Pulmonary venoocclusive disease
Overview
Pulmonary venoocclusive disease is an extremely rare form of high blood pressure in the lung area.
(aka Pulmonary veno-occlusive disease, Venoocclusive disease of lung, Pulmonary vaso-occlusive disease)
Source: Medline Plus
Symptoms
• Shortness of breath
• Fatigue on exertion
• Fainting
• Coughing up blood
• Difficulty breathing while lying flat
Source: Medline Plus
Causes
In most cases, the cause of pulmonary venoocclusive disease is unknown. The condition may be related to a viral infection. It may occur as a complication of certain diseases such as lupus, or as a complication of leukemia, lymphoma, chemotherapy, 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, and congestion and swelling of the lungs.
Source: Medline Plus
Diagnosis
The doctor will perform a physical exam.
The exam may reveal:
• High blood pressure in the veins of the lungs
• Increased pressure in the neck veins
• Fingernail clubbing
• Bluish coloration of the skin due to lack of oxygen (cyanosis)
• Swelling in the legs
Your doctor may hear abnormal heart sounds when listening to the chest and lungs with a stethoscope.
The following tests may be done:
• Arterial blood gases
• Chest x-ray
• Chest CT
• Cardiac catheterization
• Pulmonary angiogram
• Echocardiogram
• Lung biopsy
Source: Medline Plus
Prognosis
The outcome is often very poor in infants with a survival rate of just a few weeks. Survival may be months to a few years in adults.
Source: Medline Plus
Treatment
There is currently no known effective medical treatment. However, the following medications may be helpful for some patients:
• Vasodilator drugs (drugs that widen the blood vessels)
• Drugs that control immune system's response (such as azathioprine or steroids)
A lung transplant may be needed.
Possible Complications
• Progressive difficulty breathing
• Pulmonary hypertension
• Right sided heart failure (cor pulmonale)
• Coughing up blood
Source: Medline Plus