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Multicenter Study
. 2024 Nov 1;9(11):1001-1008.
doi: 10.1001/jamacardio.2024.2824.

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

Raymond H Chan  1 , Laurine van der Wal  2 , Gabriela Liberato  3 , Ethan Rowin  4 , Jonathan Soslow  5 , Shiraz Maskatia  6   7 , Sherwin Chan  8 , Amee Shah  9 , Mark Fogel  10 , Lazaro Hernandez  11 , Shafkat Anwar  12   13 , Inga Voges  14   15 , Marcus Carlsson  16 , Sujatha Buddhe  17 , Kai Thorsten Laser  18 , Gerald Greil  19 , Emanuela Valsangiacomo-Buechel  20 , Iacopo Olivotto  21   22 , Derek Wong  23 , Cordula Wolf  24 , Heynric Grotenhuis  25 , Carsten Rickers  26 , Kan Hor  27 , Tobias Rutz  28 , Shelby Kutty  29   30 , Margaret Samyn  31 , Tiffanie Johnson  32 , Keren Hasbani  33 , Jeremy P Moore  34 , Ludger Sieverding  35 , Jon Detterich  36   37 , Rodrigo Parra  38 , Paweena Chungsomprasong  39 , Olga Toro-Salazar  40 , Arno A W Roest  41 , Sven Dittrich  42 , Henrik Brun  43 , Joseph Spinner  7 , Wyman Lai  44 , Adrian Dyer  19   45 , Robert Jablonowsk  46 , Christian Meierhofer  24 , Dominik Gabbert  14 , Milan Prsa  28 , Jyoti Kandlikar Patel  32 , Andreas Hornung  35 , Simone Goa Diab  43 , Aswathy Vaikom House  47 , Harry Rakowski  1 , Lee Benson  48 , Martin S Maron  4 , Lars Grosse-Wortmann  49
Affiliations
Multicenter Study

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

Raymond H Chan et al. JAMA Cardiol. .

Erratum in

  • Error in Byline.
    [No authors listed] [No authors listed] JAMA Cardiol. 2025 Mar 1;10(3):300. doi: 10.1001/jamacardio.2024.5630. JAMA Cardiol. 2025. PMID: 39878992 Free PMC article. No abstract available.

Abstract

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 < .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.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr S. Chan reported grants from Jazz Pharmaceuticals and Hyperfine Inc and personal fees from GE Healthcare and Deepsight Technologies outside the submitted work. Dr Rowin reported research funding from Pfizer and iRhythm. Dr Fogel reported a US National Institutes of Health (NIH) grant in myocardial scarring. Dr Carlsson reported grants from the Swedish Heart and Lung Foundation and from the Agreement on Medical Education and Research Skane during the conduct of the study; grants from the Swedish research council outside the submitted work; and personal fees from serving as a contractor for the NIH outside the submitted work. Dr Olivotto reported personal fees from Bristol Myers Squibb, Cytokinetics, Rocket Pharma, and Tenaya; grants from Amicus, Menarini International, and Chiesi outside the submitted work; and serving on the advisory board of and receiving research grants from Bristol Myers Squibb-Myokardia, Cytokinetics, Sanofi Genzyme, Shire Takeda, Amicus, Menarini International, Tenaya, Lexeo, Edgewise, Lexicon, and Rocket Pharma. Dr Wolf reported a small fee per patient entered in eCRF from Oregon Health and Science University during the conduct of the study. Dr Hor reported consultancy or advisory roles with Bayer AG, Bristol Myers Squibb, Capricor Therapeutics, Daiichi Sankyo, PTC Therapeutics, Revidia Therapeutics, Sarepta Therapeutics, Stealth Biotherapeutics, Vertex Pharmaceuticals, Wave Life Science, Blade Therapeutics, FibroGen, and Foresee Pharmaceuticals; research funding from Sarepta Therapeutics; and speakers’ bureau activity for NS Pharma and PTC Therapeutics. Dr Sieverding reported grants from Stiftung zur Förderung der Erforschung von Zivilisationserkarnkungen both during the conduct of the study and outside the submitted work. Dr Hornung reported grants from Stiftung zur Förderung der Erforschung von Zivilisationserkrankungen both during the conduct of the study and outside the submitted work. Dr Grosse-Wortmann reported grants from the Children’s Cardiomyopathy Foundation during the conduct of the study and personal fees from Siemens Healthineers outside the submitted work. No other disclosures were reported.

Figures

Figure.
Figure.. Kaplan-Meier Curves
Kaplan-Meier curves comparing the sudden cardiac death–free survival between patients with and without late gadolinium enhancement (LGE) (A) and between patients without vs with <10% and ≥10% LGE, respectively (B), relative to total left ventricular myocardium.
See this image and copyright information in PMC

References

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