Mathilde Minier1, Vincent Probst2, Pauline Berthome1, Romain Tixier3, Jean Briand4, Olivier Geoffroy5, Nicolas Clementy6, Jacques Mansourati7, Laurence Jesel8, Jean-Marc Dupuis9, Paul Bru10, Florence Kyndt2, Béatrice Guyomarch2, Aurélie Thollet2, Nathalie Behar4, Philippe Mabo4, Frédéric Sacher3, Jean-Baptiste Gourraud11. 1. l'Institut du Thorax, CHU Nantes, Nantes, France. 2. l'Institut du Thorax, INSERM, CNRS, UNIV Nantes, CHU Nantes, Nantes, France. 3. Service de Cardiologie, Institut Lyric, CHU de Bordeaux, Bordeaux, France. 4. Service de Cardiologie, CHU de Rennes, Rennes, France. 5. Service de Cardiologie, CHU La Réunion, Saint-Denis de la Réunion, France. 6. Service de Cardiologie, CHU Tours, Tours, France. 7. Service de Cardiologie, CHU Brest, Brest, France. 8. Service de Cardiologie, CHRU Strasbourg, Strasbourg, France. 9. Service de Cardiologie, CHU Angers, Angers, France. 10. Service de Cardiologie, CH La Rochelle, La Rochelle, France. 11. l'Institut du Thorax, INSERM, CNRS, UNIV Nantes, CHU Nantes, Nantes, France. Electronic address: jeanbaptiste.gourraud@chu-nantes.fr.
Abstract
BACKGROUND: Despite a strong genetic background, Brugada syndrome (BrS) mainly affects middle-age patients. Data are scarce in the youngest and oldest age groups. OBJECTIVE: The purpose of this study was to describe the clinical characteristics and variations in rhythmic risk in BrS patients according to age. METHODS: Consecutive BrS patients diagnosed in 15 French tertiary centers in France were enrolled from 1993 to 2016 and followed up prospectively. All of the clinical and ECG data were double reviewed. RESULTS: Among the 1613 patients enrolled (age 45 ± 15 years; 69% male), 3 groups were defined according to age (52 patients <17 years; 1285 between 17 and 59 years; and 276 >60 years). In the youngest patients, we identified more female gender (42%), diagnosis by familial screening (63%), previous sudden cardiac death (15%), SCN5A mutation (62%) sinus dysfunction (8%) and aVR sign (37%) (P <.001). The oldest patients had the same clinical characteristics except for gender (40% women; P <.001). During median follow-up of 5.5 [2.1, 10.0] years, 91 patients experienced an arrhythmic event, including 7 (13%) in the youngest patients, 80 (6%) in middle-age patients, and 4 (1%) in the oldest patients. Annual event rates were 2.1%, 1%, and 0.3%, respectively (P <.01). CONCLUSION: Age on diagnosis changes the clinical presentation of BrS. Although children are identified more during familial screening, they present the highest risk of sudden cardiac death, which is an argument for early and extensive familial screening. The oldest patients present the lowest risk of SCD.
BACKGROUND: Despite a strong genetic background, Brugada syndrome (BrS) mainly affects middle-age patients. Data are scarce in the youngest and oldest age groups. OBJECTIVE: The purpose of this study was to describe the clinical characteristics and variations in rhythmic risk in BrSpatients according to age. METHODS: Consecutive BrSpatients diagnosed in 15 French tertiary centers in France were enrolled from 1993 to 2016 and followed up prospectively. All of the clinical and ECG data were double reviewed. RESULTS: Among the 1613 patients enrolled (age 45 ± 15 years; 69% male), 3 groups were defined according to age (52 patients <17 years; 1285 between 17 and 59 years; and 276 >60 years). In the youngest patients, we identified more female gender (42%), diagnosis by familial screening (63%), previous sudden cardiac death (15%), SCN5A mutation (62%) sinus dysfunction (8%) and aVR sign (37%) (P <.001). The oldest patients had the same clinical characteristics except for gender (40% women; P <.001). During median follow-up of 5.5 [2.1, 10.0] years, 91 patients experienced an arrhythmic event, including 7 (13%) in the youngest patients, 80 (6%) in middle-age patients, and 4 (1%) in the oldest patients. Annual event rates were 2.1%, 1%, and 0.3%, respectively (P <.01). CONCLUSION: Age on diagnosis changes the clinical presentation of BrS. Although children are identified more during familial screening, they present the highest risk of sudden cardiac death, which is an argument for early and extensive familial screening. The oldest patients present the lowest risk of SCD.
Authors: Mohammad Iqbal; Iwan Cahyo Santosa Putra; Raymond Pranata; Michael Nathaniel Budiarso; Miftah Pramudyo; Hanna Goenawan; Mohammad Rizki Akbar; Arief Sjamsulaksan Kartasasmita Journal: Front Cardiovasc Med Date: 2022-06-17
Authors: P J Peltenburg; Y M Hoedemaekers; S A B Clur; N A Blom; A C Blank; E P Boesaard; S Frerich; F van den Heuvel; A A M Wilde; J A E Kammeraad Journal: Neth Heart J Date: 2022-10-12 Impact factor: 2.854
Authors: Maully J Shah; Michael J Silka; Jennifer N Avari Silva; Seshadri Balaji; Cheyenne M Beach; Monica N Benjamin; Charles I Berul; Bryan Cannon; Frank Cecchin; Mitchell I Cohen; Aarti S Dalal; Brynn E Dechert; Anne Foster; Roman Gebauer; M Cecilia Gonzalez Corcia; Prince J Kannankeril; Peter P Karpawich; Jeffery J Kim; Mani Ram Krishna; Peter Kubuš; Martin J LaPage; Douglas Y Mah; Lindsey Malloy-Walton; Aya Miyazaki; Kara S Motonaga; Mary C Niu; Melissa Olen; Thomas Paul; Eric Rosenthal; Elizabeth V Saarel; Massimo Stefano Silvetti; Elizabeth A Stephenson; Reina B Tan; John Triedman; Nicholas H Von Bergen; Philip L Wackel Journal: Indian Pacing Electrophysiol J Date: 2021-07-29