A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy

Figures

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  Figure 1.. Cumulative proportion of SCD events estimated using competing risk models in PRIMaCY and SHaRe cohorts. A, PRIMaCY cohort (n=572): The cumulative proportion of SCD events in patients with pediatric HCM at 1-year follow-up was 2.8% (95% CI, 1.4%–4.2%), at 5 years was 9.1% (95% CI, 6.3%–11.9%), and at 10 years was 15.0% (95% CI, 10.0%–19.7%). The cumulative proportion of death from other causes at 1-year follow-up was 0.9% (95% CI, 0.1%–1.8%), at 5 years was 1.8% (95% CI, 0.6%–3.0%), and at 10 years was 1.8% (95% CI, 0.6%–3.0%). B, SHaRe cohort (n=285): The cumulative proportion of SCD events at 1-year follow-up was 2.3% (95% CI, 0.5%–4.1%), at 5 years was 6.8% (95% CI, 3.2%–10.3%), and at 10 years was 13.7% (95% CI, 6.5%–20.4%). The cumulative proportion of death from other causes at 1-year follow-up was 0.7% (95% CI, 0.0%–1.7%), at 5 years was 2.1% (95% CI, 0.2%–3.9%), and at 10 years was 4.5% (95% CI, 0.6%–8.2%). Echo indicates echocardiogram; HCM, hypertrophic cardiomyopathy; PRIMaCY, Precision Medicine for Cardiomyopathy Study; SCD, sudden cardiac death; and SHaRe, Sarcomeric Human Cardiomyopathy Registry.

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  Figure 2.. Regression-adjusted effects of continuous covariates and clinical SCD risk prediction model performance (n=572). A through E, The top of each figure shows the observed values of the continuous risk predictor among patients with pediatric HCM in the training cohort who experienced the composite SCD outcome; the bottom shows the observed values of the predictor among those who did not experience the composite SCD outcome. SCD risk increased with age at diagnosis(A); increase in IVSD z score(B); LVPWD z score(C); and LA diameter z score(D). E, The risk associated with peak resting LVOT gradient remained flat when the gradient was ≤100 mm Hg and decreased as the gradient increased to >100 mm Hg. F, The cumulative proportion of SCD events stratified by tertiles of risk predicted by the clinical-only model, that is, predicted risk <4.7%, 4.7% to 8.3%, and >8.3%. G, The calibration curve for the clinical model applied to the training cohort shows the predicted versus the observed 5-year risk of the composite SCD outcome. The prediction accuracy, that is, c-statistic of the model, was 0.75. The dashed line, the 45° line through 0, represents a perfectly calibrated model between the…

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  Figure 3.. Regression-adjusted effects of continuous covariates and clinical/genetic SCD risk prediction model performance (n=572). A through E, The top of each figure shows the observed values of the continuous risk predictor among patients with pediatric HCM in the training cohort who experienced the composite SCD outcome; the bottom shows the observed values of the predictor among those who did not experience the composite SCD outcome. SCD risk increased with age at first evaluation(A); increase in IVSD z score(B); LVPWD z score(C); and LA diameter z score(D). E, The risk associated with peak resting LVOT gradient remained flat when the gradient was ≤100 mm Hg and decreased as the gradient increased above 100 mm Hg. F, Cumulative proportion of SCD events stratified by tertiles of risk predicted by the clinical/genetic model, that is, predicted risk <4.7%, 4.7% to 8.3%, and >8.3%. G, The calibration curve for the clinical/genetic model applied to the training cohort shows the predicted versus the observed 5-year risk of the composite SCD outcome. The prediction accuracy, that is, c-statistic of the model, was 0.762. The dashed line, the 45° line through 0, represents a perfectly…