it may be secondary to a viral infection. Young children and adults are affected most commonly. The progression of the disease leads to narrowed pulmonary veins, congestion, and edema of the lungs. Men are affected more frequently than women. The incidence is 1 out of 1,000,000 people.
Prevention:
unknown
Symptoms:
Signs and Tests:
Splitting of heart sounds may be present when the chest is examined with a stethoscope ( auscultation ). There may be nail abnormalities (finger clubbing , which is a thickening of the tips of the fingers) and a bluish coloration of the skin due to lack of oxygen ( cyanosis ).
Tests can include:
Treatment:
No specific treatment is available.
Prognosis:
The outcome is often very poor in infants with a survival rate of just a few weeks. The survival rate may be several years in adults.
Complications:
Call Your Healthcare Provider:
Call your health care provider if symptoms of this disorder develop. Fainting and episodes of no breathing are emergency/urgent symptoms.
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