Obvious advances in medicine over the past few years have not led to a dramatic increase in life expectancy or the elimination of heart disease. In addition, at the turn of the XIX-XX centuries, scientists described many new diseases. The causes of some of them are still a mystery, the symptoms are blurred, and treatment is very difficult. Eisenmenger's syndrome, named after an Austrian pediatrician and cardiologist, cannot be considered common. A rare pathology received a detailed description about 100 years ago. However, even today there is no clear understanding of its etiology.
General information
Eisenmenger's syndrome (sometimes called the complex) is a very dangerous pathology that affects the pulmonary and cardiac systems. Cardiac disease is characterized by a combined ventricular septal defect and hypertension. The development of pathology leads to an increase in pressure and failures in the pulmonary circulation. As a result, a shunt is formed, which is responsible for throwing blood from right to left, disrupting normal hemodynamic processes.
Also, the concept of Eisenmenger's syndrome includes any heart disease characterized 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
Doctors cannot name a single reason for the development of pathology. However, there are a number of etiological factors that increase the likelihood of having children with Eisenmenger syndrome:
- Hereditary predisposition. Genetic disorders can be passed from parent to child, making them more likely to develop heart defects.
- Environmental factors:
- fetal intoxication during fetal development;
- prolonged 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.
The listed factors are not typical for Eisenmenger's syndrome, but theoretically they can cause various pathologies, including this one.
How does the disease manifest itself?
Patients with Eisenmenger's syndrome usually do not complain of he alth problems. Therefore, early diagnosis is not always possible. The child's body first connectscompensation mechanisms, but after a while the disease manifests itself. Internal resources become thinner, the cardiac system fails. What are the symptoms of Eisenmenger's syndrome?
- Increased weakness, worse after exercise.
- Pain in the left side of the chest.
- Increasing daytime cyanosis of the skin.
- Spontaneous fainting.
- Bouts of coughing up bloody discharge.
Such symptoms do not bother everyone. The syndrome can develop unnoticed for a long time. If the pediatrician who observes the child recommends an additional examination at the cardiology center, you should not neglect it.
Management of pregnancy in illness
Russian doctors are sure that Eisenmenger's syndrome and pregnancy are incompatible. Therefore, at any time a woman is recommended to have an abortion. Their Western counterparts think differently. In their opinion, a safe birth depends on following a number of rules and recommendations.
First of all, a woman is shown hospitalization for the entire duration of pregnancy. In the hospital, doctors must constantly monitor the condition of the fetus and measure the pressure in the pulmonary capillaries. At the first sign of shortness of breath - oxygen masks. Starting from the second trimester, treatment with coagulants is prescribed. A few days before the expected date of birth, shock therapy with glycosides is recommended to maintain the cardiac system. Delivery is only possible naturally.
If a womanfollows all the prescriptions of doctors, the probability of a positive outcome for the child is 50-90%. The prognosis for the mother is not so favorable. That is why, when deciding whether to continue a pregnancy, a woman should think about the potential risks.
Medical examination
Effective therapy for patients with Eisenmenger's syndrome cannot be imagined without a special examination. It can be passed only in specialized clinics with the appropriate equipment. If this pathology is suspected, the examination program usually includes:
- chest x-ray (assessment of changes in the contours of the pulmonary artery and heart);
- angiography (examination of blood vessels);
- ECG (detection of latent cardiac arrhythmias);
- EchoCG (visualization of pathological changes in the walls of the ventricles);
- heart catheterization (assessment of the course of the disease and the nature of damage, measurement of the numerical parameters of the work of the main muscle).
Differential diagnosis is mandatory for all patients with suspected Eisenmenger's syndrome. Pentade of Fallot, arterial stenosis, patent ductus arteriosus is a short list of pathologies to exclude.
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 tocombined strategy.
Patients with a pronounced clinical picture and increased hematocrit are prescribed phlebotomy. The procedure is repeated no more than three times a year, while it is necessary to control blood iron levels. Oxygen therapy and anticoagulant treatment are used extremely rarely, rather, optionally. 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 "Traklir" reduces vascular resistance.
Surgical intervention is carried out in two versions: implantation of a pacemaker, plastic surgery of the MPD defect. With Eisenmenger's syndrome, surgery can significantly improve the prognosis of the disease. If the above methods of treatment are ineffective, only a simultaneous transplantation of the lungs and heart can help. The transplant operation is extremely complex and requires large financial costs. On the other hand, it does not eliminate the risk of complications. When there is no other solution, the material issue does not cause difficulties, a double transplant can save a person's life. Before it is carried out, it is necessary to undergo a complex medical examination.
How do patients with Eisenmenger's syndrome live?
People who have had to deal with this cardiac disease often lead active lives. However, they are forced to constantly visit a doctor andmonitor he alth indicators. Such patients should avoid dehydration, prolonged exposure to altitude, and infectious diseases. It is important to give up addictions and medications that can cause bleeding. If the patient follows the doctor's instructions, the likelihood of being able to lead a normal life increases. Otherwise, the level of oxygen in the blood may drop to critical levels, which will lead to death.
