Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Windland Smith Rice Genetic Heart Rhythm Clinic (T.N., J.M.B., M.J.A.), Mayo Clinic, Rochester, MN.
Department of Molecular Pharmacology & Experimental Therapeutics (Windland Smith Rice Sudden Death Genomics Laboratory) (J.M.B., K.B.S., M.J.A.), Mayo Clinic, Rochester, MN.
Circ Arrhythm Electrophysiol. 2020 Dec;13(12):e008830. doi: 10.1161/CIRCEP.120.008830. Epub 2020 Nov 16.
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.
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.
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.
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.
胸腔镜下心左交感神经切除术（LCSD）是一种有效的抗纤维颤搐的微创治疗方法，适用于长 QT 综合征（LQTS）等潜在危及生命的心律失常综合征患者。尽管最初主要用于在接受β受体阻滞剂治疗的记录 LQTS 相关突破性心脏事件后强化治疗，但对于某些 LQTS 患者，LCSD 作为单次治疗需要进一步评估。我们正在介绍我们对经过精心选择的 LQTS 患者进行 LCSD 单一治疗的早期经验。
在评估和治疗的 1400 例 LQTS 患者中，对自 2005 年以来在我们机构接受 LCSD 的 204 例 LQTS 患者进行了回顾性分析，以确定 LCSD 作为单一、单一治疗的患者。分析了诊断前的症状状态、临床和遗传诊断以及诊断后突破性心脏事件的临床数据，以确定 LCSD 单一治疗的疗效。
总体而言，204 例患者中有 64 例（31%）单独接受 LCSD 治疗（37 例[58%]为女性，平均 QTc 466±30ms，16 例[25%]患者在诊断前有症状，平均诊断年龄为 17.3±11.8 岁，5 例[8%]患者在诊断后有≥1 次突破性心脏事件，LCSD 年龄平均为 21.1±11.4 岁）。LCSD 单一治疗的主要动机是 56/64 例（88%）患者因β受体阻滞剂相关副作用（即β受体阻滞剂不耐受）而导致生活质量不可接受。潜在的 LQTS 基因型为 LQT1 36 例（56%）和 LQT2 20 例（31%）。没有明显的 LCSD 相关手术并发症。目前平均随访 2.7±2.4 年，180 患者年中仅有 3 例患者在 LCSD 后发生非致命性突破性心脏事件。
LCSD 可能是一种安全有效的单一治疗方法，适用于不能耐受β受体阻滞剂的特定患者。然而，LCSD 不是根治性的，在考虑将 LCSD 作为单一治疗时，患者选择将是至关重要的。
