Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator
Por:
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
Publicada:
21 ago 2022
Ahead of Print:
1 jun 2022
Resumen:
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.
Filiaciones:
Jorda, P:
Univ Montreal, Cardiovasc Genet Ctr, Montreal Heart Inst, Montreal, PQ, Canada
Univ Barcelona, Hosp Clin, Cardiol Dept, Arrhythmia Sect, Barcelona, Spain
Inst Invest August Pi & Sunyer IDIBAPS, Barcelona, Spain
Bosman, LP:
Univ Utrecht, Univ Med Ctr Utrecht, Dept Cardiol, Div Heart & Lungs, Utrecht, Netherlands
Gasperetti, A:
Johns Hopkins Univ Hosp, Dept Med, Div Cardiol, Baltimore, MD 21287 USA
Mazzanti, A:
Univ Pavia, Dept Mol Med, Pavia, Italy
IRCCS Ist Clin Sci Maugeri, Dept Mol Cardiol, Pavia, Italy
Ctr Nacl Invest Cardiovasc, Dept Mol Cardiol, Madrid, Spain
Gourraud, JB:
CHU Nantes, Dept Cardiol, Nantes, France
Davies, B:
Univ British Columbia, Ctr Cardiovasc Innovat, Div Cardiol, Vancouver, BC, Canada
Frederiksen, TC:
Aarhus Univ Hosp, Dept Cardiol, Aarhus N, Denmark
Aarhus Univ, Dept Clin Med, Aarhus N, Denmark
Weidmann, ZM:
Univ Autonoma Barcelona, Dept Cardiol, CIBERCV, Hosp Santa Creu & St Pau,IIB St Pau, Barcelona, Spain
Di Marco, A:
Hosp Univ Bellvitge, Dept Cardiol, Arrhythmia Unit, Barcelona, Spain
Roberts, JD:
McMaster Univ, Populat Hlth Res Inst, Hamilton, ON, Canada
Hamilton Hlth Sci, Div Cardiol, Hamilton, ON, Canada
Western Univ, Dept Med, Div Cardiol, Sect Cardiac Electrophysiol, London, ON, Canada
MacIntyre, C:
Dalhousie Univ, Quenn Elisabeth II Hlth Sci Ctr, Cardiac Electrophysiol Serv, Halifax, NS, Canada
Seifer, C:
Univ Manitoba, St Boniface Hosp, Winnipeg, MB, Canada
Deliniere, A:
Hosp Civils Lyon, Louis Pradel Cardiovasc Hosp, Natl Reference Ctr Inherited Arrhythmias Lyon, Lyon, France
Alqarawi, W:
Univ Ottawa, Ottawa Heart Inst, Cardiac Electrophysiol Serv, Ottawa, ON, Canada
Kukavica, D:
Univ Pavia, Dept Mol Med, Pavia, Italy
IRCCS Ist Clin Sci Maugeri, Dept Mol Cardiol, Pavia, Italy
Ctr Nacl Invest Cardiovasc, Dept Mol Cardiol, Madrid, Spain
Minois, D:
CHU Nantes, Dept Cardiol, Nantes, France
Trancuccio, A:
Univ Pavia, Dept Mol Med, Pavia, Italy
IRCCS Ist Clin Sci Maugeri, Dept Mol Cardiol, Pavia, Italy
Ctr Nacl Invest Cardiovasc, Dept Mol Cardiol, Madrid, Spain
Arnaud, M:
CHU Nantes, Dept Cardiol, Nantes, France
Targetti, M:
Careggi Univ Hosp, Dept Cardiol, Cardiomyopathy Unit, Florence, Italy
Martino, A:
Policlin Casilino, Dept Cardiol, Rome, Italy
Oliviero, G:
Policlin Casilino, Dept Cardiol, Rome, Italy
Pipilas, DC:
Massachusetts Gen Hosp, Cardiovasc Res Ctr, Boston, MA 02114 USA
Massachusetts Gen Hosp, Cardiac Arrhythmia Serv, Boston, MA 02114 USA
Carbucicchio, C:
IRCCSC, Dept Clin Electrophysiol, Milan, Italy
IRCCSC, Cardiac Pacing Ctr Cardiol Monzino, Milan, Italy
Compagnucci, P:
Marche Polytech Univ, Univ Hosp Umberto Salesi Lancisi, Dept Biomed Sci & Publ Hlth, Cardiol & Arrhythmol Clin, Ancona, Italy
Dello Russo, A:
Marche Polytech Univ, Univ Hosp Umberto Salesi Lancisi, Dept Biomed Sci & Publ Hlth, Cardiol & Arrhythmol Clin, Ancona, Italy
Olivotto, I:
Careggi Univ Hosp, Dept Cardiol, Cardiomyopathy Unit, Florence, Italy
Calo, L:
Policlin Casilino, Dept Cardiol, Rome, Italy
Lubitz, SA:
Massachusetts Gen Hosp, Cardiovasc Res Ctr, Boston, MA 02114 USA
Massachusetts Gen Hosp, Cardiac Arrhythmia Serv, Boston, MA 02114 USA
Cutler, MJ:
Intermt Med Ctr, Heart Inst, Murray, UT USA
Chevalier, P:
Hosp Civils Lyon, Louis Pradel Cardiovasc Hosp, Natl Reference Ctr Inherited Arrhythmias Lyon, Lyon, France
Arbelo, E:
Univ Barcelona, Hosp Clin, Cardiol Dept, Arrhythmia Sect, Barcelona, Spain
Inst Invest August Pi & Sunyer IDIBAPS, Barcelona, Spain
Ctr Invest Biomed Red Enfermedades Cardiovasc CIB, Madrid, Spain
European Reference Network Rare Low Prevalence &, Madrid, Spain
Priori, SG:
Univ Pavia, Dept Mol Med, Pavia, Italy
IRCCS Ist Clin Sci Maugeri, Dept Mol Cardiol, Pavia, Italy
Ctr Nacl Invest Cardiovasc, Dept Mol Cardiol, Madrid, Spain
Healey, JS:
McMaster Univ, Populat Hlth Res Inst, Hamilton, ON, Canada
Hamilton Hlth Sci, Div Cardiol, Hamilton, ON, Canada
Calkins, H:
Johns Hopkins Univ Hosp, Dept Med, Div Cardiol, Baltimore, MD 21287 USA
Casella, M:
MarchePolytech Univ, Univ Hosp Umberto Salesi Lancisi, Dept Clin, Cardiol & Arrhythmol Clin, Ancona, Italy
Jensen, HK:
Aarhus Univ Hosp, Dept Cardiol, Aarhus N, Denmark
Aarhus Univ, Dept Clin Med, Aarhus N, Denmark
Tondo, C:
IRCCSC, Dept Clin Electrophysiol, Milan, Italy
IRCCSC, Cardiac Pacing Ctr Cardiol Monzino, Milan, Italy
Univ Milan, Dept Biomed Surg & Dent Sci, Milan, Italy
Tadros, R:
Univ Montreal, Cardiovasc Genet Ctr, Montreal Heart Inst, Montreal, PQ, Canada
James, CA:
Johns Hopkins Univ Hosp, Dept Med, Div Cardiol, Baltimore, MD 21287 USA
Krahn, AD:
Univ British Columbia, Ctr Cardiovasc Innovat, Div Cardiol, Vancouver, BC, Canada
Cadrin-Tourigny, J:
Univ Montreal, Cardiovasc Genet Ctr, Montreal Heart Inst, Montreal, PQ, Canada
hybrid, All Open Access, Hybrid Gold
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