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

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; Nicolò Martini; Tardu Oezkartal; Catherine Klersy; Josep Brugada; Peter J. Schwartz; Pedro Brugada; Bernard Belhassen; Angelo Auricchio

doi:10.1093/europace/euae288

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

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 BerneShih 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, Nicolò Martini, Tardu Oezkartal, Catherine Klersy, Josep Brugada, Peter J. Schwartz, Pedro Brugada, Bernard Belhassen, Angelo Auricchio

Research output: Contribution to journal › Article › peer-review

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Abstract

AIMS: Little is known about the distribution and clinical course of patients with inherited arrhythmia syndrome (IAS) and concomitant atrial arrhythmias (AAs). The aim of the study is (i) to characterize the distribution of AAs in patients with IAS and (ii) evaluate the long-term clinical course of these patients. METHODS AND RESULTS: An international multicentre study was performed and involved 28 centres in 16 countries. Inclusion criteria were (i) IAS and (ii) electrocardiographic documentation of AAs. The primary endpoint was a composite of sudden cardiac death, sustained ventricular arrhythmias (VAs), or appropriate implantable cardioverter defibrillator (ICD) interventions. Strokes, inappropriate ICD shocks due to AAs, and the occurrence of sinus node dysfunction were assessed. 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, catecholaminergic polymorphic ventricular tachycardia, progressive cardiac conduction diseases, or idiopathic ventricular fibrillation. Atrial fibrillation was the most prevalent AA (82%), followed by atrial flutter (9%) and atrial tachycardia (9%). Atrial arrhythmia was the first clinical manifestation of IAS in 52% of patients. More than one type of AA 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 two-fold increase in patients who experienced their first AA before the age of 20 (odds ratio 2.2, P = 0.043). This was consistent across the different forms of IAS. Inappropriate ICD shock due to AAs was reported in 2.8% of patients, strokes in 4.4%, and sinus node dysfunction in 9.6%. CONCLUSION: 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 VAs. The occurrence of stroke and sinus node dysfunction is not infrequently in this cohort.

Original language	English
Journal	Europace
Volume	26
Issue number	12
DOIs	https://doi.org/10.1093/europace/euae288
Publication status	Published - 3 Dec 2024
Externally published	Yes

Keywords

Atrial arrhythmias
Atrial fibrillation
Brugada syndrome
Channelopathies
Inherited arrhythmia syndrome
Long QT syndrome
Sudden cardiac death
Ventricular arrhythmias

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10.1093/europace/euae288

Cite this

Conte, G., Bergonti, M., Probst, V., Morita, H., Tfelt-Hansen, J., Behr, E. R., Kengo, K., Arbelo, E., Crotti, L., Sarquella-Brugada, G., Wilde, A. A. M., Calò, L., Sarkozy, A., de Asmundis, C., Mellor, G., Migliore, F., Letsas, K., Vicentini, A., Levinstein, M., ... Auricchio, A. (2024). aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study. Europace, 26(12). https://doi.org/10.1093/europace/euae288

@article{255722d73f2c40fea0bfa8022d078fe2,

title = "aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study",

abstract = "AIMS: Little is known about the distribution and clinical course of patients with inherited arrhythmia syndrome (IAS) and concomitant atrial arrhythmias (AAs). The aim of the study is (i) to characterize the distribution of AAs in patients with IAS and (ii) evaluate the long-term clinical course of these patients. METHODS AND RESULTS: An international multicentre study was performed and involved 28 centres in 16 countries. Inclusion criteria were (i) IAS and (ii) electrocardiographic documentation of AAs. The primary endpoint was a composite of sudden cardiac death, sustained ventricular arrhythmias (VAs), or appropriate implantable cardioverter defibrillator (ICD) interventions. Strokes, inappropriate ICD shocks due to AAs, and the occurrence of sinus node dysfunction were assessed. 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, catecholaminergic polymorphic ventricular tachycardia, progressive cardiac conduction diseases, or idiopathic ventricular fibrillation. Atrial fibrillation was the most prevalent AA (82%), followed by atrial flutter (9%) and atrial tachycardia (9%). Atrial arrhythmia was the first clinical manifestation of IAS in 52% of patients. More than one type of AA 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 two-fold increase in patients who experienced their first AA before the age of 20 (odds ratio 2.2, P = 0.043). This was consistent across the different forms of IAS. Inappropriate ICD shock due to AAs was reported in 2.8% of patients, strokes in 4.4%, and sinus node dysfunction in 9.6%. CONCLUSION: 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 VAs. The occurrence of stroke and sinus node dysfunction is not infrequently in this cohort.",

keywords = "Atrial arrhythmias, Atrial fibrillation, Brugada syndrome, Channelopathies, Inherited arrhythmia syndrome, Long QT syndrome, Sudden cardiac death, Ventricular arrhythmias",

