Windland Smith Rice Sudden Death Genomics Laboratory, Department of Molecular Pharmacology & Experimental Therapeutics, Division of Pediatric Cardiology, Department of Pediatric & Adolescent Medicine, Division of Pediatric Surgery, Department of Surgery, and Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, MN.
Circ Arrhythm Electrophysiol. 2013 Aug;6(4):705-11. doi: 10.1161/CIRCEP.113.000102. Epub 2013 May 31.
Long QT syndrome (LQTS) is a potentially lethal but highly treatable cardiac channelopathy. Treatment options include pharmacotherapy, device therapy, and left cardiac sympathetic denervation (LCSD). Here, we sought to determine the characteristics of LQTS patients who have had ≥1 LQTS-related breakthrough cardiac event (BCE) after LCSD.
We performed retrospective chart review for 52 consecutive patients (24 males; mean age at diagnosis, 10.0±10 years; mean QTc, 528±74 ms) with LQTS who underwent LCSD between 2005 and 2010 (mean age at LCSD, 14.1±10 years) and have been followed up for 3.6±1.3 years. A BCE was defined as either (1) an appropriate ventricular fibrillation-terminating implantable cardioverter defibrillator shock or (2) arrhythmogenic syncope, seizures, or aborted cardiac arrest after LCSD. Thirty-three patients (61%) had LCSD as primary prevention because of either high-risk assessment or β-blocker intolerance. So far, 12 of 52 (23%) patients (7 males) have experienced ≥1 BCE post LCSD. The clinical phenotype of patients with BCEs was significantly more severe than patients without a BCE. No BCEs were seen in patients undergoing LCSD for β-blocker intolerance (0/12 versus 17/40; P<0.001).
Although a marked reduction in number of cardiac events is usually seen after LCSD, ≈50% of high-risk LQTS patients have experienced ≥1 post-LCSD breakthrough. Therefore, LCSD must not be viewed as curative or as an alternative in implantable cardioverter defibrillator for high-risk patients. Prophylactic LCSD may provide another option to counter a suboptimal quality of life resulting from medication-related side effects.
长 QT 综合征（LQTS）是一种潜在致命但高度可治疗的心脏通道病。治疗选择包括药物治疗、器械治疗和左侧心脏交感神经切除术（LCSD）。在这里，我们试图确定在 LCSD 后发生≥1 次 LQTS 相关突破性心脏事件（BCE）的 LQTS 患者的特征。
我们对 52 例连续 LQTS 患者（24 例男性；诊断时的平均年龄为 10.0±10 岁；平均 QTc 为 528±74 ms）进行了回顾性图表审查，这些患者于 2005 年至 2010 年间接受了 LCSD（LCSD 时的平均年龄为 14.1±10 岁），并随访了 3.6±1.3 年。BCE 定义为（1）适当的心室颤动终止植入式心脏复律除颤器电击或（2）LCSD 后心律失常性晕厥、癫痫发作或心脏骤停中止。33 例（61%）患者因高危评估或β受体阻滞剂不耐受而进行 LCSD 作为一级预防。到目前为止，52 例患者中有 12 例（7 例男性）在 LCSD 后经历了≥1 次 BCE。有 BCE 的患者的临床表型明显比没有 BCE 的患者更严重。在因β受体阻滞剂不耐受而接受 LCSD 的患者中未发生 BCE（0/12 例与 17/40 例；P<0.001）。
尽管 LCSD 后通常会显著减少心脏事件的发生次数，但仍有约 50%的高危 LQTS 患者经历了≥1 次 LCSD 突破性事件。因此，LCSD 不能被视为高危患者的植入式心脏复律除颤器的治愈或替代方法。预防性 LCSD 可能为因药物相关副作用导致生活质量不佳提供另一种选择。
