Pulmonary veno-occlusive disease

Causes

In most cases, the cause of pulmonary veno-occlusive disease is unknown. The high blood pressure occurs in the pulmonary arteries, which are the lung arteries directly connected to the right side of the heart.

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 artery hypertension, and 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 or nurse will examine you and ask questions about your medical history and symptoms

The exam may reveal:

• Increased pressure in the neck veins
• 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.

The following tests may be done:

• Arterial blood gases
• Blood oximetry
• Chest x-ray
• Chest CT
• Cardiac catheterization
• Lung function tests
• 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.

Alternative Names

Pulmonary vaso-occlusive disease

References

Channick RN, Rubin LJ. Pulmonary hypertension. In: Mason RJ,VC Broaddus, Martin TR, et al, eds. Murray and Nadel’s Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 52.

McLaughlin V. Pulmonary hypertension. In: Goldman L, SchaferAI, eds. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 68.

McLaughlin VV, Archer SL, Badesch DB, et al: American College of Cardiology Foundation Task Force on Expert Consensus Documents;American Heart Association; American College of Chest Physicians; American Thoracic Society, Inc; Pulmonary Hypertension Association. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians: American Thoracic Society, Inc; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573-1619.

Rich S. Pulmonary hypertension. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 78.

Update Date: 6/5/2012

Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc