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 Pulmonary Vascular Disease

Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease

Respiratory system
Respiratory system

Definition:

Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung area (primary pulmonary hypertension).

Alternative Names:

Pulmonary vaso-occlusive disease

Causes, incidence, and risk factors:

In most cases, the cause of pulmonary veno-occlusive disease is unknown. The condition may be related to a viral infection. It may occur as a complication of certain diseases such as lupus, or as a complication of leukemia, lymphoma, or chemotherapy.

The disorder is most common among children and young adults. As the disease gets worse, it causes narrowed pulmonary veins, pulmonary hypertension, congestion, and edema (swelling) of the lungs.

Symptoms:
Signs and tests:

Signs of high blood pressure in the veins of the lungs are seen with physical examination. Splitting of heart sounds may be present when the chest is examined with a stethoscope (auscultation). The jugular venous pressure may be elevated. There may be nail abnormalities (finger clubbing, an enlargement of the bases of the fingernails) and a bluish coloration of the skin due to lack of oxygen (cyanosis).

Tests can include:


Review Date: 5/3/2006
Reviewed By: David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.

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