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aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study.

Title: aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study.
Authors: Conte, G; Bergonti, M; Probst, V; Morita, H; Tfelt-Hansen, J; Behr, ER; Kengo, K; Arbelo, E; Crotti, L; Sarquella-Brugada, G; Wilde, AAM; Calò, L; Sarkozy, A; de Asmundis, C; Mellor, G; Migliore, F; Letsas, K; Vicentini, A; Levinstein, M; Berne, P; Chen, S-A; Veltmann, C; Biernacka, EK; Carvalho, P; Kabawata, M; Sojema, K; Gonzalez, MC; Tse, G; Thollet, A; Svane, J; Caputo, ML; Scrocco, C; Kamakura, T; Pardo, LF; Lee, S; Juárez, CK; Martino, A; Lo, L-W; Monaco, C; Reyes-Quintero, ÁE; Martini, N; Oezkartal, T; Klersy, C; Brugada, J; Schwartz, PJ; Brugada, P; Belhassen, B; Auricchio, A
Publisher Information: Oxford University Press
Publication Year: 2024
Collection: St George's University of London: Repository
Description: BACKGROUND: Little is known about the distribution and clinical course of patients with inherited arrhythmia syndrome (IAS) and concomitant atrial arrhythmias (AAs). AIM: 1) to characterize the distribution of AAs in patients with IAS and 2) evaluate the long-term clinical course of these patients. METHODS: An international multicenter study was performed and involved 28 centers in 16 countries. Inclusion criteria were: 1) IAS and 2) ECG documentation of AAs. The primary endpoint was a composite of sudden cardiac death, sustained VAs or appropriate ICD interventions. Strokes, inappropriate ICD shocks due to AAs, and the occurrence of sinus node dysfunction were assessed. RESULTS: A total of 522 patients with IAS and AAs were included. Most patients were diagnosed with Brugada syndrome (n=355, 68%) and long-QT syndrome (n=93, 18%). The remaining patients (n=71, 14%) presented with short-QT syndrome, early repolarization syndrome (ERS), catecholaminergic polymorphic ventricular tachycardia (CPVT), progressive cardiac conduction diseases, or idiopathic ventricular fibrillation. Atrial fibrillation (AF) was the most prevalent AA (82%), followed by atrial flutter (9%) and atrial tachycardia (9%). AA was the first clinical manifestation of IAS in 52% of patients. More than one type of AAs was documented in 23% of patients. Nine patients (3%) experienced VA before the diagnosis of IAS, due the use of anti-arrhythmic medications taken for the AA. The incidence of the primary endpoint was 1.4% per year, with a twofold increase observed in patients who experienced their first AA before the age of 20 (OR 2.2, p=0.043). This was consistent across the different forms of IAS. Inappropriate ICD shock due to AAs were reported in 2.8% of patients, strokes in 4.4% and sinus node dysfunction in 9.6%. CONCLUSIONS: Among patients with IAS and AAs, AA is the first clinical manifestation in about half of the cases, with more than one form of AAs present in one-fourth of the patients. The occurrence of AA earlier in life may be ...
Document Type: article in journal/newspaper
File Description: application/pdf
Language: English
ISSN: 1532-2092
Relation: https://openaccess.sgul.ac.uk/id/eprint/116961/6/euae288.pdf; https://openaccess.sgul.ac.uk/id/eprint/116961/1/euae288.pdf; Conte, G; Bergonti, M; Probst, V; Morita, H; Tfelt-Hansen, J; Behr, ER; Kengo, K; Arbelo, E; Crotti, L; Sarquella-Brugada, G; et al. Conte, G; Bergonti, M; Probst, V; Morita, H; Tfelt-Hansen, J; Behr, ER; Kengo, K; Arbelo, E; Crotti, L; Sarquella-Brugada, G; Wilde, AAM; Calò, L; Sarkozy, A; de Asmundis, C; Mellor, G; Migliore, F; Letsas, K; Vicentini, A; Levinstein, M; Berne, P; Chen, S-A; Veltmann, C; Biernacka, EK; Carvalho, P; Kabawata, M; Sojema, K; Gonzalez, MC; Tse, G; Thollet, A; Svane, J; Caputo, ML; Scrocco, C; Kamakura, T; Pardo, LF; Lee, S; Juárez, CK; Martino, A; Lo, L-W; Monaco, C; Reyes-Quintero, ÁE; Martini, N; Oezkartal, T; Klersy, C; Brugada, J; Schwartz, PJ; Brugada, P; Belhassen, B; Auricchio, A (2024) aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study. Europace, 26 (12). euae288. ISSN 1532-2092 https://doi.org/10.1093/europace/euae288 SGUL Authors: Behr, Elijah Raphael
DOI: 10.1093/europace/euae288
Availability: https://openaccess.sgul.ac.uk/id/eprint/116961/; https://openaccess.sgul.ac.uk/id/eprint/116961/6/euae288.pdf; https://openaccess.sgul.ac.uk/id/eprint/116961/1/euae288.pdf; https://doi.org/10.1093/europace/euae288
Rights: cc_by_nc_4
Accession Number: edsbas.D6C3DA20
Database: BASE