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A rare cause of sudden death in young adults with "normal" heart: the Brugada syndrome

Lafaye, A.L.; Desachy, A.; Roustan, J.; Lagrange, P.; Vignon, P.; François, B.

Presse Medicale 28(10): 527-530

1999


ISSN/ISBN: 0755-4982
PMID: 10209540
Accession: 045103703

Brugada syndrome is a rare condition causing sudden death in young adults due to unexpected sudden-onset ventricular fibrillation. Diagnosis is based on the presence of an ST depression in the anteroseptal territory and a right branch block. No underlying dysrhythmic condition or arrhythmogenic heart disease can be detected. A 15-year old boy with an uneventful past history experienced cardiocirculatory failure due to ventricular fibrillation during a sports competition. Cardiac arrest developed and resuscitation was initially successful but death ensued due to cerebral anoxia. Characteristic electrocardiographic signs and the presence of similar anomalies in a brother and an aunt led to the diagnosis of Brugada syndrome. Brugada syndrome is a recently discovered hereditary condition with a probably underestimated prevalence. Systematic family studies have demonstrated autosomal dominant inheritance. The characteristic electrocardiographic anomalies can be transitory and may be unmasked by sensitivization tests. The only currently effective treatment is the implantable defibrillator programmed to prevent sudden death by ventricular fibrillation.

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