{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T15:38:24Z","timestamp":1791041904512,"version":"4.1.0"},"reference-count":52,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2025,10,15]],"date-time":"2025-10-15T00:00:00Z","timestamp":1760486400000},"content-version":"vor","delay-in-days":641,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,1,13]],"date-time":"2024-01-13T00:00:00Z","timestamp":1705104000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005302","name":"Alexander S. 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Onassis Public Benefit Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005302","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001279","name":"Great Ormond Street Hospital Charity","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001279","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/T024062\/1"],"award-info":[{"award-number":["MR\/T024062\/1"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>This study aimed to describe the natural history and predictors of all-cause mortality and sudden cardiac death (SCD)\/equivalent events in children with a RASopathy syndrome and hypertrophic cardiomyopathy (HCM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>This is a retrospective cohort study from 14 paediatric cardiology centres in the United Kingdom and Ireland. We included children &amp;lt;18 years with HCM and a clinical and\/or genetic diagnosis of a RASopathy syndrome [Noonan syndrome (NS), NS with multiple lentigines (NSML), Costello syndrome (CS), cardiofaciocutaneous syndrome (CFCS), and NS with loose anagen hair (NS-LAH)]. One hundred forty-nine patients were recruited [111 (74.5%) NS, 12 (8.05%) NSML, 6 (4.03%) CS, 6 (4.03%) CFCS, 11 (7.4%) Noonan-like syndrome, and 3 (2%) NS-LAH]. NSML patients had higher left ventricular outflow tract (LVOT) gradient values [60 (36\u201380) mmHg, P = 0.004]. Over a median follow-up of 197.5 [inter-quartile range (IQR) 93.58\u2013370] months, 23 patients (15.43%) died at a median age of 24.1 (IQR 5.6\u2013175.9) months. Survival was 96.45% [95% confidence interval (CI) 91.69\u201398.51], 90.42% (95% CI 84.04\u201394.33), and 84.12% (95% CI 75.42\u201389.94) at 1, 5, and 10 years, respectively, but this varied by RASopathy syndrome. RASopathy syndrome, symptoms at baseline, congestive cardiac failure (CCF), non-sustained ventricular tachycardia (NSVT), and maximal left ventricular wall thickness were identified as predictors of all-cause mortality on univariate analysis, and CCF, NSVT, and LVOT gradient were predictors for SCD or equivalent event.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>These findings highlight a distinct category of patients with Noonan-like syndrome with a milder HCM phenotype but significantly worse survival and identify potential predictors of adverse outcome in patients with RASopathy-related HCM.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ehf2.14637","type":"journal-article","created":{"date-parts":[[2024,1,13]],"date-time":"2024-01-13T07:56:55Z","timestamp":1705132615000},"page":"923-936","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Natural History and Outcomes in Paediatric RASopathy-Associated Hypertrophic Cardiomyopathy"],"prefix":"10.1093","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0254-0217","authenticated-orcid":false,"given":"Olga","family":"Boleti","sequence":"first","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Department of Cardiology, Great Ormond Street Hospital , London ,","place":["UK"]},{"name":"Institute of Cardiovascular Science, University College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabrielle","family":"Norrish","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Department of Cardiology, Great Ormond Street Hospital , London ,","place":["UK"]},{"name":"Institute of Cardiovascular Science, University College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ella","family":"Field","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Department of Cardiology, Great Ormond Street Hospital , London ,","place":["UK"]},{"name":"Institute of Cardiovascular Science, University College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen","family":"Dady","sequence":"additional","affiliation":[{"name":"Centre for Inherited Cardiovascular Diseases, Department of Cardiology, Great Ormond Street Hospital , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim","family":"Summers","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Science, University College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gauri","family":"Nepali","sequence":"additional","affiliation":[{"name":"The Heart Unit, Birmingham Children's Hospital , Birmingham ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vinay","family":"Bhole","sequence":"additional","affiliation":[{"name":"The Heart Unit, Birmingham Children's Hospital , Birmingham ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orhan","family":"Uzun","sequence":"additional","affiliation":[{"name":"Children's Heart Unit, University Hospital of Wales , Cardiff ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amos","family":"Wong","sequence":"additional","affiliation":[{"name":"Children's Heart Unit, University Hospital of Wales , Cardiff ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piers E. 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