RESULTS: A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty-five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell C index of 0.73 [95% CI, 0.65-0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03-0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function.
CONCLUSIONS: We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.
结果:回顾性研究,多中心,417例大动脉转位患者的纵向队列(中位年龄,基线时24年[四分位数间距,18-30];63%的男性)独立于模型开发和内部验证队列进行了研究。评估了预测模型在预测5年风险方面的表现,我们在队列中分别评估了其他主要临床事件的预测因子.25例患者(5.9%)在5年内达到主要临床事件终点。模型验证显示5年高风险患者和5年低风险患者之间有很好的区分(HarrellC指数为0.73[95%CI,0.65-0.81]),但倾向于高估这种风险(校准斜率为0.20[95%CI,0.03-0.36])。在我们的人口中,主要临床事件的最有力的独立预测因子是心力衰竭史和至少轻度的肺下左心室功能受损.
结论:我们报道了在患有大动脉转位的大型成人队列中首次对主要临床事件风险模型进行外部验证。该模型允许将低风险患者与中高风险患者区分开来。先前的心力衰竭发作和肺下左心室功能障碍似乎是患者预后的关键指标。需要进一步优化风险模型,以个性化大动脉移位患者的风险预测。