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Circulation. 2015 Jun 23;131(25):2185-93. doi: 10.1161/CIRCULATIONAHA.115.015731. Epub 2015 May 27.

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia: The Role of Left Cardiac Sympathetic Denervation.

Author information

1
From Department of Cardiology and Cardiovascular Clinical Research Center (G.M.D.F., V.D.) and Division of Vascular Surgery (A.O.), Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Italy (G.M.D.T., V.D., C.S., L.C.); Center for Cardiac Arrhythmias of Genetic Origin, IRCCS Istituto Auxologico Italiano, Milano, Italy (C.S., L.C., P.J.S.); Departments of Medicine, Pediatrics, and Molecular Pharmacology & Experimental Therapeutics, Divisions of Cardiovascular Diseases and Pediatric Cardiology, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, MN (J.M.B., C.R.M., M.J.A.); Cardiac Arrhythmia Service, Department of Cardiology, Children's Hospital, Boston, MA (D.J.A.); Division of Cardiology, Children's National Heart Institute, George Washington University, Washington, DC (C.I.B.); Institute of Human Genetics, Helmholtz Zentrum München, Neuherberg, Germany (L.C.); Department of Cardiology, The Royal Children's Hospital, Murdoch Children's Research Institute and Melbourne University, Melbourne, Australia (A.M.D.); Heart Institute, Leviev Heart Center, Sheba Medical Center, Sackler School of Medicine, Tel Aviv University, Tel Hashomer, Israel (M.E.); Department of Clinical Cardiology and Molecular Genetics, National Center for Preventive Medicine, Ministry of Healthcare, Russian Federation, Moscow, Russia (M.K.); Bruce Rappaport Faculty of Medicine; Technion, Haifa, Israel (A.K.); Division of Cardiology, University of British Columbia, Vancouver, Canada (A.D.K.); AP-HP, Hôpital Bichat, Service de Cardiologie et Centre de Référence des Maladies Cardiaques Héréditaires, Paris, France (A.L.); Université Paris Diderot, Sorbonne Paris Cité, Paris, France (A.L.); Heart Centre AMC, Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands (L.O.N., A.A.M.W.); Department of Pediatric Cardiology and Intensive Care Medicine, University Hospital, Georg-August-University,

Abstract

BACKGROUND:

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers 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.

KEYWORDS:

adrenergic beta-antagonists; arrhythmias, cardiac; death, sudden; genetics; sympathetic nervous system

[Indexed for MEDLINE]

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