Pulmonary veno-occlusive disease

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Respiratory system

Overview

Symptoms

Treatment

Prevention

Pulmonary veno-occlusive disease

Alternative Names:

Pulmonary vaso-occlusive disease

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.

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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