Key result
ESC guidelines show low discriminative ability for SCD risk in childhood HCM.
Why the study?
ESC guidelines recommend considering an ICD in childhood HCM if two or more clinical risk factors are present, but this approach has not been formally validated.
Comparison
Patients stratified by number of ESC clinical risk factors (0 vs 1 vs >=2)
Design
Validation cohort study
Follow-up
Median 5.5 years
Authors
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ESC guidelines show limited discrimination for pediatric HCM SCD risk; leaves open need for refined models in larger cohorts.
Cohort (n=411)
Effect estimate: C-statistic 0.62 (95% CI 0.52-0.72)
p-value: p=0.34
The current ESC guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy have a low ability to discriminate between high- and low-risk individuals.
Norrish et al. (2019) conducted a cohort in childhood hypertrophic cardiomyopathy (n=411). ESC guidelines risk stratification (≥2 risk factors) vs. 0 or 1 risk factor was evaluated on composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate ICD therapy, or sustained ventricular tachycardia), defined as a major arrhythmic cardiac event (MACE) (C-statistic 0.62, 95% CI 0.52-0.72, p=0.34). The ESC guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy showed low discriminative ability (C-statistic 0.62; 95% CI 0.52-0.72 at 5 years).
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