Right ventricular arrhythmogenic dysplasia is very rarely diagnosed. Pathology is different in that the process of replacing cardiomyocytes with a common connective or fat tissue is started, which immediately affects the functions of the heart. In the most neglected cases, this process extends to the left ventricle, worsening the patient's condition and greatly increasing the risk of sudden death.
Features of the disease
An interesting feature is noted, according to which people with frizzy hair are predisposed to the development of pathology, as well as those who have diffuse keratodermia of the feet and palms. Arrhythmogenic dysplasia is most affected by young people and predominantly of the weaker sex.
Right ventricular arrhythmogenic dysplasia( ECG results)
Classification and forms of
Arrhythmogenic dysplasia is divided into the following forms:
- Fatty. The most important cells of the heart( cardiomyocytes) almost completely begin to be replaced by adipose tissue, and the ventricle wall does not change the thickness.
- Fibrous fat. This form is characterized by thinning of the walls of the ventricle. There is a substitution of cardiomyocytes not only with fatty tissue, but also with fibrous tissue( that is, the usual connective tissue).This type of disease can affect not only the right ventricle, but also the myocardium of the left.
The classification also includes 4 additional types of arrhythmogenic cardiomyopathy:
- Hidden. The only first symptom is sudden death.
- Dysplasia with poorly manifested symptoms. They are usually common - pain, palpitations.
- Arrhythmic, which is characterized by an impressive number of cardiac problems, including extrasystole and tachycardia.
- Right ventricular heart failure.
Causes of
Allocate idiopathic causes of the disease, that is, the genesis of which remains unknown. Genetic predisposition in patients is detected in 50% of cases.
Earlier it was thought that the main cause of it is myocarditis, but now it is established that there is no connection between these pathologies.
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Symptoms of arrhythmogenic right ventricular dysplasia
Already in adolescence, it is possible to identify a number of characteristic features:
- poor load tolerance, fast fatigue;
- fainting and blurred vision;
- a number of disorders of the heart( "fluttering, fast heartbeat, etc.);
- circulatory arrest.
Sometimes dysplasia is asymptomatic.
Diagnosis
Initially, an anamnesis of family illnesses and the patient's life is collected in order to reveal a predisposition to right ventricular dysplasia. After the examination, the doctor sends him for the delivery of laboratory tests. Further, a variety of studies are used to establish the disease and its features:
- Radiocontrast ventriculography. It allows to reveal the extensions of the ventricle, the incorrect development of its tissues, as well as violations of the contractile function.
- ECG.Determine all the characteristic signs of arrhythmogenic right ventricular dysplasia. Also, concomitant diseases - extrasystole, tachycardia - can be identified.
- MRI.Helps visualize and accurately represent the spread of adipose tissue to the heart muscle.
- Holter monitoring takes place during the day. The study fixes the slightest changes that occur with the heart, diagnoses ventricular arrhythmia and tachycardia.
- Echocardiography. The dilatation with the asinergic character of ventricular contraction is diagnosed.
- Myocardial scintigraphy shows the segment of the ventricular lesion.
Treatment
Therapeutic
Therapy includes monitoring nutrition, as well as refusing physical exertion. Such a measure is important for all patients, including those whose dysplasia is asymptomatic and those who have a genetic predisposition to pathology.
Medication
At the moment, the medical method of treatment includes the use of a number of beta-blockers that help prevent arrhythmia and reduce the burden on the heart. Antiarrhythmics are prescribed in cases where rhythm disturbances have already been identified.
Operative intervention
For the treatment of arrhythmogenic dysplasia several methods of surgical intervention are used, among them:
- Cryodestruction of the site of the myocardium, on which the arrhythmogenic focus is located. To do this, use cold temperatures, the action of which is directed directly to the desired area.
- Catheter radiofrequency ablation is most suitable when the patient has ventricular rhythm disturbances. Therapy is the directional effect of radio frequencies on the focus of arrhythmia. Most often it is used in the absence of the effect of pharmacotherapy, in the presence of contraindications to drug treatment, as well as in cases where patients often recurve tachycardia.
- Implantation of a cardioverter-defibrillator( ICD).This technique is one of the best to prevent the risk of sudden death. CD is somewhat similar to the pacemaker, but this device does not just detect ventricular tachycardia, but also eliminates it with a special electrical impulse.
If it is impossible to control the normal rhythm of the heartbeat by any of the methods described above, then heart transplantation is used. It happens, however, very rarely.
Prevention of
The reasons for the appearance of arrhythmogenic right ventricular dysplasia remain unexplored until now, so specific prevention is also not developed. It includes only general recommendations and is aimed at eliminating secondary factors:
- exclusion of genetic predisposition;
- rejection of bad habits;
- timely treatment of poisoning, infections;
- prevention of permanent physical overvoltage;
- excludes the action on the body of such negative factors as electric shock, overheating, vibration and hypothermia.
Complications of
The consequence of an untreated disease is the development of a dangerous condition - ventricular fibrillation. This greatly reduces the likelihood of a fatal outcome. The greatest danger is created for those who often put themselves under heavy physical stress.
The appearance of right ventricular failure also refers to one of the complications that develop with dysplasia. It leads to the inability of the heart to normal work and stagnation of blood.
Forecast
A good long-term prognosis is given to patients who follow all the recommendations regarding the treatment of arrhythmogenic right ventricular dysplasia. Mortality in the first 5 years is no more than 9% of the total number of patients.
The following video contains useful information on the prevention of heart disease, including right ventricular failure: