PO-01-134 AN INTERNATIONAL MULTICENTER COHORT STUDY ON BETA-BLOCKER FREE TREATMENT STRATEGIES FOR CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR TACHYCARDIA

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare potentially life-threatening genetic heart disease. Non-selective beta-blockers (BBs, namely nadolol and propranolol) are highly effective in reducing CPVT-triggered arrhythmic events. However, some patients suffer from unacceptable BB side effects might require BB-free treatment strategies that include flecainide, left cardiac sympathetic denervation (LCSD), combination therapy, or even intentional non-therapy (INT) as has been published for long QT syndrome. To review the spectrum of outcomes associated with configurations CPVT International Registry. From Registry, all RYR2 variant-positive treated strategy ≥ 6 months were included. Four groups defined: INT, flecainide monotherapy, LCSD LCSD/flecainide therapy. Overall, 100 out 1017 (10%) on (53 females (53%), mean age at therapy initiation 24 ± 22 years). There 73 (42 males 31 females) INT group. For majority these (n=62; 88%), was pursued following low-risk assessment asymptomatic absent negligible stress test phenotype (characterized by absence bigeminy, couplets, more complex ectopy). Twenty-one monotherapy intolerance main reason 14 21 (67%). Two alone because intolerance. Lastly, used 4 patients. During follow-up years patients, only 2 had self-limiting syncopal episode while after which restarted. Though non-selective BBs remain cornerstone either 10% required side-effects. After careful risk-assessment, safe can be configured without an implantable cardioverter defibrillator such