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CO 2 The impact of clinical and genetic findings on the management of young Brugada syndrome patients

Abstract : Aims - Brugada Syndrome (BrS) is an arrhythmogenic disease associated with sudden cardiac death (SCD) which seldom manifests and is recognized in childhood. We aim to describe the pediatric BrS clinical presentation to identify prognostic factors for risk stratification, and to propose a data-based approach management. Methods and results - We studied 106 patients, under 19 years of age at diagnosis with spontaneous (n=36) or drug-induced (n=70) BrS from 16 European hospitals. At diagnosis, mean age was 11.1±5.7 years and most patients were asymptomatic [family screening (n=67), incidental (n=13)] while 15 had experienced syncope, 6 aborted SCD or symptomatic ventricular tachycardia, 2 supraventricular tachycardia (SVT), 3 palpitations or presyncope. During follow-up (median: 54 months), 10 patients had life-threatening arrhythmias (LTA) including 3 deaths. Six experienced syncope and 4 SVT. Fever triggered 27% of LTA events. An ICD was implanted in 22 with major adverse events in 41%. Of the 11 patients treated with hydroquinidine, 8 remained asymptomatic. Genetic testing was performed in 75 patients and SCN5A rare variants were identified in 58; among the 32 pediatric probands tested 15 were genotype positive. Of the 10 patients with LTA the 9 with genetic testing were all genotype positive whereas the 17 SCN5A negative patients remained asymptomatic. Spontaneous BrS type 1 ECG (p=0.005) and symptoms at diagnosis (p=0.0015) were predictors of lta. Time to the first LTA event was shorter in patients with both symptoms at diagnosis and spontaneous BrS type 1 ECG pattern (p=0.01) (figure 1). Conclusions - Spontaneous type 1 ECG and symptoms at diagnosis are predictors of LTA events in the young affected by BrS. The management of BrS should become age-specific and prevention of SCD may involve genetic testing, aggressive use of anti-pyretics and quinidine with risk-specific consideration for the ICD
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Submitted on : Wednesday, April 13, 2016 - 3:18:14 PM
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Antoine Andorin, Elijah R. Behr, Isabelle Denjoy, Lia Crotti, Laurence Jesel, et al.. CO 2 The impact of clinical and genetic findings on the management of young Brugada syndrome patients. Archives of Cardiovascular Diseases Supplements, Elsevier/French Society of Cardiology, 2015, 12e congrès médico chirurgical de la FCPC & 5es rencontres francophones multidisciplinaires des cardiopathies congénitales, 7 (4), pp.259. ⟨10.1016/S1878-6480(15)30296-2⟩. ⟨hal-01302088⟩



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