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Alternative Names Return to topPulmonary vaso-occlusive disease
Definition Return to top
Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung area.
See also: Primary pulmonary hypertension
Causes Return to top
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 hypertension, congestion, and swelling of the lungs.
Symptoms Return to top
Exams and Tests Return to top
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. This type of exam is called auscultation.
The following tests may be done:
Treatment Return to top
There is currently no known effective medical treatment. However, the following medications may be helpful for some patients:
A lung transplant may be needed.
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if you have symptoms of this disorder.
References Return to top
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.Update Date: 6/10/2008 Updated by: 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.