Left cardiac sympathetic denervation in the Netherlands for the treatment of inherited arrhythmia syndromes

L R A Olde Nordkamp,N A Blom,A Odero,D R Koolbergen,P J Schwartz,A A M Wilde,A H G Driessen

doi:10.1007/s12471-014-0523-2

Abstract

IntroductionTreating therapy-resistant patients with inherited arrhythmia syndromes can be difficult and left cardiac sympathetic denervation (LCSD) might be a viable alternative treatment option. We provide an overview of the indications and outcomes of LCSD in patients with inherited arrhythmia syndromes in the only tertiary referral centre in the Netherlands where LCSD is conducted in these patients.MethodsThis was a retrospective study, including all patients with inherited arrhythmia syndromes who underwent LCSD in our institution between 2005 and 2013. LCSD involved ablation of the lower part of the left stellate ganglion and the first four thoracic ganglia.ResultsSeventeen patients, 12 long-QT syndrome (LQTS) patients (71 %) and 5 catecholaminergic polymorphic ventricular tachycardia (CPVT) patients (29 %), underwent LCSD. Most patients (94 %) were referred because of therapy-refractory cardiac events. In 87 % the annual cardiac event rate decreased. However, after 2 years the probability of complete cardiac event-free survival was 59 % in LQTS and 60 % in CPVT patients. Two patients (12 %) had major non-reversible LCSD-related complications: one patient suffered from a Harlequin face post-procedure and one severely affected LQT8 patient died the day after LCSD due to complications secondary to an arrhythmic storm during the procedure.ConclusionLSCD for inherited arrhythmia syndromes, which is applied on a relatively small scale in the Netherlands, reduced the cardiac event rate in 87 % of the high-risk patients who had therapy-refractory cardiac events, while the rate of major complications was low. Therefore, LSCD seems a viable treatment for patients with inherited arrhythmia syndromes without other options for therapy.

Highlights

Treating therapy-resistant patients with inherited arrhythmia syndromes can be difficult and left cardiac sympathetic denervation (LCSD) might be a viable alternative treatment option
From November 2005 to January 2013, 17 patients (12 long-QT syndrome (LQTS) patients (71 %) and 5 catecholaminergic polymorphic ventricular tachycardia (CPVT) patients (29 %)) had a LCSD performed at our institution
Eight patients (47 %), of whom seven were diagnosed with LQTS, had already implantable cardiac defibrillator (ICD) implanted by the time of the surgery and one LQTS patient received an ICD 1 month after LCSD

Summary

Introduction

Treating therapy-resistant patients with inherited arrhythmia syndromes can be difficult and left cardiac sympathetic denervation (LCSD) might be a viable alternative treatment option. Primary inherited arrhythmia syndromes, such as long-QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT), are genetic cardiac diseases without apparent structural heart disease that may cause cardiac syncope and sudden cardiac death, mainly in young individuals. These cardiac events are generally induced by physical or emotional stress triggers [1,2,3]. An implantable cardiac defibrillator (ICD) is often used in patients who continue to have ventricular arrhythmias despite β-blocker therapy [5, 6]. The cost of frequent shocks in terms of pain and fear is substantial [8] and young patients with ICDs are more likely to experience device complications, including inappropriate shocks and lead-related complications, over many years of use [9]

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