Pulmonary Veno-Occlusive DiseaseSymptoms, Doctors, Treatments, Advances & More
Learn About Pulmonary Veno-Occlusive Disease
Pulmonary veno-occlusive disease (PVOD) is a very rare disease. It leads to high blood pressure in the lung arteries (pulmonary hypertension).
Pulmonary vaso-occlusive disease
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 disorder that also causes skin symptoms)
Symptoms may include any of the following:
- Shortness of breath
- Dry cough
- Fatigue on exertion
- Fainting
- Coughing up blood
- Difficulty breathing while lying flat
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.
Advocate Heart Institute
Muhyaldeen Dia is an Advanced Heart Failure and Transplant Cardiologist practicing medicine in Oak Lawn, Illinois. Dr. Dia is rated as a Distinguished provider by MediFind in the treatment of Pulmonary Veno-Occlusive Disease. He is also highly rated in 15 other conditions, according to our data. His clinical expertise encompasses Pulmonary Hypertension, Pulmonary Veno-Occlusive Disease, Heart Failure, Pulmonary Edema, and Heart Transplant. Dr. Dia is board certified in American Board Of Internal Medicine, Cardiovascular Disease.
Advocate Heart Institute
Sunil Pauwaa is an Advanced Heart Failure and Transplant Cardiologist practicing medicine in Oak Lawn, Illinois. Dr. Pauwaa is rated as a Distinguished provider by MediFind in the treatment of Pulmonary Veno-Occlusive Disease. He is also highly rated in 9 other conditions, according to our data. His clinical expertise encompasses Heart Failure, Pulmonary Hypertension, Pulmonary Veno-Occlusive Disease, Pulmonary Edema, and Heart Transplant. Dr. Pauwaa is board certified in American Board Of Internal Medicine, Advanced Heart Failure And Transplant Cardiology.
Atrium Health Sanger Heart & Vascular Institute Kenilworth
Theodore Frank is a Cardiologist and an Advanced Heart Failure and Transplant Cardiologist practicing medicine in Charlotte, North Carolina. Dr. Frank is rated as a Distinguished provider by MediFind in the treatment of Pulmonary Veno-Occlusive Disease. He is also highly rated in 12 other conditions, according to our data. His clinical expertise encompasses Pulmonary Hypertension, Pulmonary Veno-Occlusive Disease, Restrictive Cardiomyopathy (RCM), Heart Transplant, and Endoscopy. Dr. Frank is board certified in American Board Of Internal Medicine and American Board Of Internal Medicine, Cardiovascular Disease. Dr. Frank is currently accepting new patients.
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.
Published Date: May 01, 2026
Published By: Warren Brenner, MD, Oncologist, Lynn Cancer Institute, Boca Raton, 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.
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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: Bonow RO, Mann DL, Tomaselli GF, et al, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 13th ed. Philadelphia, PA: Elsevier; 2026:chap 80.