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:
Possible risk factors for PVOD include:
Symptoms may include any of the following:
There is currently no known effective medical treatment. However, the following medicines may be helpful for some people:
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:
Call your provider if you have symptoms of this disorder.
Published Date: April 29, 2022
Published 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.
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.
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.