Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a heritable or passed down disease in which abnormal and life-threatening heart rhythms occur during exercise or heightened emotion (such as anger or excitement). Patients with this condition are often treated with beta-blocker medication but that appears not always sufficient. The aim of this study was to develop a model for individualized prediction or specific to the person, of Arrhythmic Events in patients with RYR2-mediated CPVT on β-blocker monotherapy.
The group in which we studied consisted of 743 patients with CPVT (all included in the international CPVT registry) and a second group in which the outcome was validated (known) consisted of 129 patients (pediatric patients included in an international registry mainly from the US and Canada). Arrhythmic events were defined as passing out due to an arrhythmia (i.e. arrhythmic syncope), appropriate shock of an implantable cardioverter (i.e. correct intervention of the defibrillator), sudden cardiac arrest (SCA), and sudden cardiacdeath. Near-fatal or fatal arrhythmic events included all arrhythmic events except for arrhythmic syncope.
A total of 102 (13.7%) patients in the large group and 24 (18.6%) patients in the validation group experienced one or more arrhythmic events over a median follow-up of a little over 5 and 2.5 years, respectively. Predictors of arrhythmic events were arrhythmic syncope or SCA prior to diagnosis and age at β-blocker initiation. Hence, patients who are young (at the moment treatment is started) and presented with arrhythmic syncope prior to diagnosis are at higher risk.
For near fatal or fatal arrhythmic events, ventricular arrhythmia severity before β-blocker initiation was a fourth independent predictor.
Conclusions: These externally validated risk prediction models using clinical parameters (limits or boundaries) accurately distinguished CPVT patients on β- blocker monotherapy (one medication) at low and high risk for future arrhythmic events while treated with β-blockers. These models provide guidance for implementation of clinical management therapies to prevent arrhythmic events in patients with CPVT.
Lieve KV, van der Werf C, Kallas D, Denjoy I, Bos JM, Aiba T, Behr ER, van den Berg MP, Bergeman AT, Blom NA, Borggrefe M, Brugada R, Carrillo Mora LM, Chorin E, Crotti L, Davis A, Drago F, Dusi V, Extramiana F, Franciosi S, Giudicessi JR, González Llopis FÁ, Haugaa KH, van den Heuvel F, Horie M, Ingles J, Kammeraad J, Kannankeril PJ, Khan HR, Krahn AD, MacIntyre C, Maltret A, Marjamaa A, Ohno S, Peltenburg PJ, Perez GJ, Probst V, Roberts JD, Robyns T, Rootwelt-Norberg C, Roses I Noguer F, Roston TM, Rydberg A, Sacher F, Sarquella-Brugada G, Schwartz PJ, Semsarian C, Shimizu W, Starling L, Sumitomo N, Skinner JR, Tavacova T, Tfelt-Hansen J, Till JA, Yap SC, Wada Y, Wangüemert F, Zorio E, Ackerman MJ, Leenhardt A, Sanatani S, Tanck MW, Wilde AA. Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification.
Eur Heart J. 2025 Dec 19:ehaf965. doi: 10.1093/eurheartj/ehaf965. Online ahead of print. PMID: 41416846
Prepared by Arthur Wilde and Lorraine McGlinchey