Importance of genotype for risk stratification in arrhythmogenic right ventricular cardiomyopathy using the 2019 ARVC risk calculator
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- Alexandros Protonotarios
- Institute of Cardiovascular Science, University College London , London , UK
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- Riccardo Bariani
- Department of Cardiac Thoracic, Vascular Sciences and Public Health, University of Padua , Padua , Italy
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- Chiara Cappelletto
- Cardio-Thoraco-Vascular Department, University of Trieste , Trieste , Italy
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- Menelaos Pavlou
- Department of Statistical Science, University College London , London , UK
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- Alba García-García
- Inherited Cardiac Diseases Unit (CSUR-ERN), Department of Cardiology, Hospital Clínico Universitario Virgen de la Arrixaca , Murcia , Spain
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- Alberto Cipriani
- Department of Cardiac Thoracic, Vascular Sciences and Public Health, University of Padua , Padua , Italy
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- Ioannis Protonotarios
- Nikos Protonotarios Medical Centre , Naxos , Greece
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- Adrian Rivas
- Heart Failure and Inherited Cardiac Diseases Unit, Hospital Universitario Puerta de Hierro Majadahonda , Madrid , Spain
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- Regitze Wittenberg
- Department of Cardiology, Odense University Hospital , Odense , Denmark
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- Maddalena Graziosi
- Cardiology Unit, St Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna , Bologna , Italy
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- Zafeirenia Xylouri
- Nikos Protonotarios Medical Centre , Naxos , Greece
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- José M Larrañaga-Moreira
- Unidad de Cardiopatías Familiares, Instituto de Investigación Biomédica de A Coruña (INIBIC), Complexo Hospitalario Universitario de A Coruña, Servizo Galego de Saúde (SERGAS), Universidade da Coruña, CIBERCV , A Coruña , Spain
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- Antonio de Luca
- Cardio-Thoraco-Vascular Department, University of Trieste , Trieste , Italy
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- Rudy Celeghin
- Department of Cardiac Thoracic, Vascular Sciences and Public Health, University of Padua , Padua , Italy
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- Kalliopi Pilichou
- Department of Cardiac Thoracic, Vascular Sciences and Public Health, University of Padua , Padua , Italy
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- Athanasios Bakalakos
- Institute of Cardiovascular Science, University College London , London , UK
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- Luis Rocha Lopes
- Institute of Cardiovascular Science, University College London , London , UK
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- Konstantinos Savvatis
- Institute of Cardiovascular Science, University College London , London , UK
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- Davide Stolfo
- Cardio-Thoraco-Vascular Department, University of Trieste , Trieste , Italy
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- Matteo Dal Ferro
- Cardio-Thoraco-Vascular Department, University of Trieste , Trieste , Italy
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- Marco Merlo
- Cardio-Thoraco-Vascular Department, University of Trieste , Trieste , Italy
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- Cristina Basso
- Department of Cardiac Thoracic, Vascular Sciences and Public Health, University of Padua , Padua , Italy
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- Javier Limeres Freire
- European Reference Networks for rare, low prevalence and complex diseases of the heart (ERN GUARD-Heart)
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- Jose F Rodriguez-Palomares
- European Reference Networks for rare, low prevalence and complex diseases of the heart (ERN GUARD-Heart)
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- Toru Kubo
- Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University , Japan
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- Tomas Ripoll-Vera
- Inherited Cardiovascular Diseases Unit, Son Llatzer University Hospital & IdISBa , Palma de Mallorca , Spain
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- Roberto Barriales-Villa
- Unidad de Cardiopatías Familiares, Instituto de Investigación Biomédica de A Coruña (INIBIC), Complexo Hospitalario Universitario de A Coruña, Servizo Galego de Saúde (SERGAS), Universidade da Coruña, CIBERCV , A Coruña , Spain
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- Loizos Antoniades
- Cyprus Institute of Cardiomyopathies and Inherited Cardiovascular Diseases , Nicosia , Cyprus
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- Jens Mogensen
- Aalborg University Hospital , Denmark
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- Pablo Garcia-Pavia
- Heart Failure and Inherited Cardiac Diseases Unit, Hospital Universitario Puerta de Hierro Majadahonda , Madrid , Spain
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- Karim Wahbi
