Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With β-Blockers
| Autor: | Andrea Mazzanti, Deni Kukavica, Alessandro Trancuccio, Mirella Memmi, Raffaella Bloise, Patrick Gambelli, Maira Marino, Martín Ortíz-Genga, Massimo Morini, Nicola Monteforte, Umberto Giordano, Roberto Keegan, Luca Tomasi, Aristides Anastasakis, Andrew M. Davis, Wataru Shimizu, Nico A. Blom, Demetrio Julián Santiago, Carlo Napolitano, Lorenzo Monserrat, Silvia G. Priori |
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| Přispěvatelé: | Pediatrics, ACS - Heart failure & arrhythmias, Paediatric Cardiology, Italian Ministry of Health, Italian Ministry of Research and University, University of Pavia |
| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: | |
| Zdroj: | JAMA Cardiol JAMA cardiology, 7(5), 504-512. American Medical Association JAMA Cardiology, 7(5), 504-512. AMER MEDICAL ASSOC Mazzanti, A, Kukavica, D, Trancuccio, A, Memmi, M, Bloise, R, Gambelli, P, Marino, M, Ortíz-Genga, M, Morini, M, Monteforte, N, Giordano, U, Keegan, R, Tomasi, L, Anastasakis, A, Davis, A M, Shimizu, W, Blom, N A, Santiago, D J, Napolitano, C, Monserrat, L & Priori, S G 2022, ' Outcomes of Patients with Catecholaminergic Polymorphic Ventricular Tachycardia Treated with β-Blockers ', JAMA cardiology, vol. 7, no. 5, pp. 504-512 . https://doi.org/10.1001/jamacardio.2022.0219 |
| ISSN: | 2380-6583 |
| Popis: | Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) may experience life-threatening arrhythmic events (LTAEs) despite β-blocker treatment. Further complicating management, the role of implantable cardioverter defibrillator (ICD) in CPVT is debated. To investigate the long-term outcomes of patients with RYR2 CPVT treated with β-blockers only and the cost to benefit ratio of ICD. This prospective cohort study conducted from January 1988 to October 2020 with a mean (SD) follow-up of 9.4 (7.5) years included patients who were referred to the Molecular Cardiology Clinics of ICS Maugeri Hospital, Pavia, Italy. Participants included consecutive patients with CPVT who were carriers of a pathogenic or likely pathogenic RYR2 variant with long-term clinical follow-up. Treatment with selective and nonselective β-blocker only and ICD implant when indicated. The main outcome was the occurrence of the first LTAE while taking a β-blocker. LTAE was defined as a composite of 3 hard end points: sudden cardiac death, aborted cardiac arrest, and hemodynamically nontolerated ventricular tachycardia. The cohort included 216 patients with RYR2 CPVT (121 of 216 female [55%], median [IQR] age 14, [9-30] years). During a mean (SD) follow-up of 9.4 (7.5) years taking β-blockers only, 28 of 216 patients (13%) experienced an LTAE (annual rate, 1.9%; 95% CI, 1.3-2.7). In multivariable analysis, experiencing either an LTAE (hazard ratio [HR], 3.3; 95% CI, 1.2-8.9; P = .02) or syncope before diagnosis (HR, 4.5; 95% CI, 1.8-11.1; P = .001) and carrying a C-terminal domain variant (HR, 18.1; 95% CI, 4.1-80.8; P |
| Databáze: | OpenAIRE |
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