Gadolinium Enhancement Improves Sudden Cardiac Death in kids with Hypertrophic Cardiomyopathy: Study
Researchers have found that late gadolinium enhancement (LGE) by cardiovascular magnetic resonance imaging (CMR) is an important predictor of sudden cardiac death in children and adolescents suffering from hypertrophic cardiomyopathy (HCM). This finding suggests the inadequacy of currently used methods to predict sudden cardiac death among this younger population, where the risk stratification of patients is a challenging issue. A study was recently published in the journal JAMA Cardiology by Raymond H. and colleagues.
Hypertrophic cardiomyopathy, a genetic heart disorder characterised by hypertrophied walls of the heart, typically predisposes patients to serious arrhythmias and sudden cardiac death (SCD). This study enrolled 700 patients with HCM less than 21 years old who were undergoing CMR imaging from April 2015 to September 2022. Overall, the cohort was predominantly males (74.0%) with a median age of 14.8 years (IQR, 11.9-17.4). Follow-up was performed in all participants for about 2 years to monitor events for SCD or resuscitated cardiac arrests and appropriate defibrillator discharges.
The study established several important findings:
• LGE was detected in 230 patients (32.9%), with a mean burden of 5.9% (SD, 7.3%) of the left ventricular myocardium.
• SCD or equivalent events occurred in 35 patients (5.0%) during the follow-up period.
• Patients whose myocardium was covered by LGE in 10% or greater were at high risk for SCD; the unadjusted hazard ratio was 2.19 (95% CI, 1.59-3.02; P < .001).
• The original HCM Risk-Kids score was at 0.66 (95% CI, 0.58 to 0.75); with the inclusion of LGE, the score was at 0.73 (95% CI, 0.66 to 0.81).
• Similar increases were noted in the Precision Medicine in Cardiomyopathy score, which, when combined with the LGE burden, rose from 0.68 (95% CI, 0.49 to 0.77) to 0.73 (95% CI, 0.64 to 0.82).
The researchers concluded that this study identifies a prognostic value of LGE in pediatric and adolescent patients with hypertrophic cardiomyopathy, especially if quantitative LGE analysis is used as the strongest predictor for death due to arrhythmias.
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