Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With β-Blockers.

Autor:	Mazzanti A; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy.; Department of Molecular Medicine, University of Pavia, Pavia, Italy.; ERN Guard-Heart European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart.; Department of Molecular Cardiology, Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain., Kukavica D; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy.; Department of Molecular Medicine, University of Pavia, Pavia, Italy.; Department of Molecular Cardiology, Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain., Trancuccio A; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy.; Department of Molecular Medicine, University of Pavia, Pavia, Italy.; Department of Molecular Cardiology, Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain., Memmi M; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy., Bloise R; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy., Gambelli P; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy., Marino M; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy., Ortíz-Genga M; Health in Code, A Coruña, Spain., Morini M; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy., Monteforte N; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy., Giordano U; Department of Cardiology, ARNAS Civico Cristina Di Benefratelli, Palermo, Italy., Keegan R; Department of Electrophysiology, Hospital Privado Del Sur, Buenos Aires, Argentina., Tomasi L; Department of Cardiology, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy., Anastasakis A; Unit of Inherited and Rare Cardiovascular Diseases, Onassis Cardiac Surgery Center, Athens, Greece., Davis AM; Department of Cardiology, The Royal Children's Hospital, Melbourne, Australia.; Department of Pediatrics, University of Melbourne, Melbourne, Australia.; Murdoch Children's Research Institute, Melbourne, Australia., Shimizu W; Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan., Blom NA; Department of Pediatric Cardiology, University of Leiden, Leiden, the Netherlands., Santiago DJ; Department of Molecular Cardiology, Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain., Napolitano C; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy.; Department of Molecular Medicine, University of Pavia, Pavia, Italy.; ERN Guard-Heart European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart., Monserrat L; Health in Code, A Coruña, Spain., Priori SG; Department of Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy.; Department of Molecular Medicine, University of Pavia, Pavia, Italy.; ERN Guard-Heart European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart.; Department of Molecular Cardiology, Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain.
Jazyk:	angličtina
Zdroj:	JAMA cardiology [JAMA Cardiol] 2022 May 01; Vol. 7 (5), pp. 504-512.
DOI:	10.1001/jamacardio.2022.0219
Abstrakt:	Importance: 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. Objective: To investigate the long-term outcomes of patients with RYR2 CPVT treated with β-blockers only and the cost to benefit ratio of ICD. Design, Settings, and Participants: 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. Exposures: Treatment with selective and nonselective β-blocker only and ICD implant when indicated. Main Outcome and Measures: 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. Results: 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 < .001) were associated with an increased LTAE risk during β-blocker therapy only. The risk of LTAE among those taking selective β-blockers vs nadolol was increased 6-fold (HR, 5.8; 95% CI, 2.1-16.3; P = .001). Conversely, no significant difference was present between propranolol and nadolol (HR, 1.8; 95% CI, 0.4-7.3; P = .44). An ICD was implanted in 79 of 216 patients (37%) who were followed up for a mean (SD) of 8.6 (6.3) years. At the occurrence of LTAE, ICD carriers were more likely to survive (18 of 18 [100%]) than non-ICD carriers (6 of 10 [60%]; P = .01). Conclusions and Relevance: In this cohort study, selective β-blockers were associated with a higher risk of LTAE as compared with nadolol. Independently from treatment, LTAE and syncope before diagnosis and C-terminal domain variants identified patients at higher risk of β-blocker failure, and the ICD was associated with reduced mortality in high-risk patients with CPVT.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu