Pulmonary veno-occlusive disease
Pulmonary vaso-occlusive diseasePulmonary veno-occlusive disease (PVOD) is a very rare disease. It leads to high blood pressure in the lung arteries (pulmonary hypertension).
Pulmonary hypertension
Pulmonary hypertension is high blood pressure in the pulmonary arteries of the lungs. It makes the right side of the heart work harder than normal....
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.
Lupus
Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It c...
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...
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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Systemic sclerosis (autoimmune skin disorder)
Systemic sclerosis
Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. It also damages the cells that line the ...
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Symptoms
Symptoms may include any of the following:
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Shortness of breath
Shortness of breath
Breathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough air
ImageRead Article Now Book Mark Article - Dry cough
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Fatigue on exertion
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
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Fainting
Fainting
Fainting is a brief loss of consciousness due to a drop in blood flow to the brain. The episode most often lasts less than a couple of minutes and y...
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Coughing up blood
Coughing up blood
Coughing up blood is the spitting up of blood or bloody mucus from the lungs and throat (respiratory tract). Hemoptysis is the medical term for cough...
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Difficulty breathing while lying flat
Difficulty breathing while lying flat
Breathing difficulty while lying down is an abnormal condition in which a person has a problem breathing normally when lying flat. The head must be ...
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Exams and Tests
Your health care provider will examine you and ask about your medical history and symptoms.
The exam may reveal:
- Increased pressure in the neck veins
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Clubbing of the fingers
Clubbing
Clubbing is changes in the areas under and around the toenails and fingernails that occur with some disorders. The nails may also show changes....
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Bluish coloration of the skin due to lack of oxygen (cyanosis)
Bluish coloration of the skin
A bluish color to the skin or mucous membrane is usually due to a lack of oxygen in the blood. The medical term is cyanosis.
ImageRead Article Now Book Mark Article - Swelling in the legs
Your provider may hear abnormal heart sounds when listening to the chest and lungs with a stethoscope.
Heart sounds
A heart murmur is a blowing, whooshing, or rasping sound heard during a heartbeat. The sound is caused by turbulent (rough) blood flow through the h...
The following tests may be done:
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Arterial blood gases
Arterial blood gases
Blood gases are a measurement of how much oxygen and carbon dioxide are in your blood. They also determine the acidity (pH) of your blood.
ImageRead Article Now Book Mark Article - Blood oximetry
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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
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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Cardiac catheterization
Cardiac catheterization
Cardiac catheterization involves passing a thin flexible tube (catheter) into the right or left side of the heart. The catheter is most often insert...
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Lung function tests
Lung function tests
Pulmonary function tests are a group of tests that measure breathing and how well the lungs are functioning.
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Echocardiogram
Echocardiogram
An echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...
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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.
Lung transplant
Lung transplant is surgery to replace one or both diseased lungs with healthy lungs from a human donor.
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:
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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
ImageRead Article Now Book Mark ArticleSleep apnea
Obstructive sleep apnea (OSA) is a problem in which your breathing pauses during sleep. This occurs because of narrowed or blocked airways.
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- Right-sided heart failure (cor pulmonale)
Cor pulmonale
Cor pulmonale is a condition that causes the right side of the heart to fail. Long-term high blood pressure in the pulmonary arteries of the lung an...
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When to Contact a Medical Professional
Contact your provider if you have symptoms of this disorder.
References
Churg A, Wright JL. Pulmonary hypertension. In: Smith ML, Leslie KO, Wick MR, eds. Practical Pulmonary Pathology: A Diagnostic Approach. 4th ed. Philadelphia, PA: Elsevier; 2024: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.
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Respiratory system - illustration
Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.
Respiratory system
illustration
Review Date: 6/17/2024
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.
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