Pulmonary veno-occlusive diseasePulmonary vaso-occlusive disease
Pulmonary veno-occlusive disease (PVOD) is a very rare disease. It leads to high blood pressure in the lung arteries (pulmonary hypertension).
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 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
- 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
- Coughing up blood
- Difficulty breathing while lying flat
Exams and Tests
The 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
- 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)
When to Contact a Medical Professional
Call your provider if you have symptoms of this disorder.
Chin K, Channick RN. Pulmonary hypertension. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 58.
Churg A, Wright JL. Pulmonary hypertension. In: Leslie KO, Wick MR, eds. Practical Pulmonary Pathology: A Diagnostic Approach. 3rd ed. Philadelphia, PA: Elsevier; 2018:chap 12.
Mclaughlin VV, Humbert M. Pulmonary hypertension. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 85.
Review Date: 5/27/2020
Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.