{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T12:18:25Z","timestamp":1792671505169,"version":"4.5.0"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T00:00:00Z","timestamp":1681689600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cardiovasc. Med."],"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Cardiomyopathy is the fourth most common cause of heart failure. The spectrum of cardiomyopathies may be impacted by changes in environmental factors and the prognosis may be influenced by modern treatment. The aim of this study is to create a prospective clinical cohort, the Sahlgrenska CardioMyoPathy Centre (SCMPC) study, and compare patients with cardiomyopathies in terms of phenotype, symptoms, and survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The SCMPC study was founded in 2018 by including patients with all types of suspected cardiomyopathies. This study included data on patient characteristics, background, family history, symptoms, diagnostic examinations, and treatment including heart transplantation and mechanical circulatory support (MCS). Patients were categorized by the type of cardiomyopathy on the basis of the diagnostic criteria laid down by the European Society of Cardiology (ESC) working group on myocardial and pericardial diseases. The primary outcomes were death, heart transplantation, or MCS, analyzed by Kaplan\u2013Meier and Cox proportional regression, adjusted for age, gender, LVEF and QRS width on ECG in milliseconds.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In all, 461 patients and 73.1% men with a mean age of 53.6\u2009\u00b1\u200916 years were included in the study. The most common diagnosis was dilated cardiomyopathy (DCM), followed by cardiac sarcoidosis and myocarditis. Dyspnea was the most common initial symptom in patients with DCM and amyloidosis, while patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) presented with ventricular arrythmias. Patients with ARVC, left-ventricular non-compaction cardiomyopathy (LVNC), hypertrophic cardiomyopathy (HCM), and DCM had the longest time from the debut of symptoms until inclusion in the study. Overall, 86% of the patients survived without heart transplantation or MCS after 2.5 years. The primary outcome differed among the cardiomyopathies, where the worst prognosis was reported for ARVC, LVNC, and cardiac amyloidosis. In a Cox regression analysis, it was found that ARVC and LVNC were independently associated with an increased risk of death, heart transplantation, or MCS compared with DCM. Further, female gender, a lower LVEF, and a wider QRS width were associated with an increased risk of the primary outcome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The SCMPC database offers a unique opportunity to explore the spectrum of cardiomyopathies over time. There is a large difference in characteristics and symptoms at debut and a remarkable difference in outcome, where the worst prognosis was reported for ARVC, LVNC, and cardiac amyloidosis.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fcvm.2023.1160089","type":"journal-article","created":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T05:39:42Z","timestamp":1681709982000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":9,"title":["Differences in phenotypes, symptoms, and survival in patients with cardiomyopathy\u2014a prospective observational study from the Sahlgrenska CardioMyoPathy Centre"],"prefix":"10.3389","volume":"10","author":[{"given":"C.","family":"Ljungman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Bollano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Rawshani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Nordberg\u00a0Backelin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Dahlberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Valeljung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Bj\u00f6rkenstam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Hjalmarsson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Fu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Mellberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.-E.","family":"Bartfay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. L.","family":"Polte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Andersson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Bergh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,4,17]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1093\/eurheartj\/ehm342","article-title":"Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on myocardial and pericardial diseases","volume":"29","author":"Elliott","year":"2008","journal-title":"Eur Heart J"},{"key":"B2","doi-asserted-by":"publisher","first-page":"1682","DOI":"10.1093\/eurjpc\/zwaa147","article-title":"Burden of heart failure and underlying causes in 195 countries and territories from 1990 to 2017","volume":"28","author":"Bragazzi","year":"2021","journal-title":"Eur J Prev Cardiol"},{"key":"B3","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1002\/ehf2.12224","article-title":"High validity of cardiomyopathy diagnoses in western Sweden (1989\u20132009)","volume":"5","author":"Basic","year":"2018","journal-title":"ESC Heart Fail"},{"key":"B4","doi-asserted-by":"publisher","first-page":"553","DOI":"10.1002\/ejhf.1461","article-title":"Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology","volume":"21","author":"Seferovic","year":"2019","journal-title":"Eur J Heart Fail"},{"key":"B5","doi-asserted-by":"publisher","first-page":"400","DOI":"10.1016\/S0140-6736(16)31713-5","article-title":"Dilated cardiomyopathy","volume":"390","author":"Weintraub","year":"2017","journal-title":"Lancet"},{"key":"B6","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1161\/CIRCHEARTFAILURE.112.969261","article-title":"Diagnosis, treatment, and