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Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000833","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000282","name":"Sir Jules Thorn Charitable Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000282","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>To characterize the phenotype, clinical outcomes and rate of disease progression in patients with early-stage non-ischaemic cardiomyopathy (early-NICM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>We conducted a prospective observational cohort study of patients with early-NICM assessed by late gadolinium enhancement cardiovascular magnetic resonance (CMR). Cases were classified into the following subgroups: isolated left ventricular dilatation (early-NICM H\u2212\/D+), non-dilated left ventricular cardiomyopathy (early-NICM H+\/D\u2212), or early dilated cardiomyopathy (early-NICM H+\/D+). Clinical follow-up for major adverse cardiovascular events (MACE) included non-fatal life-threatening arrhythmia, unplanned cardiovascular hospitalization or cardiovascular death. A subset of patients (n\u2009=\u2009119) underwent a second CMR to assess changes in cardiac structure and function. Of 254 patients with early-NICM (median age 46\u2009years [interquartile range 36\u201358], 94 [37%] women, median left ventricular ejection fraction [LVEF] 55% [52\u201359]), myocardial fibrosis was present in 65 (26%). There was no difference in the prevalence of fibrosis between subgroups (p\u2009=\u20090.90), however fibrosis mass was lowest in early-NICM H\u2212\/D+, higher in early-NICM H+\/D\u2212 and highest in early-NICM H+\/D+ (p\u2009=\u20090.03). Over a median follow-up of 7.9 (5.5\u201310.0) years, 28 patients (11%) experienced MACE. Non-sustained ventricular tachycardia (hazard ratio [HR] 5.1, 95% confidence interval [CI] 2.36\u201311.00, p\u2009&amp;lt;\u20090.001), myocardial fibrosis (HR 3.77, 95% CI 1.73\u20138.20, p\u2009&amp;lt;\u20090.001) and diabetes mellitus (HR 5.12, 95% CI 1.73\u201315.18, p\u2009=\u20090.003) were associated with MACE in a multivariable model. Only 8% of patients progressed from early-NICM to dilated cardiomyopathy with LVEF &amp;lt;50% over a median of 16 (11\u201334) months.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Early-NICM is not benign. Fibrosis develops early in the phenotypic course. In-depth characterization enhances risk stratification and might aid clinical management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ejhf.3037","type":"journal-article","created":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T05:00:09Z","timestamp":1695186009000},"page":"2050-2059","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Phenotype, Outcomes and Natural History of Early-Stage Non-Ischaemic Cardiomyopathy"],"prefix":"10.1093","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1164-5993","authenticated-orcid":false,"given":"Daniel J.","family":"Hammersley","sequence":"first","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard E.","family":"Jones","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]},{"name":"Anglia Ruskin Medical School, UK , Cambridge ,","place":["UK"]},{"name":"Essex Cardiothoracic Centre , Basildon ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth","family":"Owen","sequence":"additional","affiliation":[{"name":"London School of Hygiene and Tropical Medicine , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lukas","family":"Mach","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amrit S.","family":"Lota","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zohya","family":"Khalique","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"De Marvao","sequence":"additional","affiliation":[{"name":"Department of Women and Children's Health, King's College London , London ,","place":["UK"]},{"name":"British Heart Foundation Centre of Research Excellence, 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,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Won Young","family":"Yoon","sequence":"additional","affiliation":[{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suprateeka","family":"Talukder","sequence":"additional","affiliation":[{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riya","family":"Shah","sequence":"additional","affiliation":[{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Resham","family":"Baruah","sequence":"additional","affiliation":[{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaushik","family":"Guha","sequence":"additional","affiliation":[{"name":"Portsmouth Hospitals NHS Trust , Portsmouth ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonis","family":"Pantazis","sequence":"additional","affiliation":[{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. John","family":"Baksi","sequence":"additional","affiliation":[{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Gregson","sequence":"additional","affiliation":[{"name":"London School of Hygiene and Tropical Medicine , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John G.F.","family":"Cleland","sequence":"additional","affiliation":[{"name":"British Heart Foundation Centre of Research Excellence, School of Cardiovascular and Metabolic Health, University of Glasgow , Glasgow ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Upasana","family":"Tayal","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dudley J.","family":"Pennell","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James S.","family":"Ware","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]},{"name":"MRC London Institute of Medical Sciences, Imperial College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian P.","family":"Halliday","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjay K.","family":"Prasad","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]},{"name":"Royal Brompton & Harefield Hospital, Guy's and St Thomas' NHS Foundation Trust , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,10,11]]},"reference":[{"key":"2026010913115533200_ejhf3037-bib-0001","doi-asserted-by":"crossref","first-page":"1850","DOI":"10.1093\/eurheartj\/ehv727","article-title":"Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice: A position statement of the ESC Working Group on Myocardial and Pericardial Diseases","volume":"37","author":"Pinto","year":"2016","journal-title":"Eur Heart J"},{"key":"2026010913115533200_ejhf3037-bib-0002","doi-asserted-by":"crossref","first-page":"3503","DOI":"10.1093\/eurheartj\/ehad194","article-title":"2023 ESC Guidelines for the management of cardiomyopathies","volume":"44","author":"Arbelo","year":"2023","journal-title":"Eur Heart J"},{"key":"2026010913115533200_ejhf3037-bib-0003","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCHEARTFAILURE.117.004682","article-title":"Prevalence of pathogenic gene mutations and prognosis do not differ in isolated left ventricular dysfunction compared with dilated cardiomyopathy","volume":"11","author":"Hazebroek","year":"2018","journal-title":"Circ Heart Fail"},{"key":"2026010913115533200_ejhf3037-bib-0004","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1002\/ejhf.2333","article-title":"2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: Developed by the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC). 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