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Validated Model for Predicting Sudden Cardiac Death in Pediatric HCM
Quick Takes
- Predicting SCD in pediatric HCM has typically been wrought with imprecision and required the adaptation of adult-specific risk criteria.
- Risk factors for SCD in pediatric patients are not universally reflective of those in adult HCM patients.
- This newly developed model to predict risk of SCD in pediatric HCM patients will improve the appropriate utilization of primary prevention ICDs in this population.
Study Questions:
What is the validity of a sudden cardiac death (SCD) risk prediction model that is developed for pediatric hypertrophic cardiomyopathy (HCM)?
Methods:
The model was developed using a multicenter observational cohort study of phenotypically positive pediatric HCM patients to identify risk factors associated with SCD, cardiac arrest, or aborted SCD. The final model was validated in a large independent cohort.
Results:
The final model included age at diagnosis, nonsustained ventricular tachycardia, unexplained syncope, septal and left ventricular (LV) posterior wall thickness z-scores, left atrial diameter, LV outflow tract gradient, and ± presence of a pathogenic variant. Independent models were created for including or not including genotype data. The model was developed from 572 HCM patients diagnosed prior to age 18 years, of which 53 experienced a SCD event during over 2,800 patient-years of follow-up (9.1% risk of SCD over 5 years). Patients predicted high risk by the model had a mean 20% risk of SCD in 5 years. The model was validated in an independent cohort of 285 childhood-onset HCM patients demonstrating a c-statistic (measure of risk model validity) of 0.7-0.72; indicating a prediction accuracy of >70%.
Conclusions:
This pediatric-specific prediction model for SCD in HCM will significantly improve the management of these patients regarding the use of primary prevention implantable cardioverter-defibrillators (ICDs).
Perspective:
The ability to predict which pediatric HCM patient will benefit from a primary prevention ICD has typically been wrought with imprecision—a daunting reality considering the relatively high risk of adverse effects from ICDs in this population. This comprehensively developed and externally validated prediction model will be highly beneficial in the management of this patient population. The authors are currently developing a computerized calculator for further validation and subsequent widespread availability.
Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Prevention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure
Keywords: Arrhythmias, Cardiac, Cardiomyopathy, Hypertrophic, Death, Sudden, Cardiac, Defibrillators, Implantable, Genotype, Heart Arrest, Heart Failure, Pediatrics, Risk Factors, Secondary Prevention, Tachycardia, Ventricular
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