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Pulmonary veno-occlusive disease

Pulmonary vaso-occlusive disease

 

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:

  • Shortness of breath
  • Fatigue on exertion
  • Fainting
  • Coughing up blood (hemoptysis)
  • Difficulty breathing while lying flat

 

Exams and Tests

 

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

The exam may reveal:

  • Increased pressure in the neck veins
  • Fingernail clubbing
  • Bluish coloration of the skin due to lack of oxygen (cyanosis)
  • Swelling in the legs

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

The following tests may be done:

  • Arterial blood gases
  • Blood oximetry
  • Chest x-ray
  • Chest CT
  • Cardiac catheterization
  • Lung function tests
  • Echocardiogram
  • Lung biopsy

 

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

 


  • Progressive difficulty breathing
  • Pulmonary hypertension
  • Right-sided heart failure (cor pulmonale)
  • Coughing up blood

 

When to Contact a Medical Professional

 

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

 

 

References

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.

McLaughlin V. Pulmonary hypertension. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 68.

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        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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