Brugada Syndrome

Brugada syndrome is a hereditary disorder of cardiac electrophysiology, which has an increased risk of syncope and sudden death.

Several different mutations are involved, most concern the SCN5A gene encoding the ?-subunit of the voltage-dependent sodium channel in the heart. Typically, patients without structural heart disease. However, there is an overlap with other genetic and acquired structural heart disease, such as the long QT syndrome type 3 and arrhythmogenic right dysplasia (ARVD).

Brugada syndrome is a hereditary disorder of cardiac electrophysiology, which has an increased risk of syncope and sudden death. Several different mutations are involved, most concern the SCN5A gene encoding the ?-subunit of the voltage-dependent sodium channel in the heart. Typically, patients without structural heart disease. However, there is an overlap with other genetic and acquired structural heart disease, such as the long QT syndrome type 3 and arrhythmogenic right dysplasia (ARVD). In some patients, the Brugada syndrome manifests clinically not. In many patients, however, it leads to syncope or sudden cardiac death due to polymorphic ventricular tachycardia and ventricular fibrillation. The events often occur at night and are not usually associated with physical exertion. Events can also be triggered by fever and by treatment with certain medications (u. A. Sodium channel blockers, beta blockers, antidepressants, lithium and cocaine). Diagnostic ECG The first diagnosis of Burgada syndrome based on a characteristic ECG patterns (Brugada ECG pattern type 1 Brugada syndrome type 1) with a striking ST elevation in V1 and V2 (sometimes V3), whereby the QRS complex looks a right bundle branch block similar in these derivatives. The ST segment is curved and merges into an inverted T-wave. Lower values ??of these patterns (Brugada ECG pattern type 2 and type 3) are not regarded as diagnostically. The pattern type 2 and type 3 can be transformed by fever or in response to drugs spontaneously in a type 1 pattern. The latter ECG patterns represent the basis for a challenge test, usually with Ajmalin or procainamide,. The same diagnosis should be considered in patients with unexplained cardiac arrest or syncope or a family history. The role of electrophysiological studies is unclear and is the subject of ongoing studies. Brugada syndrome type 1 Marked elevation of the J-point resulting in a curved ST-segment to an inverted T-wave in leads V1 and V2. Therapy insertion of an implantable cardioverter defibrillator (ICD) patients with syncope and patients who were revived after a cardiac arrest, an implantable cardioverter defibrillator should be used. The best treatment of Burgada syndrome in patients whose diagnosis is based on ECG changes and family history unclear, although with them an increased risk of sudden death.

Health Life Media Team

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