LATE gadolinium enhancement (LGE) is significantly associated with sudden cardiac death (SCD) in young patients with hypertrophic cardiomyopathy (HCM), and its inclusion enhances risk prediction tools.
Hypertrophic cardiomyopathy (HCM) is a genetic cardiac condition that predisposes children and adolescents to sudden cardiac death (SCD). Identifying high-risk patients is crucial for improving outcomes, but current risk prediction models have limitations. This study investigated whether late gadolinium enhancement (LGE), identified via cardiovascular magnetic resonance (CMR) imaging, could improve risk stratification for SCD in patients under 21 years old.
The study was a multicentre, retrospective cohort analysis of 700 patients from 37 international centres, conducted between 2015 and 2022. Participants, with a median age of 14.8 years, underwent CMR imaging. LGE, indicative of myocardial scarring, was detected in 32.9% of patients, with a mean burden of 5.9% of left ventricular myocardium. Over a median follow-up of 1.9 years, 35 patients (5%) experienced SCD or equivalent events, such as cardiac arrest or appropriate defibrillator discharges. Analysis revealed that LGE presence and burden were significantly associated with higher SCD risk, independent of established clinical parameters. For example, patients with ≥10% LGE had a hazard ratio of 2.19 (95% CI, 1.59-3.02; P < .001) for SCD. Incorporating LGE burden into current risk tools, such as the HCM Risk-Kids score, enhanced their predictive performance, increasing the model’s discriminatory capacity from 0.66 to 0.73.
These findings highlight the value of LGE in evaluating SCD risk in children and adolescents with HCM. Clinically, LGE assessment should be incorporated into routine evaluation of paediatric HCM patients to refine risk stratification and guide targeted interventions. Future research could explore how LGE data integrates with other emerging biomarkers to develop precision medicine approaches for these patients.
Katrina Thornber, EMJ
Reference
Chan RH et al. Myocardial scarring and sudden cardiac death in young patients with hypertrophic cardiomyopathy: a multicenter cohort study. JAMA Cardiol. 2024;9(11):1001-8.
