{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T09:34:19Z","timestamp":1793698459376,"version":"4.5.1"},"reference-count":23,"publisher":"American Medical Association (AMA)","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Cardiol"],"published-print":{"date-parts":[[2024,11,1]]},"abstract":"\n Importance<\/jats:title>\n 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.<\/jats:p>\n <\/jats:sec>\n \n Objective<\/jats:title>\n To examine the prognostic significance of LGE in patients with HCM who are younger than 21 years.<\/jats:p>\n <\/jats:sec>\n \n Design, Setting, and Participants<\/jats:title>\n 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.<\/jats:p>\n <\/jats:sec>\n \n Exposure<\/jats:title>\n Natural history of HCM.<\/jats:p>\n <\/jats:sec>\n \n Main Outcome and Measures<\/jats:title>\n 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.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n 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;\n P<\/jats:italic>\n \u2009&amp;lt;\u2009.001). 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.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions and Relevance<\/jats:title>\n 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.<\/jats:p>\n <\/jats:sec>","DOI":"10.1001\/jamacardio.2024.2824","type":"journal-article","created":{"date-parts":[[2024,9,25]],"date-time":"2024-09-25T17:39:30Z","timestamp":1727285970000},"page":"1001","source":"Crossref","is-referenced-by-count":31,"title":["Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy"],"prefix":"10.1001","volume":"9","author":[{"given":"Raymond H.","family":"Chan","sequence":"first","affiliation":[{"name":"Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurine","family":"van der Wal","sequence":"additional","affiliation":[{"name":"Department of Primary and Long-term Care, University of Groningen, Groningen, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela","family":"Liberato","sequence":"additional","affiliation":[{"name":"Heart Institute, University of S\u00e3o Paulo Medical School, S\u00e3o Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ethan","family":"Rowin","sequence":"additional","affiliation":[{"name":"Hypertrophic Cardiomyopathy Center, Lahey Hospital and Medical Center, Burlington, 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Dallas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Valsangiacomo-Buechel","sequence":"additional","affiliation":[{"name":"University Children\u2019s Hospital, Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iacopo","family":"Olivotto","sequence":"additional","affiliation":[{"name":"Careggi University Hospital, Florence, Italy"},{"name":"Meyer Children\u2019s Hospital IRCCS, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derek","family":"Wong","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Eastern Ontario, Ottawa, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cordula","family":"Wolf","sequence":"additional","affiliation":[{"name":"German Heart Center Munich, Department of Congenital Heart Defects and Pediatric Cardiology, Technical University of Munich, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heynric","family":"Grotenhuis","sequence":"additional","affiliation":[{"name":"Wilhelmina Children\u2019s Hospital, University Medical Center Utrecht, Utrecht, the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten","family":"Rickers","sequence":"additional","affiliation":[{"name":"University Heart Center, Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kan","family":"Hor","sequence":"additional","affiliation":[{"name":"Nationwide Children\u2019s Hospital, Columbus, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Rutz","sequence":"additional","affiliation":[{"name":"Service of Cardiology, Lausanne University Hospital, University of Lausanne, Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shelby","family":"Kutty","sequence":"additional","affiliation":[{"name":"Division 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