Syndrome Brugada in children: symptoms, diagnosis, treatment

Syndrome Brugada refers to the primary electrical diseases of the heart with a high risk of sudden arrhythmic death.

In this syndrome, there is a delay in the right ventricle (right bundle bundle blockage), ST segment elevation in the right precordial leads (V1-V3) to a resting ECG, and a high incidence of ventricular fibrillation and sudden death, mainly at night.

Epidemiology

It is generally accepted that the syndrome is diagnosed with a frequency of 4 to 12% among all cases of sudden cardiac death. Syndrome Brugada in 8-10 times is more often met at persons of a male.

[1], [2], [3], [4], [5], [6]

Causes of Brugada Syndrome

15-15% of patients with Brugada syndrome have a mutation in the gene of sodium channels SCN5A. In most patients, the genetic nature of the phenotype of Brugada syndrome is still unclear.

[7], [8], [9], [10], [11], [12]

Symptoms of the Brugada syndrome

The criteria for diagnosis Brugada syndrome considered combination compulsory detection archway (Engl. Coved) lifting segment ST (type 1 syndrome) in at least one of the right precordial leads (V1-V3) for ECG or during tests with a blocker of the sodium channel with one of the following clinical signs:

• documented episodes of ventricular fibrillation;
• polymorphic ventricular tachycardia;
• cases of sudden death in a family under the age of 45;
• presence of the phenotype of Brugada syndrome (type I) in family members;
• Induction of ventricular fibrillation with the standard protocol of programmed ventricular stimulation;
• syncope or night attacks of severe breathing disorders.

Individuals with an ECG-type phenomenon 1 should be observed as patients with an idiopathic ECG pattern, rather than Brugada syndrome. Thus, the term "Brugada Syndrome" currently refers only to a combination of type 1 with one of the above clinical and anamnestic factors.

The first symptoms of Brugada syndrome usually appear in the 4th decade of life. Sudden death and deactivation of the defibrillator in patients are predominantly recorded at night, which indicates the importance of increased parasympathetic influences. However, the syndrome can manifest in childhood. Symptoms may appear during a rise in body temperature. When a combination of spontaneous type 1 ECG phenomenon is revealed in childhood, Brugada with loss of consciousness diagnoses Brugada's syndrome and recommends the implantation of a cardioverter-defibrillator with the subsequent administration of quinidine.

Treatment of Brugada syndrome

Current treatment recommendations include the appointment of quinidine at a dose of 300-600 mg / day or more. Isoproterenol is also indicated as an effective drug that can suppress an "electric storm" in patients with Brugada syndrome. Implantation of a cardioverter-defibrillator is the only effective method of preventing sudden death. Patients with symptoms should definitely be considered candidates for implantation. In regard to asymptomatic patients, the following are considered indications for implantation:

• Induction of ventricular fibrillation during electrophysiological examination in combination with spontaneous or registered at a sample with a sodium channel blocker type 1 ECG phenomenon Brugada;
• the phenomenon of type 1 Brugada induced in the course of the trial, combined with the presence of sudden deaths in young people in young people.

Persons who have an ECG Brugada phenomenon (type 1), induced in samples without symptoms and sudden arrhythmic deaths in the family, need observation. The electrophysiological examination and the implantation of a cardioverter-defibrillator are not shown in these cases.

The optimal strategy for the prevention of sudden arrhythmic death in patients with primary electrical heart diseases is the determination of the underlying risk (modifiable and non-modifiable factors and risk markers) and subsequent monitoring of patients in accordance with the individual risk profile.