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).
Causes
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.
Bone marrow transplantation
A bone marrow transplant is a procedure to replace damaged or diseased bone marrow with healthy bone marrow stem cells. Bone marrow is the soft, fatt...
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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
Chemotherapy
The term chemotherapy is used to describe cancer-killing drugs. Chemotherapy may be used to:Cure the cancerShrink the cancerPrevent the cancer from ...
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Symptoms
Symptoms may include any of the following:
- Shortness of breath
Shortness of breath
Breathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough air
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- Fatigue on exertion
- Fainting
- Coughing up blood
Coughing up blood
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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 x-ray
A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.
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Chest CT
A chest CT (computed tomography) scan is an imaging method that uses x-rays to create cross-sectional pictures of the chest and upper abdomen....
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- Lung function tests
- Echocardiogram
- Lung biopsy
Treatment
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.
Outlook (Prognosis)
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.
Possible Complications
Complications of PVOD may include:
- Difficulty breathing that gets worse, including at night (sleep apnea)
Difficulty breathing
Breathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough air
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- Right-sided heart failure (cor pulmonale)
When to Contact a Medical Professional
Call your provider if you have symptoms of this disorder.
Reviewed 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.