Can sudden cardiac death risk in children with hypertrophic cardiomyopathy be predicted?
In this cohort study of 1024 consecutively evaluated children (age ≤16 years), a prognostic model was developed using preselected predictor variables identified from the literature. The model’s ability to predict risk at 5 years was internally validated using bootstrapping.
This new, validated risk stratification model for sudden cardiac death risk in childhood hypertrophic cardiomyopathy may provide individualized estimates of risk at 5 years using readily obtained data on clinical risk factors; external validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.
Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk.
To develop and validate an SCD risk prediction model that provides individualized risk estimates.
Design, Setting, and Participants
A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017.
The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping.
Main Outcomes and Measures
A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise).
Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model’s ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95%, CI 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years.
Conclusions and Relevance
This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.
Norrish G, Ding T, Field E, et al. Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids). JAMA Cardiol. Published online August 14, 2019. doi:10.1001/jamacardio.2019.2861
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