In most cases, the cause of PVOD is unknown. The high blood pressure occurs in the pulmonary arteries. These lung arteries are 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 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
• Congestion and swelling of the lungs

Possible risk factors for PVOD include:

• Family history of the condition
• Smoking
• Exposure to substances like trichloroethylene or chemotherapy medicines
• Systemic sclerosis (autoimmune skin disorder)

Symptoms may include any of the following:

• Shortness of breath
• Dry cough
• Fatigue on exertion
• Fainting
• Coughing up blood
• Difficulty breathing while lying flat

Your health care provider will examine you and ask about your medical history and symptoms.

The exam may reveal:

• Increased pressure in the neck veins
• Clubbing of the fingers
• Bluish coloration of the skin due to lack of oxygen (cyanosis)
• Swelling in the legs

Your provider 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

There is currently no known effective medical treatment. However, the following medicines may be helpful for some people:

• Medicines that widen the blood vessels (vasodilators)
• Medicines that control the immune system response (such as azathioprine or steroids)

A lung transplant may be needed.

The outcome is often very poor in infants, with a survival rate of just a few weeks. Survival in adults may be months to a few years.

Complications of PVOD may include:

• Difficulty breathing that gets worse, including at night (sleep apnea)
• Pulmonary hypertension
• Right-sided heart failure (cor pulmonale)

Contact your provider if you have symptoms of this disorder.

Pulmonary vaso-occlusive disease

Churg A, Wright JL. Pulmonary hypertension. In: Smith ML, Leslie KO, Wick MR, eds. Practical Pulmonary Pathology: A Diagnostic Approach. 4th ed. Philadelphia, PA: Elsevier; 2024:chap 12.

Lammi MR, Mathai SC. Pulmonary hypertension: general approach. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 83.

Maron BA. Pulmonary hypertension. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 88.

Updated by: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.