Details

Autor(en) / Beteiligte

• Andorin, Antoine
• Behr, Elijah R.
• Denjoy, Isabelle
• Crotti, Lia
• Dagradi, Federica
• Jesel, Laurence
• Sacher, Frederic
• Petit, Bertrand
• Mabo, Philippe
• Maltret, Alice
• Wong, Leonie C. H.
• Degand, Bruno
• Bertaux, Geraldine
• Maury, Philippe
• Dulac, Yves
• Delasalle, Béatrice
• Gourraud, Jean-Baptiste
• Babuty, Dominique
• Blom, Nico A.
• Schwartz, Peter J.
• Wilde, Arthur A.
• Probst, Vincent

Titel

The Impact of Clinical and Genetic Findings on The Management of Young Brugada Syndrome Patients

Ist Teil von

• Heart rhythm, 2016-02, Vol.13 (6), p.1274-1282

Ort / Verlag

Elsevier

Erscheinungsjahr

2016

Quelle

Access via ScienceDirect (Elsevier)

Beschreibungen/Notizen

• Background - Brugada syndrome (BrS) is an arrhythmogenic disease associated with sudden cardiac death (SCD) that seldom manifests or is recognized in childhood. Objectives - The objectives of this study were to describe the clinical presentation of pediatric BrS to identify prognostic factors for risk stratification and to propose a data-based approach management. Methods - We studied 106 patients younger than 19 years at diagnosis of BrS enrolled from 16 European hospitals. Results - At diagnosis, BrS was spontaneous (n = 36, 34%) or drug-induced (n = 70, 66%). The mean age was 11.1 ± 5.7 years, and most patients were asymptomatic (family screening, (n = 67, 63%; incidental, n = 13, 12%), while 15 (14%) experienced syncope, 6(6%) aborted SCD or symptomatic ventricular tachycardia, and 5 (5%) other symptoms. During follow-up (median 54 months), 10 (9%) patients had life-threatening arrhythmias (LTA), including 3 (3%) deaths. Six (6%) experienced syncope and 4 (4%) supraventricular tachycardia. Fever triggered 27% of LTA events. An implantable cardioverter-defibrillator was implanted in 22 (21%), with major adverse events in 41%. Of the 11 (10%) patients treated with hydroquinidine, 8 remained asymptomatic. Genetic testing was performed in 75 (71%) patients, and SCN5A rare variants were identified in 58 (55%); 15 of 32 tested probands (47%) were genotype positive. Nine of 10 patients with LTA underwent genetic testing, and all were genotype positive, whereas the 17 SCN5A-negative patients remained asymptomatic. Spontaneous Brugada type 1 electrocardiographic (ECG) pattern (P = .005) and symptoms at diagnosis (P = .001) were predictors of LTA. Time to the first LTA event was shorter in patients with both symptoms at diagnosis and spontaneous Brugada type 1 ECG pattern (P = .006). Conclusion - Spontaneous Brugada type 1 ECG pattern 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 and aggressive use of antipyretics and quinidine, with risk-specific consideration for the implantable cardioverter-defibrillator.