Surgical Cardiac Denervation Therapy for Treatment of Congenital Ion Channelopathies in Pediatric Patients: A Contemporary, Single Institutional Experience. (January 2015)

• Record Type: Journal Article
• Authors: Costello, John P.; Wilson, Jennifer K.; Louis, Clauden; Peer, Syed M.; Zurakowski, David; Nadler, Evan P.; Qureshi, Faisal G.; Jonas, Richard A.; Greene, E. Anne; Berul, Charles I.; Moak, Jeffrey P.; Nath, Dilip S.
• Is Part Of: World journal for pediatric & congenital heart surgery. Volume 6:Number 1(2015:Jan.)
• Page Start: 33
• Page End: 38
• Publication Date: 2015-01
• Subjects: congenital long QT syndrome -- congenital ion channel disorders -- surgical cardiac denervation therapy -- sympathectomy -- stellate ganglionectomy; Pediatric cardiology -- Periodicals; Congenital heart disease in children -- Periodicals; Heart -- Abnormalities -- Surgery -- Periodicals; Heart -- Surgery -- Periodicals; Heart Defects, Congenital -- surgery -- Periodicals; Cardiac Surgical Procedures -- Periodicals; Child -- Periodicals; Adult -- Periodicals; 618.9212
• DOI: 10.1177/2150135114555203
• Languages: English
• ISSNs: 2150-1351

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.