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Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy: A Multicenter Cohort

Raymond H Chan1, Laurine van der Wal2, Gabriela Liberato3

  • 1Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.

JAMA Cardiology
|September 25, 2024
PubMed

Insights

Late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) imaging predicts sudden cardiac death (SCD) in children and adolescents with hypertrophic cardiomyopathy (HCM). This finding improves risk stratification for these young patients.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Pediatrics

Background:

  • Predicting sudden cardiac death (SCD) in pediatric hypertrophic cardiomyopathy (HCM) remains challenging.
  • Late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR) imaging is linked to SCD in adult HCM patients.

Purpose of the Study:

  • To assess the prognostic value of LGE in predicting SCD in patients under 21 years with HCM.
  • To determine if LGE improves existing risk stratification models for pediatric HCM.

Main Methods:

  • A multicenter, retrospective cohort study involving 700 patients (<21 years) with HCM who underwent CMR.
  • Follow-up for SCD and surrogate events, with LGE presence and burden quantified.
  • Kaplan-Meier and Cox proportional hazards models were used for analysis.

Main Results:

  • LGE was present in 32.9% of patients and associated with a higher risk of SCD.
  • A higher LGE burden (≥10%) significantly increased SCD risk (HR, 2.19).
  • Incorporating LGE burden improved the predictive performance of established risk scores like HCM Risk-Kids and Precision Medicine in Cardiomyopathy.

Conclusions:

  • Quantitative LGE is a significant risk factor for SCD in pediatric HCM patients.
  • LGE assessment enhances the accuracy of risk stratification for young individuals with HCM.
  • This highlights the importance of LGE in managing pediatric HCM and preventing SCD.
Abstract

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