Pulmonary veno-occlusive disease
Definition
Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung area.
See also: Primary pulmonary hypertension
Alternative Names
Pulmonary vaso-occlusive disease
Causes
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 hypertension, congestion, and swelling of the lungs.
Symptoms
- Shortness of breath
- Fatigue on exertion
- Fainting
- Coughing up blood
- Difficulty breathing while lying flat
Exams 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 vein (jugular vein)
- 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. This type of exam is called auscultation.
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.
Outlook (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.
Possible Complications
- Progressive difficulty breathing
- Pulmonary hypertension
- Right sided heart failure (cor pulmonale)
- Coughing up blood
When to Contact a Medical Professional
Call your health care provider if you have symptoms of this disorder.
References
Schwarz MI, Collard HR, King TE. Diffuse Alveolar Hemorrhage and Other Rare Infiltrative Disorders. In: Mason RJ, Murray J, VC Broaddus, Nadel J. Murray & Nadel's Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa: WB Saunders; 2005: chap. 56.
Reviewed By: Sean O. Stitham, MD, private practice in Internal Medicine, Seattle, Washington; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
