TY - JOUR
T1 - aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes
T2 - results from the TETRIS study
AU - Conte, Giulio
AU - Bergonti, Marco
AU - Probst, Vincent
AU - Morita, Hiroshi
AU - Tfelt-Hansen, Jacob
AU - Behr, Elijah R
AU - Kengo, Kusano
AU - Arbelo, Elena
AU - Crotti, Lia
AU - Sarquella-Brugada, Georgia
AU - Wilde, Arthur A M
AU - Calò, Leonardo
AU - Sarkozy, Andrea
AU - de Asmundis, Carlo
AU - Mellor, Greg
AU - Migliore, Federico
AU - Letsas, Kostantinos
AU - Vicentini, Alessandro
AU - Levinstein, Moises
AU - Berne, Paola
AU - Chen, Shih-Ann
AU - Veltmann, Christian
AU - Biernacka, Elżbieta Katarzyna
AU - Carvalho, Paula
AU - Kabawata, Mihoko
AU - Sojema, Kyoko
AU - Gonzalez, Maria Cecilia
AU - Tse, Gary
AU - Thollet, Aurélie
AU - Svane, Jesper
AU - Caputo, Maria Luce
AU - Scrocco, Chiara
AU - Kamakura, Tsukasa
AU - Pardo, Livia Franchetti
AU - Lee, Sharen
AU - Juárez, Christian Krijger
AU - Martino, Annamaria
AU - Lo, Li-Wei
AU - Monaco, Cinzia
AU - Reyes-Quintero, Álvaro E
AU - Martini, Nicolo'
AU - Oezkartal, Tardu
AU - Klersy, Catherine
AU - Brugada, Josep
AU - Schwartz, Peter J
AU - Brugada, Pedro
AU - Belhassen, Bernard
AU - Auricchio, Angelo
N1 - © The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
PY - 2024
Y1 - 2024
N2 - 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.
AB - 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.
U2 - 10.1093/europace/euae288
DO - 10.1093/europace/euae288
M3 - Journal article
C2 - 39527076
VL - 26
JO - Europace
JF - Europace
SN - 1099-5129
IS - 12
M1 - euae288
ER -