{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T19:36:30Z","timestamp":1777923390700,"version":"3.51.4"},"reference-count":23,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2023,11,23]],"date-time":"2023-11-23T00:00:00Z","timestamp":1700697600000},"content-version":"vor","delay-in-days":22,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001279","name":"Great Ormond Street Hospital Children\u2019s Charity","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001279","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Max\u2019s Foundation"},{"name":"Medical Research Council Clinical"},{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Clinical Academic Research Partnership","award":["MR\/T024062\/1"],"award-info":[{"award-number":["MR\/T024062\/1"]}]},{"DOI":"10.13039\/501100019256","name":"NIHR GOSH BRC","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100019256","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>The validated HCM Risk-Kids model provides accurate individualized estimates of sudden cardiac death risk in children with hypertrophic cardiomyopathy (HCM). A second validated model, PRIMaCY, also provides individualized estimates of risk, but its performance and clinical impact has not been independently investigated. The aim of this study was to investigate the clinical impact of using the PRIMaCY sudden cardiac death (SCD) risk model in childhood HCM.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>The estimated 5-year SCD risk was calculated for children meeting diagnostic criteria for HCM in a large single-centre cohort using PRIMaCY (clinical and genetic) and HCM Risk-Kids model, and model performance was assessed. Three hundred one patients [median age 10 (interquartile range 4\u201314)] were followed up for an average of 4.9 (\u00b13.8) years, during which 30 (10.0%) reached the SCD or equivalent event endpoint. Harrell\u2019s C-statistic for the clinical and genetic models was 0.66 [95% confidence interval (CI) 0.52\u20130.8] and 0.66 (95% CI 0.54\u20130.80) with a calibration slope of 0.19 (95% CI 0.04\u20130.54) and 0.26 (95% CI \u22120.03\u20130.62), respectively. The number needed to treat to potentially treat one life-threatening arrhythmia for the PRIMaCY clinical, PRIMaCY genetic, and HCM Risk-Kids models was 13.7, 14.5, and 9.4, respectively.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Although PRIMaCY has a similar discriminatory ability to that reported for HCM Risk-Kids, estimated risk estimates did not correlate well with observed risk. A higher proportion of patients met implantable cardioverter-defibrillator thresholds using PRIMaCY model compared with HCM Risk-Kids. This has important clinical implications as these patients will be exposed to a lifetime risk of complications and inappropriate therapies.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/europace\/euad330","type":"journal-article","created":{"date-parts":[[2023,11,23]],"date-time":"2023-11-23T18:06:02Z","timestamp":1700762762000},"source":"Crossref","is-referenced-by-count":12,"title":["Performance of the PRIMaCY sudden death risk prediction model for childhood hypertrophic cardiomyopathy: implications for implantable cardioverter-defibrillator decision-making"],"prefix":"10.1093","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6355-9280","authenticated-orcid":false,"given":"Gabrielle","family":"Norrish","sequence":"first","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Zayed Centre for Research, Great Ormond Street Hospital , Great Ormond Street, London, WC1N 4JH , UK"},{"name":"Institute of Cardiovascular Sciences, University College London , 62 Huntley St, London, WC1E 6DD , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8595-7212","authenticated-orcid":false,"given":"Alexandros","family":"Protonotarios","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Sciences, University College London , 62 Huntley St, London, WC1E 6DD , UK"},{"name":"St Bartholomew\u2019s Centre for Inherited Cardiovascular Diseases, St Bartholomew\u2019s Hospital , London , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7205-3900","authenticated-orcid":false,"given":"Maria","family":"Stec","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Zayed Centre for Research, Great Ormond Street Hospital , Great Ormond Street, London, WC1N 4JH , UK"},{"name":"1st Department of Cardiology, Faculty of Medical Sciences in Katowice, Medical University of Silesia , Katowice , Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0254-0217","authenticated-orcid":false,"given":"Olga","family":"Boleti","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Zayed Centre for Research, Great Ormond Street Hospital , Great Ormond Street, London, WC1N 4JH , UK"},{"name":"Institute of Cardiovascular Sciences, University College London , 62 Huntley St, London, WC1E 6DD , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ella","family":"Field","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Zayed Centre for Research, Great Ormond Street Hospital , Great Ormond Street, London, WC1N 4JH , UK"},{"name":"Institute of Cardiovascular Sciences, University College London , 62 Huntley St, London, WC1E 6DD , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2612-1014","authenticated-orcid":false,"given":"Elena","family":"Cervi","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Zayed Centre for Research, Great Ormond Street Hospital , Great Ormond Street, London, WC1N 4JH , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3383-3984","authenticated-orcid":false,"given":"Perry M","family":"Elliott","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Sciences, University College London , 62 Huntley St, London, WC1E 6DD , UK"},{"name":"St Bartholomew\u2019s Centre for Inherited Cardiovascular Diseases, St Bartholomew\u2019s Hospital , London , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0014-9927","authenticated-orcid":false,"given":"Juan P","family":"Kaski","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Zayed Centre for Research, Great Ormond Street Hospital , Great Ormond Street, London, WC1N 4JH , UK"},{"name":"Institute of Cardiovascular Sciences, University College London , 62 Huntley St, London, WC1E 6DD , UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2023,11,23]]},"reference":[{"key":"2023112314222312800_euad330-B1","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1093\/eurheartj\/ehy798","article-title":"Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom","volume":"40","author":"Norrish","year":"2019","journal-title":"Eur Heart J"},{"key":"2023112314222312800_euad330-B2","doi-asserted-by":"crossref","first-page":"1559","DOI":"10.1093\/europace\/euz118","article-title":"A validation study of the European Society of Cardiology 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