{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:41:57Z","timestamp":1787499717690,"version":"build-2736575974"},"reference-count":22,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2019,6,3]],"date-time":"2019-06-03T00:00:00Z","timestamp":1559520000000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000274","name":"British Heart Foundation","doi-asserted-by":"publisher","award":["FS\/16\/72\/32270"],"award-info":[{"award-number":["FS\/16\/72\/32270"]}],"id":[{"id":"10.13039\/501100000274","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Max\u2019s Foundation"},{"name":"Great Ormond Street Hospital Children\u2019s Charity"},{"DOI":"10.13039\/100006662","name":"NIHR","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006662","id-type":"DOI","asserted-by":"publisher"}]},{"name":"GOSH BRC"},{"name":"NHS"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Sudden cardiac death (SCD) is the most common cause of death in children with hypertrophic cardiomyopathy (HCM). The European Society of Cardiology (ESC) recommends consideration of an implantable cardioverter-defibrillator (ICD) if two or more clinical risk factors (RFs) are present, but this approach to risk stratification has not been formally validated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>Four hundred and eleven paediatric HCM patients were assessed for four clinical RFs in accordance with current ESC recommendations: severe left ventricular hypertrophy, unexplained syncope, non-sustained ventricular tachycardia, and family history of SCD. The primary endpoint was a composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate ICD therapy, or sustained ventricular tachycardia), defined as a major arrhythmic cardiac event (MACE). Over a follow-up period of 2890 patient years (median 5.5\u2009years), MACE occurred in 21 patients (7.5%) with 0 RFs, 19 (16.8%) with 1 RFs, and 3 (18.8%) with 2 or more RFs. Corresponding incidence rates were 1.13 [95% confidence interval (CI) 0.7\u20131.73], 2.07 (95% CI 1.25\u20133.23), and 2.52 (95% CI 0.53\u20137.35) per 100 patient years at risk. Patients with two or more RFs did not have a higher incidence of MACE (log-rank test P\u2009=\u20090.34), with a positive and negative predictive value of 19% and 90%, respectively. The C-statistic was 0.62 (95% CI 0.52\u20130.72) at 5\u2009years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The incidence of MACE is higher for patients with increasing numbers of clinical RFs. However, the current ESC guidelines have a low ability to discriminate between high- and low-risk individuals.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/europace\/euz118","type":"journal-article","created":{"date-parts":[[2019,4,5]],"date-time":"2019-04-05T16:52:58Z","timestamp":1554483178000},"page":"1559-1565","source":"Crossref","is-referenced-by-count":59,"title":["A validation study of the European Society of Cardiology guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy"],"prefix":"10.1093","volume":"21","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, Great Ormond Street Hospital, Great Ormond Street, London, UK"},{"name":"Institute of Cardiovascular Sciences University College London, London, UK"},{"name":"ERN GUARD-HEART (European Reference Network for Rare and Complex Diseases of the Heart)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tao","family":"Ding","sequence":"additional","affiliation":[{"name":"Department of Statistical Science, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ella","family":"Field","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London, UK"},{"name":"Institute of Cardiovascular Sciences University College London, London, UK"},{"name":"ERN GUARD-HEART (European Reference Network for Rare and Complex Diseases of the Heart)"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"McLeod","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Royal Hospital for Children, Glasgow, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Ilina","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Royal Hospital for Children, Glasgow, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graham","family":"Stuart","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, University Hospitals Bristol NHS Foundation Trust, Bristol, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vinay","family":"Bhole","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Birmingham Women and Children\u2019s NHS Foundation Trust, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orhan","family":"Uzun","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, University Hospital of Wales, Cardiff, 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UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sujeev","family":"Mathur","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Evelina London Children\u2019s Hospital, Guys and St Thomas\u2019 NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tara","family":"Bharucha","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khoon Li","family":"Kok","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, University Hospital Southampton NHS Foundation Trust, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satish","family":"Adwani","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Oxford University Hospitals NHS Foundation Trust, Oxford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline B","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Alder Hey Children\u2019s Hospital, Liverpool, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zdenka","family":"Reinhardt","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, The Freeman Hospital, Newcastle, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rumana Z","family":"Omar","sequence":"additional","affiliation":[{"name":"Department of Statistical Science, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan Pablo","family":"Kaski","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London, UK"},{"name":"Institute of Cardiovascular Sciences University College London, London, 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