aTrial arrhythmias in inhEriTed aRrhythmIa Syndromes: results from the TETRIS study
Por:
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-Terradellas J, Schwartz PJ, Brugada P, Belhassen B and Auricchio A
Publicada:
5 dic 2024
Ahead of Print:
11 nov 2024
Resumen:
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.
Filiaciones:
Conte G:
Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland
Faculty of Biomedical Sciences, Università della Svizzera Italiana, Switzerland
Bergonti M:
Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland
Probst V:
L'institut du thorax
CHU de Nantes, Nantes, France
Morita H:
Department of Cardiovascular Therapeutics, Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan
Tfelt-Hansen J:
ERN GUARDHEART
Rigshospitalet - Copenhagen University Hospital, Copenhagen
Behr ER:
ERN GUARDHEART
Cardiovascular and Genomics Research Institute, St. George's, University of London and St. George's University Hospitals NHS Foundation Trust, London, UK
Kengo K:
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
Arbelo E:
ERN GUARDHEART
Arrhythmia Section, Cardiology Department, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain
Crotti L:
IRCCS, Istituto Auxologico Italiano, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy
Departement of Medicine and Surgery, University Milano Bicocca, Milan, Italy
Sarquella-Brugada G:
ERN GUARDHEART
Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Hospital Sant Joan de Déu, Universitat de Barcelona
Wilde AAM:
Department of Cardiology, Amsterdam University Medical Center, Amsterdam, the Netherlands
Calò L:
Cardiology Department, Policlinico Casilino, Rome, Italy
Sarkozy A:
University Hospital Antwerp, Antwerp, Belgium
Heart Rhythm Management Centre, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel - Vrije Universiteit Brussel, European Reference Networks Guard-Heart, Brussels, Belgium
de Asmundis C:
ERN GUARDHEART
Heart Rhythm Management Centre, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel - Vrije Universiteit Brussel, European Reference Networks Guard-Heart, Brussels, Belgium
Mellor G:
Cardiology Department, Royal Papworth Hospital NHS Foundation Trust, Cambridge, UK
Migliore F:
Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, Italy
Letsas K:
Arrhythmia Unit, Onassis Cardiac Surgery Center, Athens, Greece
Vicentini A:
Divisione di Cardiologia, IRCCS Policlinico S. Matteo, Pavia, Italy
Levinstein M:
Nacional de Cardiología "Ignacio Chávez", Mexico City, Mexico
Berne P:
Cardiology Department, Ospedale Santissima Annunziata, Azienda Ospedaliera Universitaria, Sassari, Italy
Chen SA:
Heart Rhythm center, Taipei veterans general hospital and Cardiovascular center, Taichung veterans general hospital
National Yang Ming Chiao Tung University and National Chung Hsing University, Taiwan
Veltmann C:
Heart Center Bremen, Electrophysiology Bremen, Bremen, Germany
Biernacka EK:
Cardinal Wyszynski National Institute of Cardiology, Warsaw, Poland
Carvalho P:
Cardiology department, University Hospital San Luigi Gonzaga di Orbassano, Italia
Kabawata M:
Department of Cardiovascular Disease, AOI Universal Hospital, Kanagawa, Japan
Sojema K:
Department Cardiovascular Medicine, Kyorin University, Japan
Gonzalez MC:
Pediatric Cardiology and Electrophysiology, Sainte Justine- University of Montreal, Canada
Tse G:
Faculty of Medicine, Chinese University of Hong Kong, Hong Kong, China
Thollet A:
L'institut du thorax
CHU de Nantes, Nantes, France
Svane J:
Rigshospitalet - Copenhagen University Hospital, Copenhagen
Caputo ML:
Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland
Scrocco C:
Cardiovascular and Genomics Research Institute, St. George's, University of London and St. George's University Hospitals NHS Foundation Trust, London, UK
Kamakura T:
Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan
Pardo LF:
Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland
Lee S:
Faculty of Medicine, Chinese University of Hong Kong, Hong Kong, China
Cardiovascular Analytics Group, Hong Kong SAR
Juárez CK:
Department of Cardiology, Amsterdam University Medical Center, Amsterdam, the Netherlands
Martino A:
Cardiology Department, Policlinico Casilino, Rome, Italy
Lo LW:
Heart Rhythm Center, Cardiovascular Center, Taipei Veterans General Hospital, Taipei, Taiwan
Monaco C:
Heart Rhythm Management Centre, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel - Vrije Universiteit Brussel, European Reference Networks Guard-Heart, Brussels, Belgium
Reyes-Quintero ÁE:
Centro Universitario de Ciencias de la Salud, Universidad de Guadalajara, Guadalajara, Jalisco, México
Martini N:
Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, Italy
Oezkartal T:
Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland
Klersy C:
Biostatistics & Clinical Trial Center, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
Brugada-Terradellas J:
Arrhythmia Section, Cardiology Department, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain
Schwartz PJ:
IRCCS, Istituto Auxologico Italiano, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy
Brugada P:
Heart Rhythm Management Centre, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel - Vrije Universiteit Brussel, European Reference Networks Guard-Heart, Brussels, Belgium
Belhassen B:
Heart Institute, Hadassah Medical Center, Jerusalem, Israel
Tel Aviv University, Tel Aviv, Israel
Auricchio A:
Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland
Faculty of Biomedical Sciences, Università della Svizzera Italiana, Switzerland
Green Submitted, hybrid
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