Left Cardiac Sympathetic Denervation Monotherapy in Patients With Congenital Long QT Syndrome

Background: Videoscopic left cardiac sympathetic denervation (LCSD) is an effective antifibrillatory, minimally invasive therapy for patients with potentially life-threatening arrhythmia syndromes like long QT syndrome (LQTS). Although initially used primarily for treatment intensification following documented LQTS-associated breakthrough cardiac events while on beta-blockers, LCSD as 1-time monotherapy for certain patients with LQTS requires further evaluation. We are presenting our early experience with LCSD monotherapy for carefully selected patients with LQTS. Methods: Among the 1400 patients evaluated and treated for LQTS, a retrospective review was performed on the 204 patients with LQTS who underwent LCSD at our institution since 2005 to identify the patients where the LCSD served as stand-alone, monotherapy. Clinical data on symptomatic status before diagnosis, clinical, and genetic diagnosis, and breakthrough cardiac events after diagnosis were analyzed to determine efficacy of LCSD monotherapy. Result: Overall, 64 of 204 patients (31%) were treated with LCSD alone (37 [58%] female, mean QTc 466±30 ms, 16 [25%] patients were symptomatic before diagnosis with a mean age at diagnosis 17.3±11.8 years, 5 had [8%] ≥1 breakthrough cardiac event after diagnosis, and mean age at LCSD was 21.1±11.4 years). The primary motivation for LCSD monotherapy was an unacceptable quality of life stemming from beta-blocker related side effects (ie, beta-blocker intolerance) in 56/64 patients (88%). The underlying LQTS genotype was LQT1 in 36 (56%) and LQT2 in 20 (31%). There were no significant LCSD-related surgical complications. With a mean follow-up of 2.7±2.4 years so far, only 3 patients have experienced a nonlethal, post-LCSD breakthrough cardiac event in 180 patient-years. Conclusions: LCSD may be a safe and effective stand-alone therapy for select patients who do not tolerate beta-blockers. However, LCSD is not curative and patient selection will be critical when potentially considering LCSD as monotherapy.

Re-do left cardiac sympathetic denervation (LCSD following breakthrough cardiac events in long qt syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT)

Background Left cardiac sympathetic denervation (LCSD) is an effective anti-fibrillatory, minimally invasive therapy for patients with either LQTS or CPVT. As a pre-ganglionic fiber resection, re-growth or re-innervation is not possible. However, if a suboptimal CSD was performed, chances for post-LCSD breakthrough cardiac events (BCEs) are increased. Purpose To examine the prevalence and outcomes of patients requiring a re-do LCSD. Methods We performed a retrospective review of the 251 LQTS and CPVT patients who underwent CSD (mostly LCSD) at our institution to identify the subset referred for additional CSD because of recurrent BCEs after their primary LCSD that was performed elsewhere. Clinical data on symptomatic status prior to diagnosis and BCEs after first surgery as well as the surgical reports were reviewed to determine the reasons for the repeat procedure. Results Among the 15 patients (6% overall, 6 female; 13 LQTS, 2 CPVT) referred for additional CSD, 3 patients (20%) had a re-do LCSD performed instead of a sequential RCSD because of incomplete resection with the primary LCSD. Patient 1 is a male with Jervell-and-Lange Nielsen Syndrome (JLNS; KCNQ1-K421fs9X; KCNQ1-N365H), who first experienced syncope with torsades at age 3 after which a primary bilateral CSD was performed elsewhere. He had 2 BCEs and at age 12 underwent re-do LCSD, where his left-sided T2-T4 sympathetic chain with remnants of T1 were found to be intact. Patient 2 is a male who had 3 cardiac events prior to diagnosis (genotype negative LQTS) and LCSD at age 3. Following 3 BCEs, he underwent re-do LCSD at age 5 where scarring of T3 and intact stellate ganglion were found. Patient 3 is an adult female with CPVT (RYR2-deletion exon 3) who had multiple syncopal events, out-of-hospital arrest (leading to ICD) and 2 ICD-storms before primary bilateral CSD. Re-do LCSD was performed after identifying an intact left stellate ganglion and T2. Conclusions Before performing a primary bilateral CSD, proceeding to a RCSD after post-LCSD BCEs, or doing a videoscopic LCSD in the first place, it must be recognized that LCSD's success hinges on fully resecting the lower half of the stellate ganglion and the sympathetic chain through T4. Anything less is a partial denervation and likely ineffective procedure. Funding Acknowledgement Type of funding source: None

Video-thoracoscopic left cardiac sympathetic denervation for long-QT syndrome

Background Congenital long-QT syndrome represents the most common cardiac channelopathy and manifests as potentially lethal ventricular arrhythmias. Prevention strategies include beta-blockade pharmacotherapy, implantable cardioverter-defibrillators, and left cardiac sympathetic denervation, which can increase the threshold for ventricular fibrillation. Herein, we report our experience with video-assisted thoracoscopic left cardiac sympathetic denervation. Methods We performed a retrospective review of the electronic medical records of all patients with congenital long-QT syndrome who underwent video-assisted thoracoscopic left cardiac sympathetic denervation at our institution. Results From September 2009 to May 2016, 6 patients with a mean age of 30.5 years (range 20–47 years) underwent video-assisted thoracoscopic left cardiac sympathetic denervation for medically refractory long-QT syndrome. All patients had an uneventful recovery and were discharged 1–3 days after the operation. At a median follow-up of 14 months (range 12–60 months), 4 patients had no cardiac events while 2 experienced 1 episode of arrhythmic syncope and 1 episode of appropriate implantable cardioverter-defibrillator shock. Following surgery, the mean annual cardiac events in the study cohort decreased from 2.13 to 0.33 ( p = 0.004) and the mean corrected QT interval reduced from 560 ms to 491 ms ( p = 0.006). Conclusions Video-assisted thoracoscopic left cardiac sympathetic denervation is a safe and effective therapy in patients with congenital long-QT syndrome who continue to suffer from recurrent life-threatening arrhythmias or frequent implantable cardioverter-defibrillator discharges despite maximum tolerated doses of beta blockers.

Sympathectomy via a posterior approach after a failed trans-thoracic approach: a case of its use for arrhythmia

OBJECTIVECongenital long QT syndrome (LQTS) provides an opportunity for neurosurgical intervention. Medication and implantable cardiac defibrillator (ICD)–refractory patients often require left cardiac sympathetic denervation (LCSD) via anterior video-assisted thoracoscopic surgery (VATS). However, this approach has major pulmonary contraindications and risks, with a common concern in children being their inability to tolerate single-lung ventilation. At Oregon Health & Science University, the authors have developed a posterior approach—extrapleural, minimally invasive, T1–5 LCSD—that minimizes this risk.METHODSA 9-year-old girl with LQTS type III presented to the emergency department while experiencing ventricular tachycardia (VT) and ventricular fibrillation (VF) with multiple ICD firings. Medical management failed to resolve the VF/VT. VATS was attempted but could not be safely performed due to respiratory insufficiency. The patient was reintubated for dual-lung ventilation and repositioned prone. Her respiratory insufficiency resolved. Using METRx serial dilating tubes under the microscope, the left T1–5 sympathetic ganglia were sectioned and removed.RESULTSPostoperatively, the patient had no episodes of VF/VT, pneumothorax, hemothorax, or Horner syndrome. With mexiletine and propranolol, she has remained largely VF/VT free, with only one VT episode during the 2-year follow-up period.CONCLUSIONSMinimally invasive, posterior, extrapleural, T1–5 LCSD is safe and effective for treating congenital LQTS in children, while minimizing the risks associated with VATS.