Pulmonary veno-occlusive disease
Key points
- 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.
- In most cases, the cause is unknown, but it may be related to a viral infection.
- Signs of High blood pressure in the veins of the lungs are seen with physical examination.
- Currently, there are no known effective medical treatments.
Alternative names
Pulmonary vaso-occlusive disease
Definition
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
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
- 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 may be harmful in pulmonary veno-occlusive disease. Lung transplantation is the only treatment that has proven to be effective.
Written and revised by
David Cohen, M.D.
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