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.

Alternative Names

Pulmonary vaso-occlusive disease

Causes, incidence, and risk factors

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.

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 , which is a thickening of the tips of the fingers) and a bluish coloration of the skin due to lack of oxygen ( cyanosis ). Tests can include:

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

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.