Giulio Conte, Marco Bergonti, Vincent Probst, Hiroshi Morita, Jacob Tfelt-Hansen, Elijah R Behr, Kusano Kengo, Elena Arbelo, Lia Crotti, Georgia Sarquella-Brugada, Arthur A M Wilde, Leonardo Calò, Andrea Sarkozy, Carlo de Asmundis, Greg Mellor, Federico Migliore, Kostantinos Letsas, Alessandro Vicentini, Moises Levinstein, Paola Berne, Shih-Ann Chen, Christian Veltmann, Elżbieta Katarzyna Biernacka, Paula Carvalho, Mihoko Kabawata, Kyoko Sojema, Maria Cecilia Gonzalez, Gary Tse, Aurélie Thollet, Jesper Svane, Maria Luce Caputo, Chiara Scrocco, Tsukasa Kamakura, Livia Franchetti Pardo, Sharen Lee, Christian Krijger Juárez, Annamaria Martino, Li-Wei Lo, Cinzia Monaco, Álvaro E Reyes-Quintero, Nicolo' Martini, Tardu Oezkartal, Catherine Klersy, Josep Brugada, Peter J Schwartz, Pedro Brugada, Bernard Belhassen, Angelo Auricchio
{"title":"aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study.","authors":"Giulio Conte, Marco Bergonti, Vincent Probst, Hiroshi Morita, Jacob Tfelt-Hansen, Elijah R Behr, Kusano Kengo, Elena Arbelo, Lia Crotti, Georgia Sarquella-Brugada, Arthur A M Wilde, Leonardo Calò, Andrea Sarkozy, Carlo de Asmundis, Greg Mellor, Federico Migliore, Kostantinos Letsas, Alessandro Vicentini, Moises Levinstein, Paola Berne, Shih-Ann Chen, Christian Veltmann, Elżbieta Katarzyna Biernacka, Paula Carvalho, Mihoko Kabawata, Kyoko Sojema, Maria Cecilia Gonzalez, Gary Tse, Aurélie Thollet, Jesper Svane, Maria Luce Caputo, Chiara Scrocco, Tsukasa Kamakura, Livia Franchetti Pardo, Sharen Lee, Christian Krijger Juárez, Annamaria Martino, Li-Wei Lo, Cinzia Monaco, Álvaro E Reyes-Quintero, Nicolo' Martini, Tardu Oezkartal, Catherine Klersy, Josep Brugada, Peter J Schwartz, Pedro Brugada, Bernard Belhassen, Angelo Auricchio","doi":"10.1093/europace/euae288","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Little is known about the distribution and clinical course of patients with inherited arrhythmia syndrome (IAS) and concomitant atrial arrhythmias (AAs).</p><p><strong>Aim: </strong>1) to characterize the distribution of AAs in patients with IAS and 2) evaluate the long-term clinical course of these patients.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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%.</p><p><strong>Conclusions: </strong>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 associated with a higher risk of ventricular arrhythmias. The occurrence of stroke and sinus node dysfunction is not-infrequently in this cohort.</p>","PeriodicalId":11981,"journal":{"name":"Europace","volume":" ","pages":""},"PeriodicalIF":7.9000,"publicationDate":"2024-11-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Europace","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/europace/euae288","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
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Abstract
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 associated with a higher risk of ventricular arrhythmias. The occurrence of stroke and sinus node dysfunction is not-infrequently in this cohort.
期刊介绍：
EP - Europace - European Journal of Pacing, Arrhythmias and Cardiac Electrophysiology of the European Heart Rhythm Association of the European Society of Cardiology. The journal aims to provide an avenue of communication of top quality European and international original scientific work and reviews in the fields of Arrhythmias, Pacing and Cellular Electrophysiology. The Journal offers the reader a collection of contemporary original peer-reviewed papers, invited papers and editorial comments together with book reviews and correspondence.
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