Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
Department of Cardiovascular Diseases/Division of Heart Rhythm Services, Mayo Clinic, Rochester, Minnesota.
J Am Coll Cardiol. 2017 Jul 25;70(4):453-462. doi: 10.1016/j.jacc.2017.05.046.
Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy with a 1% to 5% annual risk of LQTS-triggered syncope, aborted cardiac arrest, or sudden cardiac death.
This study sought to evaluate LQTS outcomes from a single center in the contemporary era.
The authors conducted a retrospective study comprising the 606 patients with LQTS (LQT1 in 47%, LQT2 in 34%, and LQT3 in 9%) who were evaluated in Mayo Clinic's Genetic Heart Rhythm Clinic from January 1999 to December 2015. Breakthrough cardiac events (BCEs) were defined as LQTS-attributable syncope or seizures, aborted cardiac arrest, appropriate ventricular fibrillation-terminating implantable cardioverter-defibrillator shocks, and sudden cardiac death.
There were 166 (27%) patients who were symptomatic prior to their first Mayo Clinic evaluation. Median age at first symptom was 12 years. Treatment strategies included no active therapy in 47 (8%) patients, beta-blockers alone in 350 (58%) patients, implantable cardioverter-defibrillators alone in 25 (4%) patients, left cardiac sympathetic denervation alone in 18 (3%) patients, and combination therapy in 166 (27%) patients. Over a median follow-up of 6.7 (IQR: 3.9 to 9.8) years, 556 (92%) patients have not experienced an LQTS-triggered BCE. Only 8 of 440 (2%) previously asymptomatic patients have experienced a single BCE. In contrast, 42 of 166 (25%) previously symptomatic patients have experienced ≥1 BCE. Among the 30 patients with ≥2 BCEs, 2 patients have died and 3 LQT3 patients underwent cardiac transplantation.
Although outcomes have improved markedly, further optimization of treatment strategies is still needed given that 1 in 4 previously symptomatic patients experienced at least 1 subsequent, albeit nonlethal, LQTS-triggered cardiac event.
长 QT 综合征（LQTS）是一种潜在致命的心脏通道病，每年有 1%至 5%的风险发生由 LQTS 引发的晕厥、心搏骤停或心源性猝死。
本研究旨在评估单中心在当代的 LQTS 结局。
作者进行了一项回顾性研究，纳入了 1999 年 1 月至 2015 年 12 月在梅奥诊所遗传心脏节律诊所接受评估的 606 例 LQTS 患者（LQT1 占 47%，LQT2 占 34%，LQT3 占 9%）。突破性心脏事件（BCEs）定义为与 LQTS 相关的晕厥或癫痫发作、心搏骤停、适当的心室颤动终止植入式心脏复律除颤器电击和心源性猝死。
在首次梅奥诊所评估前有 166 例（27%）患者出现症状。首次出现症状的中位年龄为 12 岁。治疗策略包括 47 例（8%）患者无积极治疗、350 例（58%）患者单独使用β受体阻滞剂、25 例（4%）患者单独使用植入式心脏复律除颤器、18 例（3%）患者单独行左侧心脏交感神经切除术、166 例（27%）患者采用联合治疗。中位随访 6.7 年（IQR：3.9 至 9.8）期间，556 例（92%）患者未发生由 LQTS 引发的 BCE。440 例（2%）既往无症状患者中仅有 8 例发生单一 BCE。相比之下，166 例（25%）既往有症状患者中 42 例发生≥1 次 BCE。在 30 例有≥2 次 BCE 的患者中，2 例患者死亡，3 例 LQT3 患者进行了心脏移植。
尽管结局显著改善，但鉴于 1/4 例既往有症状患者发生至少 1 次后续非致命性由 LQTS 引发的心脏事件，仍需进一步优化治疗策略。
