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P. P. Martorano, F. Boncagni

Brugada syndrome

Brugada syndrome

Schlüsselwörter Brugada syndrome; ICD 10: I47 .2; SUNDS - Sudden unexplained nocturnal death syndrome, Idiopathic ventricular fibrillation, Pokkuri (Japanese), Bangungut, Lai Tai (Philippines and Southeastern Asia)
Keywords Brugada syndrome; ICD 10: I47 .2; SUNDS - Sudden unexplained nocturnal death syndrome, Idiopathic ventricular fibrillation, Pokkuri (Japanese), Bangungut, Lai Tai (Philippines and Southeastern Asia)
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Summary

Brugada syndrome is an arrhythmogenic cardiopathy defined both by the presence of ECG alterations at rest and by the occurrence of malignant tachyarrhythmias. Patients usually display complete or incomplete right bundle branch block pattern in more than one right precordial lead (V1-V3) at ECG, in association with variable ST segment elevation (more or less than 2 mm, coved-type or saddle-back type). Clinical presentation includes syncope, typically occurring at rest or during sleep, and it is caused by fast polymorphic ventricular tachycardia. In some cases ventricular fibrillation may occur, leading to cardiac arrest and sudden death. The prevalence of the disease ranges from 5/10000 (Caucasians) to 14/10000 (Japanese) and it is considered a major cause of sudden death among young males of asian origin without cardiopathy background.

Although de novo mutations are possible, the syndrome shows an autosomal dominant inheritance with incomplete penetrance.Thus far, only loss of function mutations in the gene encoding for cardiac sodium channel (SCN5A on chromosome 3p21-23) are certainly linked to the syndrome, but they are identified just in 20% of patients.Therefore, other ionic channel disturbances may have a role in the disease (e.g. calcium channel CACNA1c and CACNB2b alterations). Average age of diagnosis is 40 years and implantable cardioverter-defibrillator (ICD) is the only effective therapeutic option for symptomatic patients with spontaneous or pharmacologic-induced ECG pattern. Local anaesthetics (especially bupivacaine), as well as increased vagal tone, fever, inadequate analgesia and electrolyte imbalances, may trigger malignant arrhythmias in these patients.

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