Pulmonary veno-occlusive disease

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Respiratory system

Pulmonary vaso-occlusive disease

Currently, there are no known effective medical treatments. Vasodilator drugs (drugs that dilate the blood vessels) that are used in other forms of pulmonary hypertension may be harmful in pulmonary veno-occlusive disease. Lung transplantation is the only treatment that has proven to be effective.

The outcome is often very poor in infants with a survival rate of just a few weeks. Survival may be months to a few years in adults.

• Progressive difficulty breathing
• Pulmonary hypertension
• Right sided heart failure (cor pulmonale)
• Coughing up blood

Call your health care provider if symptoms of this disorder develop. Fainting, shortness of breath, and episodes of no breathing are emergency/urgent symptoms.

Review Date: 05/03/2006
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.