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Abstract：
BACKGROUND: Robust risk assessment is crucial for the growing repaired tetralogy of Fallot population at risk of major adverse clinical outcomes; however, current tools are hindered by lack of validation. This study aims to develop and validate a risk prediction model for death in the repaired tetralogy of Fallot population.
RESULTS: Patients with repaired tetralogy of Fallot enrolled in the INDICATOR (International Multicenter Tetralogy of Fallot Registry) cohort with clinical, arrhythmia, cardiac magnetic resonance, and outcome data were included. Patients from London, Amsterdam, and Boston sites were placed in the development cohort; patients from the Toronto site were used for external validation. Multivariable Cox regression was used to evaluate factors associated with time from cardiac magnetic resonance until the primary outcome: all-cause death. Of 1552 eligible patients (n=1221 in development, n=331 in validation; median age at cardiac magnetic resonance 23.4 [interquartile range, 15.6-35.6] years; median follow up 9.5 years), 102 (6.6%) experienced the primary outcome. The multivariable Cox model performed similarly during development (concordance index, 0.83 [95% CI, 0.78-0.88]) and external validation (concordance index, 0.80 [95% CI, 0.71-0.90]) and identified older age at cardiac magnetic resonance, obesity, type of tetralogy of Fallot repair, higher right ventricular end-systolic volume index, and lower biventricular global function index as independent predictors of death. A risk-scoring algorithm dividing patients into low-risk (score ≤4) versus high-risk (score >4) groups was validated to effectively discriminate risk of death (15-year survival of 95% versus 74%, respectively; P<0.001).
CONCLUSIONS: This externally validated mortality risk prediction algorithm can help identify vulnerable patients with repaired tetralogy of Fallot who may benefit from targeted interventions.
RESULTS: Patients with repaired tetralogy of Fallot enrolled in the INDICATOR (International Multicenter Tetralogy of Fallot Registry) cohort with clinical, arrhythmia, cardiac magnetic resonance, and outcome data were included. Patients from London, Amsterdam, and Boston sites were placed in the development cohort; patients from the Toronto site were used for external validation. Multivariable Cox regression was used to evaluate factors associated with time from cardiac magnetic resonance until the primary outcome: all-cause death. Of 1552 eligible patients (n=1221 in development, n=331 in validation; median age at cardiac magnetic resonance 23.4 [interquartile range, 15.6-35.6] years; median follow up 9.5 years), 102 (6.6%) experienced the primary outcome. The multivariable Cox model performed similarly during development (concordance index, 0.83 [95% CI, 0.78-0.88]) and external validation (concordance index, 0.80 [95% CI, 0.71-0.90]) and identified older age at cardiac magnetic resonance, obesity, type of tetralogy of Fallot repair, higher right ventricular end-systolic volume index, and lower biventricular global function index as independent predictors of death. A risk-scoring algorithm dividing patients into low-risk (score ≤4) versus high-risk (score >4) groups was validated to effectively discriminate risk of death (15-year survival of 95% versus 74%, respectively; P<0.001).
CONCLUSIONS: This externally validated mortality risk prediction algorithm can help identify vulnerable patients with repaired tetralogy of Fallot who may benefit from targeted interventions.
摘要：
背景：对于有重大不良临床结局风险的法洛四联症患者，可靠的风险评估至关重要；然而，当前的工具受到缺乏验证的阻碍。本研究旨在开发和验证法洛四联症修复人群死亡风险预测模型。
结果：在INDICATOR（国际多中心法洛四联症注册中心）队列中登记的法洛四联症修复患者，心律失常,心脏磁共振,并纳入结果数据。伦敦的病人,阿姆斯特丹,和波士顿站点被置于开发队列中;来自多伦多站点的患者被用于外部验证.多变量Cox回归用于评估从心脏磁共振到主要结果的时间相关因素：全因死亡。在1552名符合条件的患者中(n=1221名，n=331；心脏磁共振的中位年龄23.4[四分位距，15.6-35.6]年；中位随访9.5年），102（6.6％）经历了主要结果。多变量Cox模型在开发过程中表现相似(一致性指数，0.83[95%CI，0.78-0.88])和外部验证(一致性指数，0.80[95%CI，0.71-0.90])并在心脏磁共振时确定年龄较大，肥胖，法洛四联症修复类型,右心室收缩末期容积指数较高，和较低的双心室整体功能指数是死亡的独立预测因子。将患者分为低风险组(评分≤4)和高风险组(评分>4)的风险评分算法被验证有效区分死亡风险(15年生存率为95%对74%,分别；P<0.001)。
结论：这种外部验证的死亡率风险预测算法可以帮助识别可能受益于针对性干预措施的法洛四联症修复的脆弱患者。
结果：在INDICATOR（国际多中心法洛四联症注册中心）队列中登记的法洛四联症修复患者，心律失常,心脏磁共振,并纳入结果数据。伦敦的病人,阿姆斯特丹,和波士顿站点被置于开发队列中;来自多伦多站点的患者被用于外部验证.多变量Cox回归用于评估从心脏磁共振到主要结果的时间相关因素：全因死亡。在1552名符合条件的患者中(n=1221名，n=331；心脏磁共振的中位年龄23.4[四分位距，15.6-35.6]年；中位随访9.5年），102（6.6％）经历了主要结果。多变量Cox模型在开发过程中表现相似(一致性指数，0.83[95%CI，0.78-0.88])和外部验证(一致性指数，0.80[95%CI，0.71-0.90])并在心脏磁共振时确定年龄较大，肥胖，法洛四联症修复类型,右心室收缩末期容积指数较高，和较低的双心室整体功能指数是死亡的独立预测因子。将患者分为低风险组(评分≤4)和高风险组(评分>4)的风险评分算法被验证有效区分死亡风险(15年生存率为95%对74%,分别；P<0.001)。
结论：这种外部验证的死亡率风险预测算法可以帮助识别可能受益于针对性干预措施的法洛四联症修复的脆弱患者。
