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A.D.A.M. Medical Encyclopedia [Internet]. Atlanta (GA): A.D.A.M.; 2013.

A.D.A.M. Medical Encyclopedia.

Pulmonary veno-occlusive disease

Pulmonary vaso-occlusive disease

Last reviewed: May 29, 2014.

Pulmonary veno-occlusive disease is an extremely rare disease that causes high blood pressure in the lung arteries (pulmonary hypertension).

Causes

In most cases, the cause of pulmonary veno-occlusive disease is unknown. The high blood pressure occurs in the pulmonary arteries, which are the lung arteries directly connected to the right side of the heart.

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

Symptoms may include any of the following:

Exams and Tests

The doctor or nurse will examine you and ask about your medical history and symptoms.

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 the immune system response (such as azathioprine or steroids)

A lung transplant may be needed.

Outlook (Prognosis)

The outcome is often very poor in infants, with a survival rate of just a few weeks. Survival in adults may be months to a few years.

Possible Complications

When to Contact a Medical Professional

Call your health care provider if you have symptoms of this disorder.

References

  1. Channick RN, Rubin LJ. Pulmonary hypertension. In: Mason RJ, Broaddus VC, Martin TR, et al., eds. Murray and Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, PA: Elsevier Saunders; 2010:chap 52.
  2. McLaughlin V. Pulmonary hypertension. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 68.

Review Date: 5/29/2014.

Reviewed by: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

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