Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy
Authors: Chan Raymond H. , van der Wal Laurine , Liberato Gabriela , Rowin Ethan , Soslow Jonathan , Maskatia Shiraz , Chan Sherwin , Shah Amee , Fogel Mark , Hernandez Lazaro , Anwar Shafkat , Voges Inga , Carlsson Marcus , Buddhe Sujatha , Laser Kai Thorsten , Greil Gerald , Valsangiacomo-Buechel Emanuela , Olivotto Iacopo , Wong Derek , Wolf Cordula , Grotenhuis Heynric , Rickers Carsten , Hor Kan , Rutz Tobias , Kutty Shelby , Samyn Margaret , Johnson Tiffanie , Hasbani Keren , Moore Jeremy P. , Sieverding Ludger , Detterich Jon , Parra Rodrigo , Chungsomprasong Paweena , Toro-Salazar Olga , Roest Arno A. W. , Dittrich Sven , Brun Henrik , Spinner Joseph , Lai Wyman , Dyer Adrian , Jablonowski Robert , Meierhofer Christian , Gabbert Dominik , Prsa Milan , Patel Jyoti Kandlikar , Hornung Andreas , Diab Simone Goa , House Aswathy Vaikom , Rakowski Harry , Benson Lee , Maron Martin S. , Grosse-Wortmann Lars 
Published: September 2024

Importance

The ability to predict sudden cardiac death (SCD) in children and adolescents with hypertrophic cardiomyopathy (HCM) is currently inadequate. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) imaging is associated with SCD events in adults with HCM.

Objective

To examine the prognostic significance of LGE in patients with HCM who are younger than 21 years.

Design, Setting, and Participants

This multicenter, retrospective cohort study was conducted from April 8, 2015, to September 12, 2022, in patients with HCM who were younger than 21 years and had undergone CMR imaging across multiple sites in the US, Europe, and South America. Observers of CMR studies were masked toward outcomes and demographic characteristics.

Exposure

Natural history of HCM.

Main Outcome and Measures

The primary outcome was SCD and surrogate events, including resuscitated cardiac arrest and appropriate discharges from an implantable defibrillator. Continuous and categorical data are expressed as mean (SD), median (IQR), or number (percentage), respectively. Survivor curves comparing patients with and without LGE were constructed by the Kaplan-Meier method, and likelihood of subsequent clinical events was further evaluated using univariate and multivariable Cox proportional hazards models.

Results

Among 700 patients from 37 international centers, median (IQR) age was 14.8 (11.9-17.4) years, and 518 participants (74.0%) were male. During a median (IQR) [range] follow-up period of 1.9 (0.5-4.1) [0.1-14.8] years, 35 patients (5.0%) experienced SCD or equivalent events. LGE was present in 230 patients (32.9%), which constituted an mean (SD) burden of 5.9% (7.3%) of left ventricular myocardium. The LGE amount was higher in older patients and those with greater left ventricular mass and maximal wall thickness; patients with LGE had lower left ventricular ejection fractions and larger left atrial diameters. The presence and burden of LGE was associated with SCD, even after correcting for existing risk stratification tools. Patients with 10% or more LGE, relative to total myocardium, had a higher risk of SCD (unadjusted hazard ratio [HR], 2.19; 95% CI, 1.59-3.02; P &amp;lt; .001). Furthermore, the addition of LGE burden improved the performance of the HCM Risk-Kids score (before LGE addition: 0.66; 95% CI, 0.58-0.75; after LGE addition: 0.73; 95% CI, 0.66-0.81) and Precision Medicine in Cardiomyopathy score (before LGE addition: 0.68; 95% CI, 0.49-0.77; after LGE addition: 0.73; 95% CI, 0.64-0.82) SCD predictive models.

Conclusions and Relevance

In this retrospective cohort study, quantitative LGE was a risk factor for SCD in patients younger than 21 years with HCM and improved risk stratification.