author = "Giulio Conte and Marco Bergonti and Vincent Probst and Hiroshi Morita and Jacob Tfelt-Hansen and Behr, {Elijah R.} and Kusano Kengo and Elena Arbelo and Lia Crotti and Georgia Sarquella-Brugada and Wilde, {Arthur A.M.} and Leonardo Cal{\`o} and Andrea Sarkozy and {de Asmundis}, Carlo and Greg Mellor and Federico Migliore and Kostantinos Letsas and Alessandro Vicentini and Moises Levinstein and Paola Berne and Chen, {Shih Ann} and Christian Veltmann and Biernacka, {El{\.z}bieta Katarzyna} and Paula Carvalho and Mihoko Kabawata and Kyoko Sojema and Gonzalez, {Maria Cecilia} and Gary Tse and Aur{\'e}lie Thollet and Jesper Svane and Caputo, {Maria Luce} and Chiara Scrocco and Tsukasa Kamakura and Pardo, {Livia Franchetti} and Sharen Lee and Ju{\'a}rez, {Christian Krijger} and Annamaria Martino and Lo, {Li Wei} and Cinzia Monaco and Reyes-Quintero, {{\'A}lvaro E.} and Nicol{\`o} Martini and Tardu Oezkartal and Catherine Klersy and Josep Brugada and Schwartz, {Peter J.} and Pedro Brugada and Bernard Belhassen and Angelo Auricchio",

note = "Publisher Copyright: {\textcopyright} The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.",

year = "2024",

month = dec,

day = "3",

doi = "10.1093/europace/euae288",

language = "English",

volume = "26",

number = "12",

}

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, SA, 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, LW, 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, vol. 26, no. 12. https://doi.org/10.1093/europace/euae288

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, Nicolò

AU - Oezkartal, Tardu

AU - Klersy, Catherine

AU - Brugada, Josep

AU - Schwartz, Peter J.

AU - Brugada, Pedro

AU - Belhassen, Bernard

AU - Auricchio, Angelo

PY - 2024/12/3

Y1 - 2024/12/3

N2 - AIMS: Little is known about the distribution and clinical course of patients with inherited arrhythmia syndrome (IAS) and concomitant atrial arrhythmias (AAs). The aim of the study is (i) to characterize the distribution of AAs in patients with IAS and (ii) evaluate the long-term clinical course of these patients. METHODS AND RESULTS: An international multicentre study was performed and involved 28 centres in 16 countries. Inclusion criteria were (i) IAS and (ii) electrocardiographic documentation of AAs. The primary endpoint was a composite of sudden cardiac death, sustained ventricular arrhythmias (VAs), or appropriate implantable cardioverter defibrillator (ICD) interventions. Strokes, inappropriate ICD shocks due to AAs, and the occurrence of sinus node dysfunction were assessed. 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, catecholaminergic polymorphic ventricular tachycardia, progressive cardiac conduction diseases, or idiopathic ventricular fibrillation. Atrial fibrillation was the most prevalent AA (82%), followed by atrial flutter (9%) and atrial tachycardia (9%). Atrial arrhythmia was the first clinical manifestation of IAS in 52% of patients. More than one type of AA 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 two-fold increase in patients who experienced their first AA before the age of 20 (odds ratio 2.2, P = 0.043). This was consistent across the different forms of IAS. Inappropriate ICD shock due to AAs was reported in 2.8% of patients, strokes in 4.4%, and sinus node dysfunction in 9.6%. CONCLUSION: 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 VAs. The occurrence of stroke and sinus node dysfunction is not infrequently in this cohort.

AB - AIMS: Little is known about the distribution and clinical course of patients with inherited arrhythmia syndrome (IAS) and concomitant atrial arrhythmias (AAs). The aim of the study is (i) to characterize the distribution of AAs in patients with IAS and (ii) evaluate the long-term clinical course of these patients. METHODS AND RESULTS: An international multicentre study was performed and involved 28 centres in 16 countries. Inclusion criteria were (i) IAS and (ii) electrocardiographic documentation of AAs. The primary endpoint was a composite of sudden cardiac death, sustained ventricular arrhythmias (VAs), or appropriate implantable cardioverter defibrillator (ICD) interventions. Strokes, inappropriate ICD shocks due to AAs, and the occurrence of sinus node dysfunction were assessed. 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, catecholaminergic polymorphic ventricular tachycardia, progressive cardiac conduction diseases, or idiopathic ventricular fibrillation. Atrial fibrillation was the most prevalent AA (82%), followed by atrial flutter (9%) and atrial tachycardia (9%). Atrial arrhythmia was the first clinical manifestation of IAS in 52% of patients. More than one type of AA 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 two-fold increase in patients who experienced their first AA before the age of 20 (odds ratio 2.2, P = 0.043). This was consistent across the different forms of IAS. Inappropriate ICD shock due to AAs was reported in 2.8% of patients, strokes in 4.4%, and sinus node dysfunction in 9.6%. CONCLUSION: 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 VAs. The occurrence of stroke and sinus node dysfunction is not infrequently in this cohort.

KW - Atrial arrhythmias

KW - Atrial fibrillation

KW - Brugada syndrome

KW - Channelopathies

KW - Inherited arrhythmia syndrome

KW - Long QT syndrome

KW - Sudden cardiac death

KW - Ventricular arrhythmias

UR - http://www.scopus.com/inward/record.url?scp=85212457367&partnerID=8YFLogxK

U2 - 10.1093/europace/euae288

DO - 10.1093/europace/euae288

M3 - Article

C2 - 39527076

AN - SCOPUS:85212457367

SN - 1099-5129

VL - 26

JO - Europace

JF - Europace

IS - 12

ER -

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

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