Health Care Encyclopedia
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Pulmonary veno-occlusive diseaseDefinition: 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.
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:
Treatment: 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.
Expectations (prognosis): 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.
Complications: - Progressive difficulty breathing
- Pulmonary hypertension
- Right sided heart failure (cor pulmonale)
- Coughing up blood
Calling your health care provider: Call your health care provider if symptoms of this disorder develop. Fainting, shortness of breath, and episodes of no breathing are emergency/urgent symptoms.
References: Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 7th ed. St. Louis, Mo; WB Saunders; 2005:829. Mason RJ, Murray J, VC Broaddus, Nadel J. Murray & Nadel's Textbook of Respiratory Medicine. 3rd ed. Philadelphia, Pa: WB Saunders; 2000:1472-1473.�
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| 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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