BACKGROUND: Long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are two of the most common, potentially lethal, cardiac channelopathies. Treatment strategies for the primary and secondary prevention of life-threatening polymorphic ventricular tachycardia/fibrillation include pharmacotherapy with beta-blockers, implantable cardioverter defibrillators, and left cardiac sympathetic denervation (LCSD). OBJECTIVES: This study sought to report our institutional experience with LCSD using video-assisted thoracic surgery (VATS). METHODS: From November 2005 through November 2008, 20 patients (8 female, average age at surgery 9.1 +/- 9.7 years, range 2 months to 42 years) underwent LCSD via either a traditional approach (N = 2) or VATS (N = 18). A total of 12 patients had genotype-positive LQTS (7 LQT1, 2 LQT2, 1 LQT3, 2 LQT1/LQT2), 2 had JLNS, 4 had genotype-negative LQTS, and 2 had CPVT1. Electronic medical records were reviewed for patient selection, perioperative complications, and short-term outcomes. RESULTS: LCSD was performed as a secondary prevention strategy in 11 patients (8 LQTS patients, average QTc 549 ms) and as primary prevention in 9 patients (average QTc 480 ms). There were no perioperative complications, including no intraoperative ectopy, no uncontrolled hemorrhage, and no VATS cases requiring conversion to a traditional approach. The average length of available follow-up was 16.6 +/- 9.5 months (range 4 to 40 months). Among the 18 patients who underwent VATS-LCSD, the average time from operation to dismissal was 2.6 days (range 1 day to 15 days), the majority being next-day dismissals. Among those receiving LCSD as secondary prevention, there has been a marked reduction in cardiac events. CONCLUSIONS: We present a series of 20 patients with LQTS and CPVT who underwent LCSD, 18 using VATS. The minimally invasive VATS surgical approach was associated with minimal perioperative complications, including no intraoperative ectopy and excellent immediate and short-term outcomes. Videoscopic denervation surgery, in addition to traditional LCSD, offers a safe and effective treatment option for the personalized medicine required for patients with LQTS/CPVT.
BACKGROUND:Long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are two of the most common, potentially lethal, cardiac channelopathies. Treatment strategies for the primary and secondary prevention of life-threatening polymorphic ventricular tachycardia/fibrillation include pharmacotherapy with beta-blockers, implantable cardioverter defibrillators, and left cardiac sympathetic denervation (LCSD). OBJECTIVES: This study sought to report our institutional experience with LCSD using video-assisted thoracic surgery (VATS). METHODS: From November 2005 through November 2008, 20 patients (8 female, average age at surgery 9.1 +/- 9.7 years, range 2 months to 42 years) underwent LCSD via either a traditional approach (N = 2) or VATS (N = 18). A total of 12 patients had genotype-positive LQTS (7 LQT1, 2 LQT2, 1 LQT3, 2 LQT1/LQT2), 2 had JLNS, 4 had genotype-negative LQTS, and 2 had CPVT1. Electronic medical records were reviewed for patient selection, perioperative complications, and short-term outcomes. RESULTS: LCSD was performed as a secondary prevention strategy in 11 patients (8 LQTS patients, average QTc 549 ms) and as primary prevention in 9 patients (average QTc 480 ms). There were no perioperative complications, including no intraoperative ectopy, no uncontrolled hemorrhage, and no VATS cases requiring conversion to a traditional approach. The average length of available follow-up was 16.6 +/- 9.5 months (range 4 to 40 months). Among the 18 patients who underwent VATS-LCSD, the average time from operation to dismissal was 2.6 days (range 1 day to 15 days), the majority being next-day dismissals. Among those receiving LCSD as secondary prevention, there has been a marked reduction in cardiac events. CONCLUSIONS: We present a series of 20 patients with LQTS and CPVT who underwent LCSD, 18 using VATS. The minimally invasive VATS surgical approach was associated with minimal perioperative complications, including no intraoperative ectopy and excellent immediate and short-term outcomes. Videoscopic denervation surgery, in addition to traditional LCSD, offers a safe and effective treatment option for the personalized medicine required for patients with LQTS/CPVT.
Authors: Christine A Kenyon; Randall Flick; Christopher Moir; Michael J Ackerman; Christina M Pabelick Journal: Paediatr Anaesth Date: 2010-03-22 Impact factor: 2.556
Authors: Arthur A M Wilde; Arthur J Moss; Elizabeth S Kaufman; Wataru Shimizu; Derick R Peterson; Jesaia Benhorin; Coeli Lopes; Jeffrey A Towbin; Carla Spazzolini; Lia Crotti; Wojciech Zareba; Ilan Goldenberg; Jørgen K Kanters; Jennifer L Robinson; Ming Qi; Nynke Hofman; David J Tester; Connie R Bezzina; Marielle Alders; Takeshi Aiba; Shiro Kamakura; Yoshihiro Miyamoto; Mark L Andrews; Scott McNitt; Bronislava Polonsky; Peter J Schwartz; Michael J Ackerman Journal: Circulation Date: 2016-08-26 Impact factor: 29.690