The obvious progress in medicine over the past few years has not led to a sharp increase in life expectancy or the elimination of cardiac pathologies. 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 blurry, and treatment is very difficult. Eisenmenger syndrome, named after the Austrian pediatrician and cardiologist, cannot be considered common. A rare pathology received a detailed description about 100 years ago. However, even today one does not have to talk about a clear understanding of its etiology.
general information
Eisenmenger's syndrome (sometimes called a complex) is a very dangerous pathology that spreads to the pulmonary and cardiac systems. Cardiac disease is characterized by a combined defect in the interventricular septum and hypertension. The development of pathology leads to increased pressure and disruptions in the pulmonary circulation. As a result, a shunt is formed, which is responsible for the injection of blood from right to left, which violates the normal hemodynamic processes.
Also, the concept of Eisenmenger syndrome includes any heart disease, characterized by an open atrioventricular canal and ductus arteriosus, the presence of only one ventricle. Among patients with abnormalities 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. Disorders at the genetic level can be transmitted from parents to the child, thereby causing an increased likelihood of developing 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.
These factors are not typical specifically for Eisenmenger syndrome, but theoretically they can cause various pathologies, including this one.
How does the disease manifest?
Patients with Eisenmenger syndrome usually do not complain about health problems. Therefore, early diagnosis is not always possible. The child's body first connects compensation mechanisms, but after a while the disease manifests itself. Internal resources are becoming thinner, the cardiac system is malfunctioning. What are the symptoms of Eisenmenger syndrome?
- Increased weakness, worse after physical exertion.
- Pain in the left side of the chest.
- The growing cyanosis of the skin in the daytime.
- Spontaneous fainting.
- Coughing fits with spotting.
Such symptoms do not bother everyone. The syndrome for a long time can develop imperceptibly. If the pediatrician who observes the child recommends an additional examination at a cardiology center, they should not be neglected.
Pregnancy Management
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 colleagues think differently. In their opinion, successful delivery depends on compliance with a number of rules and recommendations.
First of all, a woman is shown hospitalization for the entire period of pregnancy. At 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, coagulant treatment is prescribed. A few days before the expected date of the baby's appearance, glycoside shock therapy is recommended to maintain the cardiac 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, when deciding whether to maintain a pregnancy, a woman should think about potential risks.
Medical examination
Effective therapy for patients with Eisenmenger syndrome cannot be imagined without a special examination. You can go through it 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 (vascular examination);
- ECG (detection of latent 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 for all patients with suspected Eisenmenger syndrome. Pentall Fallot, stenosis of arteries, obstruction of the ductus arteriosus - 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.
Phlebotomy is prescribed for patients with a pronounced clinical picture and increased hematocrit. The procedure is repeated no more than three times a year, while it is imperative to monitor 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. Also, patients are prescribed medications. Epoprostenol and Treprostinil improve hemodynamics, while Traklir reduces vascular resistance.
Surgical intervention is carried out in two versions: implantation of a pacemaker, plastic defect MPP. With Eisenmenger syndrome, surgery can significantly improve the prognosis of the disease. If the listed methods of treatment are ineffective, only a simultaneous lung and heart transplant can help. Transplantation operation is extremely complex, requires large financial costs. On the other hand, it does not eliminate the risk of complications. When there is no other solution, the material question does not cause difficulties; a double transplant can save a person’s life. Before carrying out it is necessary to undergo a complex medical examination.
How do patients with Eisenmenger syndrome live?
People who have had to deal with this cardiological 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 exposure, and infectious diseases. It is important to abandon addictions and taking medications that can trigger bleeding. If the patient follows the doctor's instructions, the likelihood of being able to lead a familiar lifestyle increases. Otherwise, the level of oxygen in the blood can drop to critical levels, which will lead to death.