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
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
DOI: 10.1001/jamacardio.2022.0219
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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