Pulmonary veno-occlusive disease

• Illustrations
• Alternative names
• Definition
• Causes, incidence, and risk factors
• Symptoms

• Signs and tests
• Treatment
• Expectations (prognosis)
• Complications
• Calling your health care provider

Alternative names    Return to top

Definition    Return to top

Pulmonary veno-occlusive disease is a rare form of primary pulmonary hypertension (high blood pressure in the blood vessels of the lungs) in which progressive obstruction of the pulmonary veins leads to elevated blood pressure in the arteries of the lungs.

Causes, incidence, and risk factors    Return to top

In most cases, the cause is unknown, but it may be related to a viral infection. It may occur as a complication of certain connective tissue diseases, such as lupus or CREST, or as a complication of certain blood cancers (leukemia or lymphoma) or chemotherapy.

The disorder is most common amongst children and young adults. Progression of the disease leads to narrowed pulmonary veins, pulmonary hypertension, congestion, and edema (swelling) of the lungs.

Symptoms    Return to top

• Shortness of breath
• Fatigue on exertion
• Fainting
• Coughing up blood

Signs and tests    Return to top

Signs of high blood pressure in the veins of the lungs are seen with physical examination. Splitting of heart sounds may be present when the chest is examined with a stethoscope (auscultation). The jugular venous pressure may be elevated. There may be nail abnormalities (finger clubbing, an enlargement of the bases of the fingernails) and a bluish coloration of the skin due to lack of oxygen (cyanosis).

Tests can include:

• Arterial blood gases
• Chest X-ray
• Chest CT
• Cardiac catheterization
• Pulmonary angiogram
• Echocardiogram
• Lung biopsy

Treatment    Return to top

Currently, there are no known effective medical treatments. Vasodilator drugs (drugs that dilate the blood vessels) that are used in other forms of pulmonary hypertension may be harmful in pulmonary veno-occlusive disease. Lung transplantation is the only treatment that has proven to be effective.

Expectations (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.

Complications    Return to top

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

Calling your health care provider    Return to top

Call your health care provider if symptoms of this disorder develop. Fainting, shortness of breath, and episodes of no breathing are emergency/urgent symptoms.