Coordinated Health

Conditions

Definition

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

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 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

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

Signs and tests

The doctor or nurse will examine you and ask questions 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 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

Calling your health care provider

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

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