Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator
Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this predicti...
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| Format: | Article |
| Status: | Published version |
| Publication date: | 2022 |
| Country: | España |
| Institution: | Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau) |
| Repository: | r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau |
| OAI Identifier: | oai:iibsantpau.fundanetsuite.com:p12005 |
| Online Access: | https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12005 https://ddd.uab.cat/record/282315 |
| Access Level: | Open access |
| Keyword: | Arrhythmogenic right ventricular cardiomyopathy Implantable cardioverter-defibrillator Sudden cardiac death Ventricular arrhythmias Risk stratification Genetic cardiomyopathies |
| _version_ | 1878740741235146752 |
|---|---|
| author | Jorda, P |
| author2 | Bosman, LP Gasperetti, A Mazzanti, A Gourraud, JB Davies, B Frederiksen, TC Weidmann, ZM Di Marco, A Roberts, JD MacIntyre, C Seifer, C Deliniere, A Alqarawi, W Kukavica, D Minois, D Trancuccio, A Arnaud, M Targetti, M Martino, A Oliviero, G Pipilas, DC Carbucicchio, C Compagnucci, P Dello Russo, A Olivotto, I Calo, L Lubitz, SA Cutler, MJ Chevalier, P Arbelo, E Priori, SG Healey, JS Calkins, H Casella, M Jensen, HK Tondo, C Tadros, R James, CA Krahn, AD Cadrin-Tourigny, J |
| author2_role | author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
| author_browse | Alqarawi, W Arbelo, E Arnaud, M Bosman, LP Cadrin-Tourigny, J Calkins, H Calo, L Carbucicchio, C Casella, M Chevalier, P Compagnucci, P Cutler, MJ Davies, B Deliniere, A Dello Russo, A Di Marco, A Frederiksen, TC Gasperetti, A Gourraud, JB Healey, JS James, CA Jensen, HK Jorda, P Krahn, AD Kukavica, D Lubitz, SA MacIntyre, C Martino, A Mazzanti, A Minois, D Oliviero, G Olivotto, I Pipilas, DC Priori, SG Roberts, JD Seifer, C Tadros, R Targetti, M Tondo, C Trancuccio, A Weidmann, ZM |
| author_facet | Jorda, P Bosman, LP Gasperetti, A Mazzanti, A Gourraud, JB Davies, B Frederiksen, TC Weidmann, ZM Di Marco, A Roberts, JD MacIntyre, C Seifer, C Deliniere, A Alqarawi, W Kukavica, D Minois, D Trancuccio, A Arnaud, M Targetti, M Martino, A Oliviero, G Pipilas, DC Carbucicchio, C Compagnucci, P Dello Russo, A Olivotto, I Calo, L Lubitz, SA Cutler, MJ Chevalier, P Arbelo, E Priori, SG Healey, JS Calkins, H Casella, M Jensen, HK Tondo, C Tadros, R James, CA Krahn, AD Cadrin-Tourigny, J |
| author_role | author |
| collection | r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau |
| description | Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. Methods and results In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Conclusion Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC. |
| eu_rights_str_mv | openAccess |
| format | article |
| id | ES_d88f374b258df03f21c4fcbad8f9f8a9 |
| institution | Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau) |
| instname_str | Institut d’Investigació Biomèdica Sant Pau (IIB Sant Pau) |
| network_acronym_str | ES |
| network_name_str | España |
| oai_identifier_str | oai:iibsantpau.fundanetsuite.com:p12005 |
| publishDate | 2022 |
| publishDateSort | 2022 |
| publisher | OXFORD UNIV PRESS |
| publisherStr | OXFORD UNIV PRESS |
| reponame_str | r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau |
| spelling | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator Jorda, P Bosman, LP Gasperetti, A Mazzanti, A Gourraud, JB Davies, B Frederiksen, TC Weidmann, ZM Di Marco, A Roberts, JD MacIntyre, C Seifer, C Deliniere, A Alqarawi, W Kukavica, D Minois, D Trancuccio, A Arnaud, M Targetti, M Martino, A Oliviero, G Pipilas, DC Carbucicchio, C Compagnucci, P Dello Russo, A Olivotto, I Calo, L Lubitz, SA Cutler, MJ Chevalier, P Arbelo, E Priori, SG Healey, JS Calkins, H Casella, M Jensen, HK Tondo, C Tadros, R James, CA Krahn, AD Cadrin-Tourigny, J Arrhythmogenic right ventricular cardiomyopathy Implantable cardioverter-defibrillator Sudden cardiac death Ventricular arrhythmias Risk stratification Genetic cardiomyopathies Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. Methods and results In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05-7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65-0.75)] and calibration slope of 1.01 (95% CI 0.99-1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Conclusion Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC. OXFORD UNIV PRESS https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12005 https://ddd.uab.cat/record/282315 |
| spellingShingle | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator Jorda, P Arrhythmogenic right ventricular cardiomyopathy Implantable cardioverter-defibrillator Sudden cardiac death Ventricular arrhythmias Risk stratification Genetic cardiomyopathies |
| status_str | publishedVersion |
| title | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator |
| title_full | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator |
| title_fullStr | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator |
| title_full_unstemmed | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator |
| title_short | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator |
| title_sort | Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator |
| topic | Arrhythmogenic right ventricular cardiomyopathy Implantable cardioverter-defibrillator Sudden cardiac death Ventricular arrhythmias Risk stratification Genetic cardiomyopathies |
| topic_facet | Arrhythmogenic right ventricular cardiomyopathy Implantable cardioverter-defibrillator Sudden cardiac death Ventricular arrhythmias Risk stratification Genetic cardiomyopathies |
| url | https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=12005 https://ddd.uab.cat/record/282315 |