Original Article Surgical Cardiac Denervation Therapy for Treatment of Congenital Ion Channelopathies in Pediatric Patients: A Contemporary, Single Institutional Experience John P. Costello, MD 1,2 , Jennifer K. Wilson 1 , Clauden Louis, BS 1 , Syed M. Peer, MD 1 , David Zurakowski, PhD 3 , Evan P. Nadler, MD 2,4 , Faisal G. Qureshi, MD 4 , Richard A. Jonas, MD 1 , E. Anne Greene, MD 5 , Charles I. Berul, MD 5 , Jeffrey P. Moak, MD 5 , and Dilip S. Nath, MD 1 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. Keywords congenital long QT syndrome, congenital ion channel disorders, surgical cardiac denervation therapy, sympathectomy, stellate ganglionectomy Submitted June 02, 2014; Accepted September 18, 2014. Introduction Congenital long QT syndrome (LQTS) is a primary cardiac arrhythmia with an estimated prevalence of 1 in 2000 patients. 1 It is characterized by a prolonged QT interval that exceeds 450 ms in preadolescent children, exceeds 460 ms in postpubertal males, and is greater than 470 ms in postpubertal females, all in the setting of a structurally normal heart 2 ; symptoms do not have to be present for the diagnosis to be made. 3 Even without precursor symptoms, congenital LQTS can result in the devel- opment of torsades de pointes and ventricular fibrillation, arrhythmias that can be fatal even on initial occurrence. 1 Although the first line of treatment is medication therapy 1 Division of Cardiovascular Surgery, Children’s National Health System, Washington, DC, USA 2 The Sheikh Zayed Institute for Pediatric Surgical Innovation, Children’s National Health System, Washington, DC, USA 3 Departments of Anesthesia and Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA 4 Department of Pediatric Surgery, Children’s National Health System, Washington, DC, USA 5 Department of Cardiology, Children’s National Health System, Washington, DC, USA Corresponding Author: Dilip S. Nath, Division of Cardiovascular Surgery, Children’s National Health System, 111 Michigan Avenue, NW, Washington, DC, 20010, USA. Email: dnath@childrensnational.org World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(1) 33-38 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114555203 pch.sagepub.com by guest on June 22, 2016 pch.sagepub.com Downloaded from