Video-Assisted Thoracoscopic Left Cardiac Sympathetic Denervation in Patients with Hereditary Ventricular Arrhythmias.

BACKGROUND: Left cardiac sympathetic denervation (LCSD) has been underutilized in patients with hereditary ventricular arrhythmia syndromes such as congenital long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). The purpose of this study was to investigate the safety and efficacy of video-assisted thoracoscopic (VATS) LCSD in such patients. METHODS: Fifteen patients (four men, 24.6 ± 10.5 years old) who underwent VATS-LCSD between November 2010 and January 2015 for hereditary ventricular arrhythmia syndromes at Kyungpook National University Hospital were enrolled in this study. The safety and efficacy of VATS-LCSD were evaluated by periprocedural epinephrine tests and assessing the development of complications and cardiac events during follow-up. RESULTS: Fourteen patients with LQTS and one patient with CPVT underwent VATS-LCSD. Six and one patients developed ventricular tachyarrhythmia during preprocedural and postprocedural epinephrine test, respectively (P = 0.063). No serious complications such as Horner syndrome, pneumothorax, or bleeding developed after LCSD. Mean hospital stay after VATS-LCSD was 3.7 ± 1.5 days. During a mean follow-up of 927 ± 350 days, one LQTS patient and one CPVT patient, neither of whom manifested tachyarrhythmia during post-LCSD epinephrine test, developed torsades de pointes and syncope, respectively. The annual event rates of six patients who were symptomatic during the period preceding LCSD decreased from 0.97 to 0.19 events/year (P = 0.045). CONCLUSIONS: VATS-LCSD was a safe, and effective procedure for patients with hereditary ventricular tachycardia syndrome, with no serious adverse events and with short hospital stay.

Surgical cardiac denervation therapy for treatment of congenital ion channelopathies in pediatric patients: a contemporary, single institutional experience.

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.

Multivalvular Replacement and Ventricular Arrhythmias in a Female Child With Congenital Polyvalvular Disease.

We report the clinical course of a female child with a normal karyotype and chromosomal microarray who presented as an infant with clinical findings consistent with congenital polyvalvular disease (CPVD). This clinical entity describes patients with multiple congenitally dysplastic valves, often showing nodular or cystic malformation in at least two cardiac valves. This patient then developed medically refractory multifocal ventricular arrhythmia and required radiofrequency ablation at seven months of age. She had good tachycardia control but became symptomatic with right heart failure related to progressive tricuspid, pulmonary, and mitral valve dysfunction necessitating multivalvular replacement at 21 months of age.

Anesthesia for videoscopic left cardiac sympathetic denervation in children with congenital long QT syndrome and catecholaminergic polymorphic ventricular tachycardia--a case series.

OBJECTIVE: To describe our experience in the anesthetic management of pediatric patients who have undergone left cardiac sympathetic denervation (LCSD) for congenital long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). BACKGROUND: Long QT syndrome and CPVT predispose patients to ventricular arrhythmias and sudden death. One treatment option for these patients is LCSD. When these patients present for LCSD or other surgical procedures, anesthetic management is challenging, as many medications may exacerbate QT prolongation. METHODS: Retrospective review of the electronic medical records of 22 pediatric patients who underwent LCSD between November 2005 and December 2008. RESULTS: Six patients (27%) received midazolam as a premedication. Eleven patients (50%) underwent inhalation induction with sevoflurane. Eighty-six percentage received either sevoflurane or isoflurane for maintenance of anesthesia, while the remaining 14% received a propofol infusion. Nine patients (41%) received esmolol infusions intraoperatively, while one patient (4.5%) received a labetalol infusion. Three patients (14%) received lidocaine infusions. no significant cardiac or other events occurred in any of these patients in the perioperative period. CONCLUSIONS: Important anesthetic considerations in this population include avoidance of sympathetic stimulation, correction of any abnormal electrolytes, and the immediate availability of a defibrillator and magnesium sulfate to treat arrhythmias. Anxious patients may benefit from premedication to reduce sympathetic tone. We have safely used both volatile agents and propofol for induction and maintenance of anesthesia. In our experience, intraoperative infusions of beta-blockers and lidocaine seem to be helpful in reducing arrhythmogenic potential, especially in patients with profound QT prolongation.