In cardiology in general and in congenital malformations in particular, at the moment there are already several dozen "nominal" diseases. Some of them have been 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 eminent Austrian pediatrician and now bears his name.
Definition
Eisenmenger's syndrome (Eisenmenger) is a violation of intrauterine development of the heart muscle, which includes the presence of a defect in the septum between the ventricles, 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 continue to grow and develop in the same way as their peers.
Statistics
Currently, about five percent of all cases of cardiac anomalies in children are Eisenmenger's syndrome. Congenital heart defects are usually separated from other heart diseases of newborns and young children. And some doctors combine any untreated hemodynamic disorders into thissyndrome.
Syndrome or complex
The complex and Eisenmenger's syndrome are distinguished separately. The syndrome is a designation of uncorrected circulatory disorders with the development of blood shunting between the cavities of the ventricles of the heart. And the complex includes specific disorders found in the fetus or newborn.
Etiology
The process of how this syndrome is formed is not completely known, scientists and doctors are still trying to find an explanation. However, they managed to study quite well the factors due to which the Eisenmenger complex appears. Causes can be divided into two categories: internal, or genetic, and external, or environmental influences.
- It is impossible to talk about the direct hereditary transmission of this disease, but if relatives of the first or second line of kinship have violations in the formation of vital organs, then there is always a chance that they will occur in the child.
- Poisoning during the antenatal period. The risk category includes pregnant women living in large cities, working in hazardous industries or dealing with chemical or physical pollutants.
- Medication taking. Some medicines are dangerous to take while carrying a child. Therefore, before conception or in the first weeks after, it is necessary to consult with an obstetrician about all the means taken. Even if it's just headache pills.
- Use of vitamins and dietary supplementsof dubious quality. Usually such substances are harmless, but in rare cases, taking them can harm the child's unformed skeleton or internal organs.
- One of the future parents has a long-term chronic illness.
Of course, the listed causes do not specifically point to Eisenmenger's syndrome. Symptoms, diagnosis and treatment 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 of the interventricular septum, as well as due to the incorrect position of the aorta, mixing of venous and aortic blood occurs in the cavity of the heart. That is, a shunt is formed and blood is discharged 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 for a long time, then there is a compensatory thickening of the walls of the capillaries and there is a stasis of blood in the lungs.
Clinicians separate "white" and "blue" Eisenmenger syndrome. In the first case, the shunt goes 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. Physical andintellectual development of children does not suffer. If reverse blood shunting appears with age, then a person has shortness of breath, blueness of the nasolabial triangle and extremities, fatigue, a noticeable heartbeat or arrhythmia, and angina-type pain. Characteristic is the position that patients take to reduce symptoms - squatting. This makes it easier for them to breathe.
From general ailments one can often hear complaints of headaches, nosebleeds and hemoptysis. A change in voice is possible due to compression of nerve endings by dilated arteries. Often one can observe pathologies such as protrusion of the chest in the form of a hump and a change in the terminal phalanges of the fingers in the form of drumsticks, indicating cardiopulmonary insufficiency caused by Eisenmenger's 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 no more than thirty years.
Diagnosis
Unfortunately, with 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, which will show visual changes in the contours of the heart and great vessels.
- Angiography howvisualization of vascular malformations is often used, but in this case it is not specific, and only a very experienced specialist can detect changes.
- ECG is indicative if it is performed under a Holter monitor. Then the doctor will have information not for five minutes of examination, but for a full day, and he will be able to identify changes in the rhythm of interest.
- EchoCG is an ultrasound of the heart. An excellent method for detecting defects in the cavities of the heart and blood flow disorders.
- Catherization of the heart 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 Eisenmenger's syndrome to diagnose. Symptoms (treatment of the disease is multi-stage and labor-intensive) are non-specific, the pathology disguises itself as more common diseases and escapes the attention of general practitioners.
Treatment
Despite the eternal confrontation between surgeons and therapists, in the case of this disease, they agreed that it is necessary to treat with joint efforts, since conservative treatment in itself does not give the desired effect, and surgical treatment cannot provide stable remission. Therefore, a set of measures was developed in order to eliminate the Eisenmenger syndrome:
- Bleeding (in a modern way it is called "phlebotomy"). A simple and trouble free tool. It is used in patients with proven pathology a couple of times a year. before andafter the procedure, blood viscosity and hemoglobin levels are necessarily determined.
- Use of oxygen inhalation. Used as adjuvant 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 frying pan and instead of thinning the blood, get its complete incoagulability.
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Other medicines:
- diuretics to relieve pulmonary edema;- drugs to improve hemodynamics.
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Surgical technique:
- pacemaker to compensate for arrhythmia;- 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.
