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

Chan RH, Van Der Wal L, Liberato G, Rowin E, Soslow J, Maskatia S, Chan S, Shah A, Fogel M, Hernandez L, Anwar S, Voges I, Carlsson M, Buddhe S, Laser KT, Greil G, Valsangiacomo-Buechel E, Olivotto I, Wong D, Wolf C, Grotenhuis H, Rickers C, Hor K, Rutz T, Kutty S, Samyn M, Johnson T, Hasbani K, Moore JP, Sieverding L, Detterich J, Parra R, Chungsomprasong P, Toro-Salazar O, Roest AA, Dittrich S, Brun H, Spinner J, Lai W, Dyer A, Jablonowsk R, Meierhofer C, Gabbert D, Prsa M, Patel JK, Hornung A, Diab SG, House AV, Rakowski H, Benson L, Maron MS, Grosse-Wortmann L (2024)

Publication Type: Journal article

Publication year: 2024

Journal

Book Volume: 9

Journal Issue: 11

DOI: 10.1001/jamacardio.2024.2824

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.

Authors with CRIS profile

Involved external institutions

Baylor College of Medicine United States (USA) (US) Lausanne University Hospital / Centre hospitalier universitaire vaudois (CHUV) Switzerland (CH) Indiana University – Purdue University Indianapolis United States (USA) (US) University of Texas at Austin United States (USA) (US) Siriraj Hospital Thailand (TH) Connecticut Children's Medical Center United States (USA) (US) Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet Norway (NO) University of Oklahoma United States (USA) (US) Doernbecher Children's Hospital United States (USA) (US) Cardiovascular Research Foundation United States (USA) (US) University of Texas Southwestern Medical Center (UT Southwestern) United States (USA) (US) Lund University / Lunds universitet Sweden (SE) Technische Universität München (TUM) Germany (DE) Universitätsklinikum Tübingen Germany (DE) University Health Network (UHN) Canada (CA) Lahey Hospital & Medical Center United States (USA) (US) University of Washington United States (USA) (US) Universitätsklinikum Schleswig-Holstein (UKSH) Germany (DE) The Hospital for Sick Children (SickKids) Canada (CA) Children's Hospital of Philadelphia United States (USA) (US) Herz- und Diabeteszentrum Nordrhein-Westfalen Germany (DE) Children's Hospital of Eastern Ontario (CHEO) / Centre hospitalier pour enfants de l'est de l'Ontario Canada (CA) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) Nationwide Children's Hospital United States (USA) (US) Ronald Reagan UCLA Medical Center United States (USA) (US) Keck School of Medicine of USC United States (USA) (US) Pontificia Universidad Católica de Chile Chile (CL) Leiden University Medical Center Netherlands (NL) Children's Hospital of Orange County (CHOC) United States (USA) (US) Joe DiMaggio Children's Hospital United States (USA) (US) University of California San Francisco (UCSF) United States (USA) (US) Universitäts-Kinderspital Zürich Switzerland (CH) Careggi University Hospital / Azienda Ospedaliero Universitaria Careggi Italy (IT) Wilhelmina Kinderziekenhuis Netherlands (NL) Medical College of Wisconsin (MCW) United States (USA) (US) University of Groningen / Rijksuniversiteit Groningen Netherlands (NL) University of São Paulo / Universidade de São Paulo (USP) Brazil (BR) Vanderbilt University United States (USA) (US) Lucile Packard Children's Hospital (LPCH) United States (USA) (US) Children's Mercy Hospital United States (USA) (US)

How to cite

APA:

Chan, R.H., Van Der Wal, L., Liberato, G., Rowin, E., Soslow, J., Maskatia, S.,... Grosse-Wortmann, L. (2024). Myocardial Scarring and Sudden Cardiac Death in Young Patients with Hypertrophic Cardiomyopathy: A Multicenter Cohort Study. JAMA Cardiology, 9(11). https://doi.org/10.1001/jamacardio.2024.2824

MLA:

Chan, Raymond H., et al. "Myocardial Scarring and Sudden Cardiac Death in Young Patients with Hypertrophic Cardiomyopathy: A Multicenter Cohort Study." JAMA Cardiology 9.11 (2024).

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