Eisenmenger syndrome: symptoms. Eisenmenger syndrome and pregnancy. Patients with Eisenmenger syndrome

The obvious progress in medicine over the past few years has not led to a dramatic increase in life expectancy or elimination of cardiac pathologies. In addition, at the turn of the XIX-XX centuries, scientists described a number of new diseases. The causes of some of them still remain a mystery, the symptoms are blurred, and treatment is very difficult. The syndrome of Eisenmenger, named after the name of the Austrian pediatrician and cardiologist, can not be considered widespread. A rare pathology was detailed in about 100 years ago. However, even today we do not have to speak about a clear understanding of its etiology.

general information

The syndrome (sometimes called the complex) of Eisenmenger is a very dangerous pathology that spreads to the pulmonary and cardiac systems. Cardiac disease is characterized by a combined defect of the interventricular septum and hypertension. The development of pathology leads to increased pressure and malfunction in the small circle of the circulation. As a result, a shunt is formed, which is responsible for injecting blood from right to left, which disrupts the normal processes of hemodynamics.

Also, the concept of Eisenmenger syndrome includes any heart defects that are distinguished by an open atrioventricular canal and arterial duct, the presence of only one ventricle. Among patients with anomalies of the interventricular septum, pathology is diagnosed in 10% of cases. In the structure of congenital defects of the main muscle of the body, the Eisenmenger complex is 3%.

Causes of the syndrome

A single cause that determines the development of pathology, doctors can not be called. However, there are a number of etiological factors that increase the likelihood of the birth of children with Eisenmenger syndrome:

1. Hereditary predisposition. Violations at the gene level can be transmitted from parents to the child, thereby causing an increased likelihood of developing heart defects.
2. Environmental factors:

• Intoxication of the fetus during intrauterine development;
• Long-term exposure to electromagnetic radiation;
• Chronic infectious diseases of one of the parents;
• Side effects of drugs and dietary supplements used by a woman during pregnancy.

These factors are not typical for Eisenmenger syndrome, but in theory they can cause various pathologies, including this one.

How does the disease manifest itself?

Patients with Eisenmenger syndrome usually do not complain about health problems. Therefore, early diagnosis is not always possible. The child's organism first connects the compensatory mechanisms, but after a while the disease manifests itself. Internal resources become thinner, the heart system fails. What are the symptoms of Eisenmenger syndrome?

1. Increased weakness, aggravated after physical exertion.
2. Pain in the left side of the chest.
3. Crawling in the daytime, cyanosis of the skin.
4. Spontaneous fainting.
5. Coughing attacks with spotting.

Such symptoms do not bother everyone. Syndrome for a long time can develop unnoticed. If a pediatrician who watches a child recommends an additional examination in a cardiac center, it should not be neglected.

Management of pregnancy in case of illness

Russian doctors are convinced that Eisenmenger's syndrome and pregnancy are incompatible. Therefore, at any time a woman is recommended to have an abortion. Their Western colleagues think differently. In their opinion, safe labor depends on the observance of a number of rules and recommendations.

First of all, a woman is shown hospitalization for the entire duration of pregnancy. In the hospital, doctors should constantly monitor the fetal condition and measure the pressure in the pulmonary capillaries. At the first sign of shortness of breath - oxygen masks. Starting with the second trimester, treatment with coagulants is prescribed. A few days before the expected date of child's appearance, shock therapy with glycosides is recommended to maintain the heart system. Delivery is possible only in a natural way.

If a woman follows all the prescriptions of doctors, the probability of a positive outcome for a child is 50-90%. The prognosis for the mother is not so favorable. That is why, in deciding whether to continue pregnancy, a woman should think about the potential risks.

Medical Examination

Effective therapy of patients with Eisenmenger syndrome is impossible to imagine without a special examination. It can be done only in specialized clinics with the appropriate equipment. If this pathology is suspected, the screening program usually includes:

• Chest X-ray of the chest (assessment of changes in pulmonary artery and heart contours);
• Angiography (study of blood vessels);
• ECG (detection of hidden cardiac arrhythmias);
• Echocardiography (visualization of pathological changes in the walls of the ventricles);
• Cardiac catheterization (assessment of the course of the disease and the nature of damage, measurement of the numerical parameters of the main muscle).

Differential diagnosis is mandatory in all patients with suspected Eisenmenger syndrome. Pentado Pento, stenosis of the arteries, nonclosure of the arterial duct - this is a short list of pathologies for exclusion.

Principles of therapy

Conservative treatment of patients is possible, but not effective enough. On the other hand, the use of surgical intervention does not always give positive results. Therefore, today doctors are increasingly resorting to a combined strategy.

Patients with a pronounced clinical picture and enlarged hematocrit are assigned phlebotomy. The procedure is repeated no more than three times a year, while it is necessary to monitor blood iron parameters. Oxygen therapy and anticoagulant treatment are used extremely rarely, rather, optional. The main disadvantage of such procedures is a large number of side effects, the most significant of which is hemorrhagic diathesis. Patients are also prescribed medications. "Epoprostenol" and "Treprostinil" improve hemodynamics, and "Trakliir" reduces the resistance of blood vessels.

Surgical intervention is carried out in two versions: implantation of a pacemaker, plastic defect MPP. With Eisenmenger's syndrome, surgery can significantly improve the prognosis of the disease. If these methods of treatment are ineffective, only a simultaneous transplant of the lungs and heart can help. The transplantation operation is extremely complicated, it requires large financial costs. On the other hand, it does not relieve the risk of complications. When there is no other solution, the material issue does not cause difficulties, a double transplantation can save a person's life. Before its carrying out it is necessary to pass difficult medical inspection.

How do patients with Eisenmenger syndrome live?

People who have had to face this cardiac disease often lead an active life. However, they are forced to constantly visit a doctor and monitor health indicators. Such patients should avoid dehydration, prolonged stay at height, infectious diseases. It is important to give up the harmful habits and taking medications that can provoke bleeding. If the patient follows the prescriptions of the doctor, the likelihood of the opportunity to lead a habitual way of life increases. Otherwise, the oxygen level in the blood can drop to critical levels, which will lead to death.