Por favor, use este identificador para citar o enlazar este Item:http://hdl.handle.net/20.500.12105/15637
Título
Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With β-Blockers.
Autor(es)
Mazzanti, Andrea | Kukavica, Deni | Trancuccio, Alessandro | Memmi, Mirella | Bloise, Raffaella | Gambelli, Patrick | Marino, Maira | Ortíz-Genga, Martín | Morini, Massimo | Monteforte, Nicola | Giordano, Umberto | Keegan, Roberto | Tomasi, Luca | Anastasakis, Aristides | Davis, Andrew M | Shimizu, Wataru | Blom, Nico A | Santiago, Demetrio Julián | Napolitano, Carlo | Monserrat, Lorenzo | Priori, Silvia G. CNIC
Fecha de publicación
2022-05-01
Cita
JAMA Cardiol. 2022 May 1;7(5):504-512
Idioma
Inglés
Tipo de documento
journal article
Resumen
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 < .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).
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.
MESH
Nadolol | Tachycardia, Ventricular | Adolescent | Adrenergic beta-Antagonists | Adult | Child | Cohort Studies | Electrocardiography | Female | Humans | Male | Prospective Studies | Ryanodine Receptor Calcium Release Channel | Syncope | Young Adult
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