Yale School of Medicine, New Haven, CT (R.L., M.B., C. Barth, J.D., F.L, L.S.).
University of Pennsylvania, Philadelphia (S.D.).
Circulation. 2024 Aug 13;150(7):516-530. doi: 10.1161/CIRCULATIONAHA.123.067590. Epub 2024 Jul 25.
Whether vigorous exercise increases risk of ventricular arrhythmias for individuals diagnosed and treated for congenital long QT syndrome (LQTS) remains unknown.
The National Institutes of Health-funded LIVE-LQTS study (Lifestyle and Exercise in the Long QT Syndrome) prospectively enrolled individuals 8 to 60 years of age with phenotypic and/or genotypic LQTS from 37 sites in 5 countries from May 2015 to February 2019. Participants (or parents) answered physical activity and clinical events surveys every 6 months for 3 years with follow-up completed in February 2022. Vigorous exercise was defined as ≥6 metabolic equivalents for >60 hours per year. A blinded Clinical Events Committee adjudicated the composite end point of sudden death, sudden cardiac arrest, ventricular arrhythmia treated by an implantable cardioverter defibrillator, and likely arrhythmic syncope. A National Death Index search ascertained vital status for those with incomplete follow-up. A noninferiority hypothesis (boundary of 1.5) between vigorous exercisers and others was tested with multivariable Cox regression analysis.
Among the 1413 participants (13% <18 years of age, 35% 18-25 years of age, 67% female, 25% with implantable cardioverter defibrillators, 90% genotype positive, 49% with LQT1, 91% were treated with beta-blockers, left cardiac sympathetic denervation, and/or implantable cardioverter defibrillator), 52% participated in vigorous exercise (55% of these competitively). Thirty-seven individuals experienced the composite end point (including one sudden cardiac arrest and one sudden death in the nonvigorous group, one sudden cardiac arrest in the vigorous group) with overall event rates at 3 years of 2.6% in the vigorous and 2.7% in the nonvigorous exercise groups. The unadjusted hazard ratio for experience of events for the vigorous group compared with the nonvigorous group was 0.97 (90% CI, 0.57-1.67), with an adjusted hazard ratio of 1.17 (90% CI, 0.67-2.04). The upper 95% one-sided confidence level extended beyond the 1.5 boundary. Neither vigorous or nonvigorous exercise was found to be superior in any group or subgroup.
Among individuals diagnosed with phenotypic and/or genotypic LQTS who were risk assessed and treated in experienced centers, LQTS-associated cardiac event rates were low and similar between those exercising vigorously and those not exercising vigorously. Consistent with the low event rate, CIs are wide, and noninferiority was not demonstrated. These data further inform shared decision-making discussions between patient and physician about exercise and competitive sports participation.
URL: https://www.clinicaltrials.gov; Unique identifier: NCT02549664.
对于已经确诊并接受先天性长 QT 综合征（LQTS）治疗的个体，剧烈运动是否会增加室性心律失常的风险尚不清楚。
美国国立卫生研究院资助的 LIVE-LQTS 研究（长 QT 综合征中的生活方式和运动）前瞻性地招募了来自 5 个国家的 37 个地点的年龄在 8 至 60 岁之间、具有表型和/或基因型 LQTS 的个体。参与者（或其父母）每 6 个月回答一次身体活动和临床事件调查，为期 3 年，并于 2022 年 2 月完成随访。剧烈运动定义为每年 >60 小时、代谢当量≥6。一个盲法临床事件委员会对突然死亡、心源性猝死、植入式心脏复律除颤器治疗的室性心律失常和可能的心律失常性晕厥的复合终点进行裁决。通过全国死亡索引搜索确定了随访不完整者的生存状态。采用多变量 Cox 回归分析对剧烈运动者和非剧烈运动者进行非劣效性假设检验（边界为 1.5）。
在 1413 名参与者中（13%<18 岁，35%18-25 岁，67%女性，25%植入式心脏复律除颤器，90%基因型阳性，49%为 LQT1，91%接受β受体阻滞剂、左心交感神经切除术和/或植入式心脏复律除颤器治疗），52%的参与者进行了剧烈运动（其中 55%是竞争性运动）。37 名参与者出现了复合终点（包括非剧烈运动组中的 1 例心源性猝死和 1 例心源性猝死、剧烈运动组中的 1 例心源性猝死），3 年时剧烈和非剧烈运动组的总事件发生率分别为 2.6%和 2.7%。与非剧烈运动组相比，剧烈运动组的事件发生率调整后危险比为 0.97（90%CI，0.57-1.67），调整后危险比为 1.17（90%CI，0.67-2.04）。95%单侧置信上限上限超出了 1.5 边界。剧烈运动或非剧烈运动在任何组或亚组中均未表现出优势。
在已确诊为表型和/或基因型 LQTS 并在经验丰富的中心进行风险评估和治疗的个体中，LQTS 相关心脏事件发生率较低，且在剧烈运动和非剧烈运动者之间相似。与低事件率一致，CI 较宽，未证明非劣效性。这些数据进一步为患者和医生之间关于运动和竞技体育参与的共同决策讨论提供了信息。
