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Pulmonary veno-occlusive disease

Definition

Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung area.

See also: 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, chemotherapy, or bone marrow transplantation.

The disorder is most common among children and young adults. As the disease gets worse, it causes narrowed pulmonary veins, pulmonary artery hypertension, and congestion and swelling of the lungs.

Symptoms

Signs and tests

The doctor will perform a physical exam.

The exam may reveal:

Your doctor may hear abnormal heart sounds when listening to the chest and lungs with a stethoscope.

The following tests may be done:

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.

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.


Review Date: 6/2/2010
Reviewed By: 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.
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