Pulmonary Veno-Occlusive Disease is a blockage of the pulmonary veins that starves the heart of oxygen. It is caused by gene mutations, mainly the EIF2AK4 gene. Other causes are viral infections, chemicals and chemotherapy drugs.
The blockage of the pulmonary veins is caused by fibrous tissue in the small veins of the lungs, obstructing blood flow. To compensate for the restricted flow, the body increases blood pressure to push the blood through more effectively. This is pulmonary hypertension.
The disease is most common in children and young adults. In addition to genetic inheritance, infections and chemotherapy, the disease may also be triggered by autoimmune disorders such as lupus.
Symptoms of Pulmonary Veno-Occlusive Disease
Symptoms of venous occlusive disease include:
- Difficulty breathing while lying flat
- Fatigue when exercising
- Coughing up blood
A doctor should investigate any of these symptoms promptly as the disease can have severe complications. Right-sided heart failure is a possible consequence. Lung function tests, echocardiograms, chest X-rays and CTs, arterial blood gases or a lung tissue biopsy may be performed to determine the diagnosis.
The disease is very rare, but it is serious and without detection and treatment, has a poor prognosis. Venous specialists can improve circulation to the lungs and heart with drug therapies.
Vasodilators – These are drugs that widen blood vessels, helping to restore normal blood flow.
Immune System Medicines – Medications to reduce the body’s inflammatory response, such as steroids, can be given.
Anti-coagulants – Medicines to thin the blood prevent clots and improve circulation.
These treatments manage symptoms and improve quality of life but can’t cure the condition. The only permanent cure is lung transplantation surgery.
Patients displaying the symptoms of pulmonary veno-occlusive disease and those who have just been diagnosed with it should seek expert medical assistance from consultants who specialize in venous diseases.
Early treatment and lung surgery offer the most positive outcome for affected people and their families.