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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: 6/10/2008
Sean O. Stitham, MD, private practice in Internal Medicine, Seattle, Washington; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Schwarz MI, Collard HR, King TE. Diffuse Alveolar Hemorrhage and Other Rare Infiltrative Disorders. In: Mason RJ, Murray J, VC Broaddus, Nadel J. Murray & Nadel's Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa: WB Saunders; 2005: chap. 56.

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