Key result
PRIMaCY risk model fails to reliably predict sudden cardiac death in childhood HCM.
Why the study?
The performance and clinical impact of the PRIMaCY sudden cardiac death risk prediction model had not been independently investigated in childhood hypertrophic cardiomyopathy.
Does the PRIMaCY risk model accurately predict sudden cardiac death risk compared to the HCM Risk-Kids model in children with hypertrophic cardiomyopathy?
Cohort (n=301)
No
Does the PRIMaCY risk model accurately predict sudden cardiac death risk compared to the HCM Risk-Kids model in children with hypertrophic cardiomyopathy?
Effect estimate: C-statistic 0.66 (95% CI 0.52-0.8)
The PRIMaCY risk model overestimates sudden cardiac death risk in childhood HCM compared to the HCM Risk-Kids model, potentially leading to unnecessary ICD implantations and associated lifetime complications.
May not guide ICD decisions in childhood HCM; leaves open comparative validation versus HCM Risk-Kids.
AIMS: The validated HCM Risk-Kids model provides accurate individualized estimates of sudden cardiac death risk in children with hypertrophic cardiomyopathy (HCM). A second validated model, PRIMaCY, also provides individualized estimates of risk, but its performance and clinical impact has not been independently investigated. The aim of this study was to investigate the clinical impact of using the PRIMaCY sudden cardiac death (SCD) risk model in childhood HCM. METHODS AND RESULTS: The estimated 5-year SCD risk was calculated for children meeting diagnostic criteria for HCM in a large single-centre cohort using PRIMaCY (clinical and genetic) and HCM Risk-Kids model, and model performance was assessed. Three hundred one patients [median age 10 (interquartile range 4-14)] were followed up for an average of 4.9 (±3.8) years, during which 30 (10.0%) reached the SCD or equivalent event endpoint. Harrell's C-statistic for the clinical and genetic models was 0.66 [95% confidence interval (CI) 0.52-0.8] and 0.66 (95% CI 0.54-0.80) with a calibration slope of 0.19 (95% CI 0.04-0.54) and 0.26 (95% CI -0.03-0.62), respectively. The number needed to treat to potentially treat one life-threatening arrhythmia for the PRIMaCY clinical, PRIMaCY genetic, and HCM Risk-Kids models was 13.7, 14.5, and 9.4, respectively. CONCLUSION: Although PRIMaCY has a similar discriminatory ability to that reported for HCM Risk-Kids, estimated risk estimates did not correlate well with observed risk. A higher proportion of patients met implantable cardioverter-defibrillator thresholds using PRIMaCY model compared with HCM Risk-Kids. This has important clinical implications as these patients will be exposed to a lifetime risk of complications and inappropriate therapies.
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Norrish et al. (2023) conducted a cohort in Childhood hypertrophic cardiomyopathy (n=301). PRIMaCY sudden cardiac death risk model vs. HCM Risk-Kids model was evaluated on Sudden cardiac death or equivalent event (C-statistic 0.66, 95% CI 0.52-0.8). The PRIMaCY risk model yielded a C-statistic of 0.66 (95% CI 0.52-0.8) for sudden cardiac death in childhood HCM, but estimated risk did not correlate well with observed risk.
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