This pathology as an Eisenmenger disease( complex, syndrome) occurs in the fetus during the perinatal period. It becomes dangerous if no correction is found and corrected in time.
If at an early age the disease is not identified, then if her signs appear at an older age, you need to consult a specialist as quickly as possible.
So, let's figure out what is included in the concept of Eisenmenger syndrome.
Features of the disease
The disease, caused by congenital anomalies in the heart, was named after a scientist who discovered and described the disease - Eisenmenger syndrome. Pathology refers to a variety of heart disease.
The syndrome has a set of congenital abnormalities:
- Aortic displacement from normal position. Its dislocation is called a "rider".Abnormal location allows to enter the aorta of blood from both ventricles.
- The septum between the ventricles has a defect, which makes it possible to communicate blood venous and arterial, which is not provided in a healthy heart. The septum can be absent altogether. Such a situation disturbs the correct ratio in the level of pressure created in different halves of the heart. In the right atrium, the pressure is increased, which affects the blood flow of the small circle badly, and there are difficulties for getting blood there.
- The right ventricle is hypertrophied, it usually increases as a result of the previous( first two) pathologies.
Deviations coincide with the signs of the tetralo of Fallot. Difference - there is no narrowing of the aorta.
Forms and classification
The disease is divided into two periods.
Stage of pallus type
Pathological abnormalities in the anatomical structure, characteristic of the Eisenmenger syndrome, result in the arterial blood from the left ventricle entering the right( venous) ventricle. This anomaly does not cause a change in the color of the skin to a bluish tone.
The deformation of the right ventricle occurs as a consequence of these disorders, an expansion of its internal volume. On the increased pressure in the right atrium, the vessels in the lungs react, strengthening the resistance with time. The prerequisites for pulmonary hypertension are created.
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The following video is about what pulmonary hypertension is:
The stage of the blue type
As a result of the situation described above, the direction of the blood flow between the ventricles through the defect in the septum to the right-left changes. Dilation of arterial blood venous and ingestion of oxygen depleted in blood into the large circle of blood circulation causes oxygen starvation.
Cyanosis appears at this stage. The intensity of the blue hue of the skin depends on the degree of the problem. Over time, complications associated with hypoxia.
- Blood acquires increased viscosity.
- Compensatory mechanisms include increased erythrocyte content. Their increased disintegration creates the prerequisites for the emergence of new diseases:
- gout,
- cholelithiasis
- and others.
Causes of
Pathology is inherent in nature. The behavior and health of the mother during childbearing has a direct impact on his future health.
Negative factors:
- effects on fetal life:
- electromagnetic radiation,
- radiation,
- vibration,
- chemicals, including drugs;
- bacteria and viruses that can cause abnormal development;
- carcinogens;
- chromosomal abnormalities that cause abnormal organ development.
Next, we'll talk about the symptoms of Eisenmenger's disease.
Symptoms of
The disease may not manifest itself for a while. Deviations cause undesirable processes, as a result of which symptoms of the disease appear over time.
It:
- a breakdown, a bad state of health during exercise;
- when loaded, the skin becomes bluish,
- pains in the left side of the chest, which can give to the shoulder blade, hand;
- possible loss of consciousness,
- shortness of breath,
- sometimes the appearance of a cough with bloody sputum.
Diagnostics of
There are some indications that a specialist on examination can assume the presence of Eisenmenger's symptom:
- cyanosis of the skin,
- change in the shape of the fingertips - "drum sticks" and nails - "watch glasses",
- listening to a certain kind of noise.
If there are signs of a disease or if this type of defect was detected at the time of birth and timely correction is not made, then the patient should be urgently examined in order to correctly prescribe the treatment.
To investigate the problem, the following methods are used:
- Angiography - provides comprehensive information. It is carried out if there are no contraindications.
- Electrocardiography is a necessary procedure for determining rhythms uncharacteristic for normal heart function. Perhaps this method is to observe the heart around the clock.
- Catheterization - through the channel of blood vessels in the heart falls catheter. With its help, you can conduct an examination and learn all the features of the pathology.
- Echocardiography is a safe method of ultrasound examination. Details of the internal structure and abnormalities. It shows the parameters of hemodynamics.
- Radiography - the procedure involves taking a picture of the chest. On it you can see the change in the contours of the heart, the increase in its size.
The following video will give a more detailed picture of what the Eisenmenger symptom is and what diagnostic method to choose:
Treatment
Properly organized care for this pathology is the correction of abnormal places at an early age by surgical means. If the disease has found itself at a later age, then correction should be done as quickly as possible.
In adulthood, as a result of complications, an irreversible situation may occur, which requires extremely radical solutions. If the patient is not ready for this, then it is possible to maintain the condition with therapeutic and medicamental methods in order to prolong the patient's life and how to improve its quality.
Therapeutic way of the
The patient receives recommendations on how to maintain the condition to avoid deterioration. Such patients are not shown:
- is in high altitude areas,
- is taking nonsteroidal medications,
- dipping into cold water,
- dehydration,
- overheating in bath,
- ache for acute respiratory disease,
- pregnancy.
Medicated
If the syndrome has developed enough and complications have developed, the patient is supported by drugs, depending on the problems that have arisen. Select the means for the patient to reduce the hypertension of the lungs:
- preparations that enhance nitric oxide;
- endothelin antagonists,
- prostacyclin antagonists.
Others
Early correction of pathological abnormalities in the structure of the heart provides for correcting the position of the aorta and the defect of the septum. To achieve the goal, closed( endovascular) and open surgical interventions are used. These actions can prevent the onset of a severe stage of the disease and make the patient's life complete.
In case the disease is started and the complications have turned the process to the irreversible side, the operation can be saved by a transplantation simultaneously of the heart and lungs. One of the variants of surgical intervention is the correction of the septum in the heart and the transplantation of the lung.
Sometimes, in order to maintain the patient's condition in an irreversible situation, highly qualified specialists undertake some adjustment through the operation. It is also possible to assign an electrocardiostimulator to the installation.
Prevention of disease
Women need to plan pregnancy, consult with geneticists and check their health in advance.
Pregnant should be observed:
- avoid contact with harmful chemicals,
- if it is necessary to take pills, consult a doctor;
- not to be in places with dangerous ecology,
- not to be exposed to ionizing radiation,
- not to consume genetic products,
- to observe a useful diet.
Complications of
Congenital disorders must be corrected in time by surgery. Disease, developing, creates ever deeper problems.
To the main symptoms are added:
- headaches,
- curvature of the spine,
- frequent respiratory diseases,
- lung infarction,
- pulmonary hemorrhage,
- thoracic deformation,
- diseases associated with the formation of thrombi;
- heart failure.
Forecast
In each case, the life expectancy will depend on the factors:
- severity of congenital anomalies,
- whether an early adjustment is made,
- how the situation is started.
Patients with this problem live 20 รท 50 years. The quality of life can be unsatisfactory, because you have to limit physical activity. If the patient does not handle this diagnosis for help to specialists, the life span is limited to 20 to 30 years.