Pulmonary veno-occlusive disease - Treatment

Alternative Names

Pulmonary vaso-occlusive disease

Treatment:

There is currently no known effective medical treatment. However, the following medications may be helpful for some patients:

Vasodilator drugs (drugs that widen the blood vessels)
Drugs that control immune system's response (such as azathioprine or steroids)

A lung transplant may be needed.

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 you have symptoms of this disorder.

Respiratory system

Reviewed last on: 6/2/2010
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Barst RJ. Pulmonary hypertension. In Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 67.

Channick RN, Rubin LJ. Pulmonary hypertension. In: Mason RJ, Broaddus CV, Martin TR, et al. Murray & Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 52.

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