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
Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA The Sheikh Zayed Institute for Pediatric Surgical Innovation, Children's National Health System, Washington, DC, USA.
Division of Cardiovascular Surgery, Children's National Health System, Washington, DC, USA.
World J Pediatr Congenit Heart Surg. 2015 Jan;6(1):33-8. doi: 10.1177/2150135114555203.
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.
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).
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.
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.
先天性离子通道疾病，包括先天性长QT综合征（LQTS），在儿科患者中会导致严重的发病率。当药物治疗无法控制症状或心律失常时，可能需要更具侵入性的治疗策略。本研究考察了我们机构采用手术性心脏去神经疗法治疗儿童这些致心律失常疾病的临床经验。
一项经机构审查委员会批准的回顾性研究确定了10例患有先天性离子通道病的儿科患者，他们于2011年5月至2014年4月在单一机构接受了手术性心脏去神经疗法。8例患者诊断为先天性LQTS，2例患者诊断为儿茶酚胺能多形性室性心动过速（CPVT）。所有患者均通过电视辅助胸腔镜手术（VATS）进行了交感神经切除术和部分星状神经节切除术。
10例患者中有6例术前记录有室性心律失常，70%的患者术前有晕厥。交感神经切除术后，75%的LQTS患者校正QT间期缩短。88%的先天性LQTS患者术后无致心律失常症状，但2例CPVT患者在整个随访期间持续有症状。中位随访10个月后，所有患者均存活。
通过VATS进行手术性心脏去神经疗法对于药物治疗无效的先天性LQTS患者是一种有用的治疗策略，其在CPVT治疗中的潜在益处尚不清楚。对先天性离子通道病患者进行手术性心脏去神经疗法与植入式心脏复律除颤器疗效的前瞻性比较既及时又关键。