- Cardiology Department, AP-HP, Cochin Hospital, FILNEMUS, Centre de Référence de Pathologie Neuromusculaire Nord/Est/Île-de-France, Paris-Descartes, Sorbonne Paris Cité University , Paris , France
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- Elena Biagini
- Cardiology Unit, St Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna , Bologna , Italy
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- Aris Anastasakis
- Unit of Inherited and Rare Cardiovascular Diseases, Onassis Cardiac Surgery Centre , Athens , Greece
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- Adalena Tsatsopoulou
- Nikos Protonotarios Medical Centre , Naxos , Greece
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- Esther Zorio
- Centre for Biomedical Network Research on Cardiovascular Diseases (CIBERCV) , Madrid , Spain
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- Juan R Gimeno
- Inherited Cardiac Diseases Unit (CSUR-ERN), Department of Cardiology, Hospital Clínico Universitario Virgen de la Arrixaca , Murcia , Spain
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- Jose Manuel Garcia-Pinilla
- Centre for Biomedical Network Research on Cardiovascular Diseases (CIBERCV) , Madrid , Spain
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- Petros Syrris
- Institute of Cardiovascular Science, University College London , London , UK
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- Gianfranco Sinagra
- Cardio-Thoraco-Vascular Department, University of Trieste , Trieste , Italy
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- Barbara Bauce
- Department of Cardiac Thoracic, Vascular Sciences and Public Health, University of Padua , Padua , Italy
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- Perry M Elliott
- Institute of Cardiovascular Science, University College London , London , UK
書誌事項
- 公開日
- 2022-06-29
- 権利情報
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- https://creativecommons.org/licenses/by/4.0/
- DOI
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- 10.1093/eurheartj/ehac235
- 公開者
- Oxford University Press (OUP)
この論文をさがす
説明
<jats:title>Abstract</jats:title> <jats:sec> <jats:title>Aims</jats:title> <jats:p>To study the impact of genotype on the performance of the 2019 risk model for arrhythmogenic right ventricular cardiomyopathy (ARVC).</jats:p> </jats:sec> <jats:sec> <jats:title>Methods and results</jats:title> <jats:p>The study cohort comprised 554 patients with a definite diagnosis of ARVC and no history of sustained ventricular arrhythmia (VA). During a median follow-up of 6.0 (3.1,12.5) years, 100 patients (18%) experienced the primary VA outcome (sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator intervention, aborted sudden cardiac arrest, or sudden cardiac death) corresponding to an annual event rate of 2.6% [95% confidence interval (CI) 1.9–3.3]. Risk estimates for VA using the 2019 ARVC risk model showed reasonable discriminative ability but with overestimation of risk. The ARVC risk model was compared in four gene groups: PKP2 (n = 118, 21%); desmoplakin (DSP) (n = 79, 14%); other desmosomal (n = 59, 11%); and gene elusive (n = 160, 29%). Discrimination and calibration were highest for PKP2 and lowest for the gene-elusive group. Univariable analyses revealed the variable performance of individual clinical risk markers in the different gene groups, e.g. right ventricular dimensions and systolic function are significant risk markers in PKP2 but not in DSP patients and the opposite is true for left ventricular systolic function.</jats:p> </jats:sec> <jats:sec> <jats:title>Conclusion</jats:title> <jats:p>The 2019 ARVC risk model performs reasonably well in gene-positive ARVC (particularly for PKP2) but is more limited in gene-elusive patients. Genotype should be included in future risk models for ARVC.</jats:p> </jats:sec>
収録刊行物
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- European Heart Journal
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European Heart Journal 43 (32), 3053-3067, 2022-06-29
Oxford University Press (OUP)
- Tweet
キーワード
- Desmoplaquinas
- Genotype
- Arritmias Cardíacas
- Arrhythmias
- Risk Assessment
- Historia
- Desfibriladores implantables
- Displasia Ventricular Derecha Arritmogénica
- Clinical Research
- Risk Factors
- Humans
- Muerte Súbita Cardíaca
- Death, Sudden, Cardiac/epidemiology
- Risk stratification
- Arrhythmogenic Right Ventricular Dysplasia
- Taquicardia ventricular
- Arrhythmogenic Right Ventricular Dysplasia/genetics
- Factores de Riesgo
- Arrhythmogenic right ventricular cardiomyopathy; Genotype; Risk stratification; Sudden cardiac death; Ventricular arrhythmia
- Diagnóstico
- Arrhythmias, Cardiac
- Sudden
- Humanos
- Death
- Arritmias cardíacas
- Sudden cardiac death
- Death, Sudden, Cardiac
- Muerte súbita cardíaca
- Ventricular arrhythmia
- Cardiac/epidemiology
- Arrhythmogenic right ventricular cardiomyopathy
- Cardiac
- Displasia ventricular derecha arritmogénica
- Genotipo
- Medición de Riesgo
詳細情報 詳細情報について
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- CRID
- 1360021392655960704
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- ISSN
- 15229645
- 0195668X
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- PubMed
- 35766183
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- データソース種別
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- Crossref
- OpenAIRE
