title: Surgical cardiac denervation therapy for treatment of congenital ion channelopathies in pediatric patients: a contemporary, single institutional experience.
abstract: BACKGROUND
  Congenital ion channel disorders, including congenital long QT syndrome (LQTS), cause significant morbidity in pediatric patients. When medication therapy does not control symptoms or arrhythmias, more invasive treatment strategies may be necessary. This study examines our institution's clinical experience with surgical cardiac denervation therapy for management of these arrhythmogenic disorders in children.
  
  
  METHODS
  An institutional review board-approved retrospective review identified ten pediatric patients with congenital ion channelopathies who underwent surgical cardiac denervation therapy at a single institution between May 2011 and April 2014. Eight patients had a diagnosis of congenital LQTS, two patients were diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT). All patients underwent sympathectomy and partial stellate ganglionectomy via video-assisted thoracoscopic surgery (VATS).
  
  
  RESULTS
  Six of the ten patients had documented ventricular arrhythmias preoperatively, and 70% of the patients had preoperative syncope. The corrected QT interval decreased in 75% of patients with LQTS following sympathectomy. Postoperative arrhythmogenic symptoms were absent in 88% of congenital LQTS patients, but both patients with CPVT continued to have symptoms throughout the duration of follow-up. All patients were alive after a median follow-up period of 10 months.
  
  
  CONCLUSIONS
  Surgical cardiac denervation therapy via VATS is a useful treatment strategy for congenital LQTS patients who fail medical management, and its potential benefit in the management of CPVT is unclear. A prospective comparison of the efficacy of surgical cardiac denervation therapy and implantable cardioverter-defibrillator use in congenital ion channelopathies is timely and crucial.
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA The Sheikh Zayed Institute for Pediatric Surgical Innovation, Children's National Health System, Washington, DC, USA.
authors.forename: John P
authors.initials: JP
authors.lastname: Costello
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA.
authors.forename: Jennifer K
authors.initials: JK
authors.lastname: Wilson
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA.
authors.forename: Clauden
authors.initials: C
authors.lastname: Louis
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA.
authors.forename: Syed M
authors.initials: SM
authors.lastname: Peer
authors.affiliation: Departments of Anesthesia and Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
authors.affiliation: The Sheikh Zayed Institute for Pediatric Surgical Innovation, Children's National Health System, Washington, DC, USA Department of Pediatric Surgery, Children's National Health System, Washington, DC, USA.
authors.forename: Evan P
authors.initials: EP
authors.lastname: Nadler
authors.affiliation: Department of Pediatric Surgery, Children's National Health System, Washington, DC, USA.
authors.forename: Faisal G
authors.initials: FG
authors.lastname: Qureshi
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Jonas
authors.affiliation: Department of Cardiology, Children's National Health System, Washington, DC, USA.
authors.forename: E Anne
authors.initials: EA
authors.lastname: Greene
authors.affiliation: Department of Cardiology, Children's National Health System, Washington, DC, USA.
authors.forename: Charles I
authors.initials: CI
authors.lastname: Berul
authors.affiliation: Department of Cardiology, Children's National Health System, Washington, DC, USA.
authors.forename: Jeffrey P
authors.initials: JP
authors.lastname: Moak
authors.affiliation: Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA dnath@childrensnational.org.
authors.forename: Dilip S
authors.initials: DS
authors.lastname: Nath
mesh_terms: D000293:Adolescent; D053447:Channelopathies / Q000151:congenital / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008133:Long QT Syndrome / Q000601:surgery*; D008297:Male; D012189:Retrospective Studies; D013233:Stellate Ganglion / Q000601:surgery*; D013562:Sympathectomy; D017180:Tachycardia, Ventricular / Q000151:congenital / Q000601:surgery*; D020775:Thoracic Surgery, Video-Assisted; D016896:Treatment Outcome; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
keywords: congenital ion channel disorders; congenital long QT syndrome; stellate ganglionectomy; surgical cardiac denervation therapy; sympathectomy