Definition of Pulmonary veno-occlusive disease:

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

See also: Primary pulmonary hypertension

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:

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

Signs and tests:

The doctor will perform a physical exam.

The exam may reveal:

• High blood pressure in the veins of the lungs
• 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
• Chest x-ray
• Chest CT
• Cardiac catheterization
• Pulmonary angiogram
• 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:

• 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.