LGE CMR Can Predict Heart Risk in Children with Hypertrophic Cardiomyopathy

A multi-institutional team including Jon Detterich, MD, Principal Investigator in the Heart Institute at Children’s Hospital Los Angeles, investigated if adding cardiovascular magnetic resonance imaging (MRI) with late gadolinium enhancement (LGE) to current risk assessment models could more effectively predict sudden cardiac death in children. While current models are compiled from patient test scores and echocardiograms, LGE can capture what is happening in specific regions of the heart. The results were published in JAMA Cardiology.

To find out if LGE predicted HCM risk in children, the team conducted a seven-year retrospective cohort study of 700 patients with hypertrophic cardiomyopathy at 37 sites around the world. Patients with greater LGE also had lower left ventricular ejection fraction and larger left atrial diameters. Patients with 10% or more LGE as a percent of total myocardial mass had a higher risk of sudden cardiac death at an unadjusted hazard ratio of 2.19. “The larger amount of late gadolinium enhancement indicates larger areas of scarring, which is predictive of worse cardiac function and sudden cardiac death," says Dr. Detterich.

Adding LGE as a prognostic indicator to the existing risk stratification models—HCM Risk-Kids and PRIMaCY score—improved their performance. “The higher the LGE, the more predictive the score was at estimating patient risk and outcomes,” Dr. Detterich noted. “This method can help us to distinguish the high-risk patients who would benefit from more immediate and intensive monitoring, therapies, and interventions—like an implantable defibrillator or surgery—from the patients at low risk of cardiac issues,” says Dr. Detterich.