title: Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome.
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.
authors: Department for Pediatric Cardiology and Intensive Care Medicine, Georg-August-University Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany. heike.schneider@med.uni-goettingen.de
authors: Heike E
authors: HE
authors: Schneider
authors: Michael
authors: M
authors: Steinmetz
authors: Ulrich
authors: U
authors: Krause
authors: Thomas
authors: T
authors: Kriebel
authors: Wolfgang
authors: W
authors: Ruschewski
authors: Thomas
authors: T
authors: Paul
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D004554:Electric Countershock / Q000295:instrumentation; D004562:Electrocardiography; D005260:Female; D020022:Genetic Predisposition to Disease; D006321:Heart / Q000294:innervation*; D006801:Humans; D008133:Long QT Syndrome / Q000150:complications / Q000235:genetics / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D009154:Mutation; D010641:Phenotype; D012189:Retrospective Studies; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics; D013562:Sympathectomy; D017180:Tachycardia, Ventricular / Q000150:complications / Q000235:genetics / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D013997:Time Factors; D016896:Treatment Outcome; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
chemical_list: D000319:Adrenergic beta-Antagonists; D000889:Anti-Arrhythmia Agents; D019837:Ryanodine Receptor Calcium Release Channel