What is the definition of 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).

What are the alternative names for Pulmonary Veno-Occlusive Disease?

Pulmonary vaso-occlusive disease

What are the causes for Pulmonary Veno-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 skin disorder)

What are the symptoms for Pulmonary Veno-Occlusive Disease?

Symptoms may include any of the following:

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

What are the current treatments for Pulmonary Veno-Occlusive Disease?

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.

What is the outlook (prognosis) for Pulmonary Veno-Occlusive Disease?

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.

What are the possible complications for Pulmonary Veno-Occlusive Disease?

Complications of PVOD may include:

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

When should I contact a medical professional for Pulmonary Veno-Occlusive Disease?

Call your provider if you have symptoms of this disorder.

REFERENCES

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.