Pulmonary veno-occlusive disease

Definition

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

The disorder is most common among children and young adults. As the disease gets worse, it causes narrowed pulmonary veins, pulmonary hypertension, congestion, and edema (swelling) of the lungs.

Symptoms

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

Signs and tests

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

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)

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 symptoms of this disorder develop. Fainting, shortness of breath, and episodes of no breathing are emergency/urgent symptoms.

References

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 7th ed. St. Louis, Mo; WB Saunders; 2005:829.

Mason RJ, Murray J, VC Broaddus, Nadel J. Murray & Nadel's Textbook of Respiratory Medicine. 3rd ed. Philadelphia, Pa: WB Saunders; 2000:1472-1473.