In cardiology in general and in congenital malformations in particular, there are already several dozen “registered” diseases. Some of them are described for a long time and are successfully treated in the ante- and neonatal period, but most are still looking for their researcher. One of them is Eisenmenger's disease. The syndrome was identified at the beginning of the twentieth century by an outstanding Austrian pediatrician and now bears his name.
Definition
Eisenmenger syndrome (Eisenmenger) is a violation of the intrauterine development of the heart muscle, which includes the presence of a defect in the septum between the ventricles, the mirrored position of the aorta and an increase in the size of the right ventricle. Fortunately, this defect can be successfully operated on, and children in the future grow and develop in the same way as their peers.
Statistics
At present, about five percent of all cases of cardiac abnormalities in children are occupied by Eisenmenger syndrome. Congenital heart diseases are commonly shared with other heart diseases in newborns and young children. And some doctors combine any untreated hemodynamic disturbances into this syndrome.
Syndrome or complex
Separate the complex and Eisenmenger syndrome separately. Syndrome is a designation of uncorrected circulatory disorders with the development of blood bypass between the cavities of the ventricles of the heart. A complex includes specific disorders found in the fetus or newborn.
Etiology
The process of how this syndrome is formed is not fully known, scientists and doctors are still trying to find an explanation. However, they managed to pretty well study the factors that make up the Eisenmenger complex. The causes can be divided into two categories: internal, or genetic, and external, or the influence of the external environment.
- It is impossible to talk about direct hereditary transmission of this disease, but if relatives of the first or second line of kinship have disturbances in the formation of vital organs, then there is always a chance that the child will have them.
- Poisoning during the antenatal period. Pregnant women living in large cities, working in hazardous industries or dealing with chemical or physical pollutants fall into the risk category.
- Taking medication. Some medicines are dangerous to take while carrying a baby. Therefore, before conception or in the first weeks after it is necessary to consult with the obstetrician about all the funds taken. Even if it's just a pill for a headache.
- The use of vitamins and dietary supplements of dubious quality. Typically, such substances are harmless, but in rare cases, their intake can harm the unformed skeleton of the child or its internal organs.
- The presence of a prolonged chronic disease in one of the future parents.
Of course, these reasons do not specifically indicate Eisenmenger syndrome. Symptoms, diagnosis and treatment of it will be discussed below.
Pathogenesis
When a person has such anatomical features, there will certainly be disturbances in the process of blood circulation. Due to the presence of a significant
defect in the interventricular septum, as well as due to the incorrect position of the aorta, venous and aortic blood is mixed in the heart cavity. That is, a shunt is formed and blood discharge from left to right. Over time, the pressure in the pulmonary trunk increases, sometimes it can even exceed that in the aorta. This, in turn, leads to a reflex spasm of small vessels in the pulmonary circulation. Pulmonary hypertension develops. If it lasts a long time, then there is a compensatory thickening of the walls of the capillaries and blood stasis in the lungs is observed.
Clinicians share the “white” and “blue” Eisenmenger syndrome. In the first case, the shunt is in its classic version, that is, from left to right, and in the second - vice versa.
Symptoms
In newborns and young children, it is quite difficult to notice special symptoms. The physical and intellectual development of children does not suffer. If the reverse blood shunting appears with age, then a person has shortness of breath, blueness of the
nasolabial triangle and limbs, fatigue, palpitations or arrhythmias, pains like angina pectoris. Characteristic is the position that patients take in order to reduce symptoms - squatting. This makes it easier for them to breathe.
Of the common ailments, complaints of headaches, nosebleeds, and hemoptysis can often be heard. Perhaps a change in voice due to compression of the nerve endings by the expanded arteries. Often, one can observe such pathologies as a protrusion of the chest like a hump and a change in the terminal phalanges of the fingers in the form of drumsticks, indicating cardiopulmonary insufficiency caused by Eisenmenger syndrome. The causes, symptoms, diagnosis of it are known to every cardiologist.
The complex can be complicated by bronchitis, pneumonia, endocarditis of bacterial etiology, bleeding from the lungs and even their heart attack. Without treatment, life expectancy in sick people is not more than thirty years.
Diagnostics
Unfortunately, during an ordinary examination, it is not possible to identify this disease. In order to confirm the alleged diagnosis, special research methods will be required:
- X-ray of the chest organs, which will show visual changes in the contours of the heart and major vessels.
- Angiography as a method of visualizing vascular malformations is often used, but in this case it is not specific, and only a very experienced specialist can detect the changes.
- An ECG is indicative if it is performed under a Holter monitor. Then the information from the doctor will not be in five minutes of examination, but in a full day, and he will be able to identify interesting changes in the rhythm.
- Echocardiography is an ultrasound of the heart. An excellent method for detecting defects in the cavities of the heart and blood flow disorders.
- Cardiac catheterization in this case is the most acceptable and reliable method of research. It helps to identify all malformations, determine the characteristics of a single heart and develop a treatment.
This is such a difficult diagnosis of Eisenmenger syndrome. Symptoms (treatment of the disease is multistage and laborious) are nonspecific, the pathology disguises itself as more common diseases and eludes the attention of general practitioners.
Treatment
Despite the eternal confrontation between surgeons and therapists, in the case of this disease, they agreed that treatment should be done jointly, since conservative treatment alone does not give the desired effect, and operative treatment cannot provide a stable remission. Therefore, a set of measures was developed in order to eliminate the Eisenmenger syndrome:
- Bloodletting (in a modern way this is called "phlebotomy"). Simple and trouble-free tool. It is used in patients with proven pathology a couple of times a year. Before and after the procedure, blood viscosity and hemoglobin level are determined.
- The use of oxygen inhalation. It is used as an adjunct therapy to combat cyanosis and secondary hypoxia. In addition, they have a psychosomatic effect on patients.
- Anticoagulants. They do not have a sufficient level of evidence, since you can get out of the fire and into the fire and instead of diluting the blood, get its complete non-coagulation.
- Other medicines:
- diuretics to relieve pulmonary edema;
- drugs to improve hemodynamics. - Surgical Technique:
- pacemaker to compensate for arrhythmias;
- complete elimination of the ventricular septal defect and closure of the abnormal shunt.
How exactly to treat Eisenmenger’s syndrome, you need to decide with your doctor or even with several specialists. Since no method can guarantee success.