July 20, 2012Clinical Research in Cardiology59 citationsOpen Access

Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome

HSHeike E. Schneider MSMichael Steinmetz UKUlrich Krause

Structured PICO

Does left cardiac sympathetic denervation reduce arrhythmia burden in young patients with CPVT and LQTS who are refractory to beta-blocker therapy?

Population

10 young patients (mean age 14.0 years, 70% male) with catecholaminergic polymorphic ventricular tachycardia (CPVT, n=5) or long QT syndrome (LQTS, n=5) who experienced recurrent syncope, symptomatic ventricular tachycardias, internal cardioverter-defibrillator (ICD) discharges, or aborted cardiac arrest despite high doses of beta-blockers.

Intervention

Transaxillary left cardiac sympathetic denervation (LCSD) added to continued medical therapy (beta-blockers).

Outcome

Reduction in arrhythmia burden and severe cardiac events (syncope, ventricular tachycardia, ICD discharges, aborted cardiac arrest).hard clinical

Transaxillary left cardiac sympathetic denervation safely and effectively reduces arrhythmia burden and ICD discharges in young patients with CPVT and LQTS who remain symptomatic despite high-dose beta-blocker therapy.

Limitations

Retrospective design
Limited patient number secondary to the rare prevalence of these diseases
Quantitative marker of arrhythmia burden has been difficult to express in statistically comparable data due to the large variability in presenting symptoms

Abstract

BACKGROUND: Left cardiac sympathetic denervation (LCSD) may be a therapeutic adjunct for young patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) and long QT syndrome (LQTS) who are not fully protected by beta-blockade. OBJECTIVE: The objective of this analysis was to report our institutional experience with LSCD in young patients for the management of life-threatening ventricular arrhythmias in CPVT and LQTS. METHODS: Ten young patients with CPVT and LQTS underwent transaxillary LSCD at our institution. Mean age at surgery was 14.0 (range 3.9-42) years, mean body weight was 45.7 (range 15.5-90) kg. Five patients had the clinical diagnosis of CPVT, three were genotype positive for a mutation in the ryanodine-receptor-2-gene. Four of five LQTS patients were genotype positive. Indications for LCSD were recurrent syncope, symptomatic episodes of ventricular tachycardias and/or internal cardioverter-defibrillator (ICD) discharges, and aborted cardiac arrest despite high doses of beta-blockers. RESULTS: LCSD was performed via the transaxillary approach. No significant complications were observed. Two patients already had an ICD, 6 patients received an ICD at the same operation or shortly thereafter. Median length of follow-up after LCSD was 2.3 (range 0.6-3.9) years. After LCSD a marked reduction in arrhythmia burden and cardiac events was observed in all patients while medication was continued. None of the patients had any further ICD discharge for sustained VT. CONCLUSIONS: After LCSD, arrhythmia burden could significantly be reduced in all our young patients with CPVT and LQTS.

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