Eisenmenger's syndrome, or vascular lung disease, is a complication of congenital heart defects associated with left-right blood leakage. The course of the disease develops gradual and irreversible pulmonary hypertension, leading to rapid death in most patients. What are the causes and symptoms of Eisenmenger syndrome? How is the treatment of patients and what are their prognosis?

Eisenmenger's syndromeisvascular lung disease , the essence of which is high pulmonary vascular resistance. The disease is most often diagnosed in young children, although its symptoms may also appear in adolescents.

Eisenmenger syndrome - causes

The cause of Eisenmenger's syndrome is one of the congenital heart defects with a left-right shunt (patent ductus arteriosus of Bottal, inter-atrial shunt, ventricular shunt). As a result of the presence of a heart defect, the pulmonary and systemic circulation is combined, which may lead to a permanent increase in pulmonary vascular resistance. Then the number of pulmonary vessels decreases and their lumen becomes narrow, because they are not adapted to high pressures. The consequence is severe pulmonary arterial hypertension, as a result of which the leakage is reversed - non-oxygenated blood enters the "large" circulation, i.e. to all organs.

Eisenmenger syndrome - symptoms

The first symptoms of pulmonary hypertension are non-specific and include reduced exercise tolerance, a feeling of lack of air, especially with exercise, as well as chest pain and palpitations, and fainting.

Then, as the oxygen saturation of the arterial blood decreases, cyanosis appears, i.e. a bluish discoloration of the skin, mucous membranes, lips and nails.

A characteristic symptom of pulmonary hypertension are stick fingers (also known as Hippocrates' or drummer's stakes) - the fingers become thick and the nails take a characteristic shape (so-called watch nails - convex and rounded). Some patients develop a hyper-sticky syndrome resulting from a large number of blood cells.

Eisenmenger syndrome - diagnosis

When Eisenmenger syndrome is suspected, blood tests are performed, as well as more specialized tests such as EKGand chest X-ray, echocardiography and Doppler examinations. Cardiac catheterization, which is an invasive test, is ordered in cases where non-invasive evaluation leaves hope for surgical correction of the defect.

Eisenmenger syndrome - treatment and prognosis

Treatment is mainly based on the prevention of complications. In recent years, modern pharmacotherapy has been introduced into the treatment of all forms of pulmonary arterial hypertension, based on three groups of drugs - endothelin-1 blockers, phosphodiesterase type 5 blockers and prostanoids, which improve the parameters characterizing pulmonary hypertension.

In the event of a life-threatening situation, surgical treatment is considered, consisting in heart-lung transplantation or lung transplantation with heart defect correction. According to the experience of doctors, the one-year survival rate after this type of surgery is 70-80 percent, but 4 years after transplantation, less than 50 percent of patients are still alive.

The life expectancy of patients with Eisenmenger syndrome is significantly limited (up to around 7 years of age). Longer survival is observed in patients with Eisenmenger syndrome, which is a complication of simple leakage defects.

Important

Special recommendations for patients with Eisenmenger syndrome

  • women struggling with vascular lung disease should not get pregnant because it poses a threat to the mother's life (approx. 50% mortality), most often due to thromboembolic complications
  • patients should not stay at high altitudes and have access to oxygen therapy when traveling by plane
  • it is also recommended to limit the effort, avoid alcohol and smoking, hot baths and saunas
  • you should also prevent the development of infections

Adam Konkol has suffered from Eisenmenger syndrome since childhood

Adam Konkol was 5 years old when he was diagnosed with Eisenmenger syndrome. Doctors did not give him a chance to survive for more than 2 years. Currently, the musician is 39 years old and is one of the longest-lived people with this disease in the world.

Source: © Newseria

Category:

Cardiology