Eisenmenger syndrome: causes, symptoms, diagnosis, treatment

In cardiology in general and in congenital malformations in particular at the moment there are already several dozen "registered" diseases. Some of them have been described for a long time and are successfully treated in the ante- and neonatal period, but the majority are still looking for their researcher. One of them is Eisenmenger's disease. The syndrome was discovered early in the twentieth century by an outstanding Austrian pediatrician and now bears his name.

Definition

Eisenmenger syndrome is a violation of the intrauterine development of the heart muscle, which includes the presence of a defect in the ventricular septum, the mirrored position of the aorta and the increase in the size of the right ventricle. Fortunately, this vice can be successfully operated on, and children in the future grow and develop in the same way as their peers.

Statistics

At the moment about five percent of all cases of cardiac anomalies in children is Eisenmenger syndrome. Congenital heart diseases are commonly shared with other heart diseases of newborns and young children. And some doctors unite any untreated hemodynamic disorders in this syndrome.

Syndrome or complex

Separate complex and Eisenmenger syndrome. The syndrome is a designation of uncorrected circulatory disturbances with the development of the shunting of blood between the cavities of the ventricles of the heart. And the complex includes specific violations 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 quite well study the factors that make the Eisenmenger complex appear. The reasons can be divided into two categories: internal, or genetic, and external, or the influence of the external environment.

1. Talk about a direct hereditary transmission of this disease is impossible, but if relatives of the first or second line of kinship have violations of the formation of vital organs, then there is always the likelihood that they will have a child.
2. Poisoning during the antenatal period. The risk category includes pregnant women living in large cities, working in hazardous production or dealing with chemical or physical pollutants.
3. Reception of medicines. Some drugs are dangerous to take during the bearing of a child. 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 from a headache.
4. The use of vitamins and dietary supplements of doubtful quality. Usually such substances are harmless, but in rare cases, their intake can harm the unformed skeleton of the child or his internal organs.
5. The presence of a long-term chronic disease in one of the future parents.

Of course, these reasons do not specifically point to Eisenmenger's syndrome. Symptoms, diagnosis and treatment of it will be discussed below.

Pathogenesis

When a person has such anatomical features, there will necessarily be irregularities in the blood circulation process. Due to the presence of a significant defect of the interventricular septum, and also because of the incorrect position of the aorta, there is mixing of venous and aortic blood in the heart cavity. That is, a shunt and a discharge of blood are formed from left to right. Over time, the pressure in the pulmonary trunk increases, sometimes it may even exceed that in the aorta. This, in turn, leads to a reflex spasm of small vessels in a small circle of blood circulation. Develops pulmonary hypertension. If it lasts a long time, then compensatory thickening of the capillary walls occurs and a stasis of blood in the lungs is observed.

Clinicians share the "white" and "blue" Eisenmenger syndrome. In the first case, the shunt goes in its classical version, that is, from left to right, and in the second - on the contrary.

Symptoms

In newborns and young children, it is rather difficult to notice the special symptomatology. The physical and intellectual development of children does not suffer. If the reverse shunting of blood occurs with age, the person has shortness of breath, blue nose and nasolabial triangle and limbs, fast fatigue, palpitations or arrhythmias, pains of the type of angina pectoris. Characteristic is the position that patients take to reduce symptoms - squatting. So it becomes easier for them to breathe.

Of common ailments, you can often hear complaints of headaches, nosebleeds and hemoptysis. It is possible to change the voice due to the clamping of the nerve endings by the enlarged arteries. Often it is possible to observe such pathologies as bulging out the chest by the type of hump and changing the end phalanges of the fingers in the form of tympanic sticks, indicating the cardiopulmonary insufficiency, which caused Eisenmenger syndrome. Causes, symptoms, diagnosis are known to every cardiologist.

Complex can be complicated by bronchitis, pneumonia, endocarditis of bacterial etiology, bleeding from the lungs and even their infarction. Without treatment, life expectancy in sick people is no more than thirty years.

Diagnostics

Unfortunately, with an ordinary examination, it is not possible to detect this disease. In order to confirm the expected diagnosis, special research methods will be required:

1. X-ray of chest organs, which will show visual changes in the contours of the heart and the main vessels.
2. Angiography as a method of visualization of vascular malformations is often used, but in this case it is not specific, and only a very experienced specialist can reveal changes.
3. The ECG is indicative only if it is performed under a Holter monitor. Then the information from the doctor will not be for five minutes of the examination, but for a full day, and he will be able to identify the interesting changes in rhythm.
4. Echocardiography is an ultrasound of the heart. An excellent method for detecting defects in the heart cavities and blood flow disorders.
5. Cardiac catheterization in this case is the most acceptable and reliable method of investigation. It helps to identify all the malformations, determine the features of a single heart and develop a treatment.

Here is such an uneasy Eisenmenger syndrome in diagnosis. Symptoms (treatment of the disease is multistage and time-consuming) are not specific, the pathology is masked for 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 proper effect, and the operative can not provide a stable remission. Therefore, a set of measures was developed in order to eliminate the Eisenmenger syndrome:

1. Bleeding (in modern terms this is called "phlebotomy"). Simple and trouble-free remedy. It is used in patients with proven pathology a couple of times a year. Before and after the procedure, the blood viscosity and hemoglobin level must be determined.
2. Use of inhalation of oxygen. Used as an auxiliary therapy for the control of cyanosis and secondary hypoxia. In addition, they have a psychosomatic effect on patients.
3. Anticoagulants. They do not have a sufficient level of evidence, since one can get from the fire into the fire and instead of diluting the blood, get its full unfolding.
4. Other medicines:
   - diuretics for removal of pulmonary edema;
   - drugs to improve hemodynamics.
5. Surgical technique:
   - pacemaker to compensate for arrhythmia;
   - complete elimination of the defect of the interventricular septum and closure of the abnormal shunt.

How to treat Eisenmenger syndrome, it is necessary to decide with your doctor or even a few specialists. Since no method can guarantee success.