Heart pathologies - severe congenital disorders of the structure and work of the organ. They have different nature and symptoms, but, in any case, lead to a decrease in the quality of life of the patient.
The anomaly of Ebstein is a kind of congenital heart disease. It is rare, but treatment is necessary to avoid fatal consequences.
Summary
Ebstein's anomaly - extremely rare disease .It is equally likely in men and women. The peculiarity of this pathology is that unlike other heart defects, it gives a chance to live to adulthood.
The essence of the pathology in is the displacement from its normal septum position of the tricuspid valve .Due to this displacement, the right atrium is enlarged in size. The walls of the valve itself are deformed in different ways, depending on the type of pathology. In some cases, the walls may be partially missing. As a result of violations of the heart, blood flow problems occur.
Causes and Risk Factors
An Ebstein anomaly occurs in the heart of a person long before his birth. This is a congenital pathology, which is sometimes the cause of the death of an unborn child. The reason for its appearance is several :
- excess intake of lithium, most often in the form of drugs containing it;
- diseases of a pregnant woman: measles, scarlet fever, rubella and other infectious;
- viral diseases during pregnancy;
- use in the period of bearing a child's alcohol, drugs;
- excessive use of certain medicines that are not permitted for pregnant women.
The state of the cardiovascular system is established from 1 to 9 weeks of gestation .It is during this period that all deviations in the work and structure of the heart are formed under the influence of certain factors. The exact cause of the appearance and development of the anomaly of Ebstein has not been established to this day. However, it is proved that heredity plays an important role.
Classification of
Physicians classify 4 types of disease. I is characterized by the following features:
- the rear valve flap is not completely or significantly offset;
- septal is completely absent or displaced from a normal location;
- front is available, but large and unnaturally mobile.
II type assumes the following changes:
- front, rear and septal valve valves are most often present, but small in size and displaced above normal position;
III type differs by the presence of such problems:
- septal and posterior valve valves are displaced and undeveloped;
- front is, but has short and merging chords;
IV - features:
- the front wing is significantly deformed, shifted closer to the right ventricular tract;
- septal consists of fibrous tissue;
- posterior is undeveloped or absent altogether.
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Dangers and complications of
The main danger of the disease under consideration is the fatal outcome of .It is worth noting that this development is not uncommon in the severe course of the disease. With a more favorable flow, the patient's quality of life decreases and there is always a mortal danger.
Symptoms
Symptomatic picture directly depends on the stage of the disease, the severity of the pathology. Not always the symptoms clearly and clearly reveal the presence of the disease. Sometimes the picture is blurred, sometimes there are no symptoms at all .Among the most common:
- shortness of breath;
- fast fatigue;
- blue skin( light cyanosis);
- pain in the heart;
- complaints of constant fatigue, the inability of long physical exertion;
- unnatural swelling of the veins around the neck;
- palpitations palpable for the patient;
- abnormal changes in finger phalanx;
- swelling of the feet;
- increase in liver size.
For the doctor there are many more reasons to suspect an anomaly. In addition to what the patient can describe, the inspection reveals the presence of the "heart hump" .This feature is explained by the incredibly large size of the right atrium.
A typical picture is heart failure, which provokes blood stasis .It has been available since the birth of some patients. With a favorable scenario for the development of pathology, the symptoms manifest as late as possible.
To which doctor to contact
The cardiologist is engaged in treatment, diagnosis and study of the abnormality of Ebstein. Like other congenital malformations, an anomaly is found at an early age. Specifically, this disease is diagnosed in the first 10 days of life, in extreme cases - in the first months. However, modern methods allow to determine the presence of pathology as early as the 20th week of pregnancy with the help of ultrasound.
More about what an Ebstein anomaly is, see the video:
Diagnostics
Includes the most accurate and up-to-date methods for differentiating Ebstein's anomaly from other defects:
- ECG is the most versatile and common method. Most often, it is with this method that the first signs of an increase in the right atrium are fixed, tachycardia uncharacteristic for the normal state of atrial tremor, etc.
- Ultrasound of the heart allows you to accurately identify all the symptoms inherent in Ebstein's anomaly. If an ultrasound is performed using a doppler, the study can be more detailed. Conventional ultrasound of the heart can detect:
- increase in the volume of the right atrium;
- modification of the valve walls;
- incorrect valve location;
- time difference between the moment of closing the tricuspid valve and the two-leaf valve.
- The phonocardiography of allows tracing the pathological changes in the heart by recording the sound of its rhythm.
- Catheterization of cardiac cavities. The method allows you to measure the pressure inside the right atrium. In addition, with the help of this doctor will determine the degree of saturation of blood with oxygen. These data allow the specialist to establish the presence or absence of heart defects.
- X-ray images of the heart and lung .In connection with the wrong size and location of the heart, the lungs also deform. This can be seen in the picture, as well as atypical heart shape, characteristic for this pathology.
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Therapy methods
To make the life of patients suffering from the Ebstein abnormality maximally complete, and in some cases possible, there are modern methods.
Medicated
Refers to conservative therapy. The main purpose of this method is to reduce arrhythmia, violations of normal heart rhythm and normalization of blood circulation. Drugs suitable for these tasks:
- beta blockers;
- inotropic preparations;
- calcium channel blockers;
Diuretics are prescribed for adjuvant therapy.
Surgical intervention
If the first method is not sufficient, patients' complaints about severe, progressive pain in the heart, resort to a surgical solution to the problem.
The optimal age for this is 15 years and older .However, when the question arises of preserving a child's life, an emergency operation( Raskind procedure) is performed to prevent the child from lethal outcome from acute heart failure.
The operation to correct the Ebstein's anomaly is performed on the fully open heart of the .Its essence consists in the plastic correction of the septum and the "suturing" of the right atrium.
Forecasts and prevention measures
In the absence of treatment, the prognosis of patients is disappointing .According to statistics, patients with Ebstein's anomaly die:
- under the age of 1 year - 6.5%;
- up to 10 years -33%;
- to 38 years - up to 86%.
Surgical intervention increases the chances of a normal life of the patient and reduces the likelihood of a lethal outcome. The forecast, with a successful operation, is favorable.
Prevention for patients is not relevant, because the disease is not acquired, but congenital. The main prevention measures make sense for pregnant women:
- the right way of life;
- rejection of bad habits;
- strict adherence to the instructions for taking medications.
Thus, the anomaly of Ebstein is a pathological condition of the heart that upsets his work. Treatment includes both medication and surgery. The prognosis depends on the degree of pathology and methods of treatment.
Prevention is the observance of a pregnant woman's right lifestyle and careful medication. The main thing is to remember that it is more reasonable to prevent illness than to treat it.
