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Independent External Evaluation of Pediatric Hypertrophic Cardiomyopathy Risk Scores in Predicting Severe Ventricular
Marie Wilkin1, Diala Khraiche1, Elena Panaioli1
1M3C-Necker-Enfants-malades, AP-HP, Université de Paris Cité, France (M.W., D.K., E.P., M.P., O.R., D.B., V.W.).
Insights
Two risk scores for childhood hypertrophic cardiomyopathy (HCM) showed imperfect prediction of sudden cardiac death. Periodic reassessment improved their performance in identifying high-risk pediatric patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Sudden Cardiac Death Risk Stratification
Background:
- Sudden cardiac death is a primary concern in childhood hypertrophic cardiomyopathy (HCM).
- Two risk scores, HCM Risk-Kids and PRIMaCY, were recently developed to predict 5-year sudden cardiac death risk.
- Independent validation of these scores in a pediatric cohort is crucial.
Purpose of the Study:
- To evaluate the performance of the HCM Risk-Kids and PRIMaCY risk scores in an independent cohort of pediatric patients with HCM.
- To assess the accuracy of these scores in predicting the risk of sudden cardiac death and related events.
- To determine if periodic reassessment of risk scores improves predictive accuracy.
Main Methods:
- Retrospective analysis of 100 pediatric patients (<18 years) with HCM diagnosed between 2003 and 2023.
- Calculation of HCM Risk-Kids and PRIMaCY scores at diagnosis and during follow-up.
- Primary composite outcome: sustained ventricular arrhythmia, appropriate ICD therapy, aborted cardiac arrest, or sudden cardiac death.
Main Results:
- 13% of patients experienced the primary composite outcome over a mean follow-up of 8.6 years.
- PRIMaCY score showed better discrimination in the first 5 years (C-index 0.70) compared to HCM Risk-Kids (C-index 0.52).
- Periodic reassessment identified 92.3% of events, improving risk stratification accuracy for both scores.
Conclusions:
- HCM Risk-Kids and PRIMaCY scores demonstrated imperfect discrimination and tended to overestimate risk in pediatric HCM.
- Periodic reassessment of risk scores significantly enhanced their performance in identifying high-risk patients.
- Regular re-evaluation is vital for optimizing risk stratification and management in pediatric HCM.
Background:
Sudden cardiac death is the most common cause of death in childhood hypertrophic cardiomyopathy (HCM). Recently, 2 risk scores have been developed to estimate the 5-year risk of sudden cardiac death. We aimed to assess their respective performances in an independent cohort.
Methods:
All patients with HCM aged <18 years from a single center were retrospectively included between 2003 and 2023. HCM Risk-Kids and PRIMaCY risk scores were calculated at diagnosis and during follow-up. The primary composite outcome included sustained ventricular arrhythmia, appropriate implantable cardioverter defibrillator (ICD) therapy, aborted cardiac arrest, or sudden cardiac death.
Results:
A total of 100 primary prevention children were included (7.1±5.6 years, 59.0% males), with a mean follow-up of 8.6±5.5 years. Overall, 13 (13.0%) patients experienced the primary composite outcome. When only considering events during the 5 first years, Harrel C index was 0.52 (95% CI, 0.27-0.77) for HCM Risk-Kids (≥6%) and 0.70 (95% CI, 0.59-0.80) for PRIMaCY (>8.3%), with 1 patient potentially treated by ICD for every 25 ICDs implanted for HCM Risk-Kids and 1 for every 14 ICDs implanted for PRIMaCY. When risk scores were repeated and all primary outcomes during follow-up were considered, 12 of 13 (92.3%) events were correctly identified using both risk scores, with 1 patient potentially treated by ICD for every 5.6 ICDs implanted for HCM Risk-Kids and 1 for every 5.3 ICDs implanted for PRIMaCY. Among 44 (44.0%) patients implanted with an ICD, all primary prevention patients who had ≥1 appropriate ICD therapy during follow-up had an HCM Risk-Kids ≥6% and PRIMaCY >8.3% at implantation.
Conclusions:
In this independent evaluation, our findings suggest imperfect discrimination between low and high-risk patients using the HCM Risk-Kids and PRIMaCY risk scores, with predicted risks tending to be overestimated compared with the actual observed events. The performance or risk scores was substantially improved by periodic reassessment during follow-up.
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