Clinical management of catecholaminergic polymorphic ventricular tachycardia: The role of left cardiac sympathetic denervation.

BACKGROUND: -Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. Β-blockers are the mainstay of therapy; when they fail, implantable cardioverter defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. METHODS AND RESULTS: -We report on 63 CPVT patients who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events (MCEs). Of the 54 patients with prior MCEs either on (n=38) or off (n=16) optimal medical therapy (OMT), 13 (24%) had at least 1 recurrence: none had an aborted cardiac arrest, 2 had syncope only, 10 had ≥1 appropriate ICD discharge and one died suddenly. The 1- and 2-year cumulative event-free survivals were 87% and 81%. The percentage of patients with MCEs despite OMT (n=38) was reduced from 100% to 32% (p<0.001) following LCSD and, among 29 patients with a pre-surgical ICD, the rate of shocks dropped by 93%, from 3.6 to 0.6 shocks/person/year (p<0.001). Patients with an incomplete LCSD (n=7) were more likely to suffer MCEs post-LCSD (71% vs 17%, p<0.01) than those with a complete LCSD. CONCLUSIONS: -LCSD is an effective anti-fibrillatory intervention for patients with CPVT. Whenever syncope occurs despite OMT, LCSD could be considered as the next step rather than an ICD and could complement ICDs in patients with recurrent shocks