Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation

Gaetano M. De Ferrari, Veronica Dusi, Carla Spazzolini, J. Martijn Bos, Dominic J. Abrams, Charles I. Berul, Lia Crotti, Andrew M. Davis, Michael Eldar, Maria Kharlap, Asaad Khoury, Andrew D. Krahn, Antoine Leenhardt, Christopher R. Moir, Attilio Odero, Louise Olde Nordkamp, Thomas Paul, Ferran Rosés I Noguer, Maria Shkolnikova, Jan TillArthur A.M. Wilde, Michael J. Ackerman, Peter J. Schwartz

Research output: Contribution to journalArticle

126 Scopus citations

Abstract

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 63 patients with CPVT 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. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P<0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year (P<0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P<0.01) than those with a complete LCSD. Conclusions-LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks.

Original languageEnglish (US)
Pages (from-to)2185-2193
Number of pages9
JournalCirculation
Volume131
Issue number25
DOIs
StatePublished - 2015

Keywords

  • Adrenergic beta-antagonists
  • Arrhythmias
  • Cardiac
  • Death
  • Genetics
  • Sudden
  • Sympathetic nervous system

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

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    De Ferrari, G. M., Dusi, V., Spazzolini, C., Bos, J. M., Abrams, D. J., Berul, C. I., Crotti, L., Davis, A. M., Eldar, M., Kharlap, M., Khoury, A., Krahn, A. D., Leenhardt, A., Moir, C. R., Odero, A., Nordkamp, L. O., Paul, T., Rosés I Noguer, F., Shkolnikova, M., ... Schwartz, P. J. (2015). Clinical management of catecholaminergic polymorphic ventricular tachycardia the role of left cardiac sympathetic denervation. Circulation, 131(25), 2185-2193. https://doi.org/10.1161/CIRCULATIONAHA.115.015731