outcome of giant-cell myocarditis in the era of combined immunosuppression","volume":"6","author":"Kandolin","year":"2013","journal-title":"Circ Heart Fail"},{"key":"B7","doi-asserted-by":"publisher","first-page":"513","DOI":"10.1016\/j.amjcard.2018.10.021","article-title":"Cardiac sarcoidosis","volume":"123","author":"Tan","year":"2019","journal-title":"Am J Cardiol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"314","DOI":"10.1016\/j.jacc.2020.11.031","article-title":"Pathological evidence for SARS-CoV-2 as a cause of myocarditis: JACC review topic of the week","volume":"77","author":"Kawakami","year":"2021","journal-title":"J Am Coll Cardiol"},{"key":"B9","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1161\/CIRCULATIONAHA.119.044056","article-title":"Body mass Index in young women and risk of cardiomyopathy: a long-term follow-up study in Sweden","volume":"141","author":"Robertson","year":"2020","journal-title":"Circulation"},{"key":"B10","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1093\/ehjqcco\/qcaa075","article-title":"Prospective follow-up in various subtypes of cardiomyopathies: insights from the ESC EORP cardiomyopathy registry","volume":"7","author":"Gimeno","year":"2021","journal-title":"Eur Heart J Qual Care Clin Outcomes"},{"key":"B11","doi-asserted-by":"publisher","first-page":"1371","DOI":"10.1161\/CIRCULATIONAHA.119.044366","article-title":"Hypertrophic cardiomyopathy with left ventricular systolic dysfunction: insights from the SHaRe registry","volume":"141","author":"Marstrand","year":"2020","journal-title":"Circulation"},{"key":"B12","doi-asserted-by":"publisher","first-page":"533","DOI":"10.1016\/j.jacep.2021.12.002","article-title":"Arrhythmogenic right ventricular cardiomyopathy","volume":"8","author":"Krahn","year":"2022","journal-title":"JACC Clin Electrophysiol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"201","DOI":"10.1016\/j.ijcard.2017.10.076","article-title":"Heart transplantation in arrhythmogenic right ventricular cardiomyopathy\u2014experience from the Nordic ARVC Registry","volume":"250","author":"Gilljam","year":"2018","journal-title":"Int J Cardiol"},{"key":"B14","doi-asserted-by":"publisher","first-page":"1807","DOI":"10.1161\/CIRCULATIONAHA.106.174287","article-title":"Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention","volume":"113","author":"Maron","year":"2006","journal-title":"Circulation"},{"key":"B15","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.jjcc.2019.09.011","article-title":"Left ventricular noncompaction\u2014risk stratification and genetic consideration","volume":"75","author":"Ichida","year":"2020","journal-title":"J Cardiol"},{"key":"B16","doi-asserted-by":"publisher","first-page":"643","DOI":"10.1016\/j.jacc.2021.06.016","article-title":"Clinical risk prediction in patients with left ventricular myocardial noncompaction","volume":"78","author":"Casas","year":"2021","journal-title":"J Am Coll Cardiol"},{"key":"B17","doi-asserted-by":"publisher","first-page":"512","DOI":"10.1002\/ejhf.2140","article-title":"Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on myocardial and pericardial diseases","volume":"23","author":"Garcia-Pavia","year":"2021","journal-title":"Eur J Heart Fail"},{"key":"B18","doi-asserted-by":"publisher","first-page":"1007","DOI":"10.1056\/NEJMoa1805689","article-title":"Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy","volume":"379","author":"Maurer","year":"2018","journal-title":"N Engl J Med"},{"key":"B19","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1080\/14017431.2020.1792972","article-title":"Clinical presentation and outcomes in women and men with advanced heart failure","volume":"54","author":"Vishram-Nielsen","year":"2020","journal-title":"Scand Cardiovasc J"},{"key":"B20","doi-asserted-by":"publisher","first-page":"1392","DOI":"10.1002\/ejhf.1216","article-title":"Sex- and age-based differences in the natural history and outcome of dilated cardiomyopathy","volume":"20","author":"Halliday","year":"2018","journal-title":"Eur J Heart Fail"},{"key":"B21","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1186\/s12872-022-02639-0","article-title":"Diagnosis, management, and outcome of cardiac sarcoidosis and giant cell myocarditis: a Swedish single center experience","volume":"22","author":"Bobbio","year":"2022","journal-title":"BMC Cardiovasc Disord"},{"key":"B22","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1177\/2040622318790197","article-title":"Epidemiology of sarcoidosis: current findings and future directions","volume":"9","author":"Arkema","year":"2018","journal-title":"Ther Adv Chronic Dis"},{"key":"B23","doi-asserted-by":"publisher","first-page":"1955","DOI":"10.1016\/j.jacc.2018.07.072","article-title":"Stress cardiomyopathy diagnosis and treatment: JACC state-of-the-art review","volume":"72","author":"Medina de Chazal","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"B24","doi-asserted-by":"publisher","first-page":"1077","DOI":"10.1056\/NEJM200004133421502","article-title":"Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy","volume":"342","author":"Felker","year":"2000","journal-title":"N Engl J Med"}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2023.1160089\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T05:39:45Z","timestamp":1681709985000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2023.1160089\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,17]]},"references-count":24,"alternative-id":["10.3389\/fcvm.2023.1160089"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2023.1160089","relation":{},"ISSN":["2297-055X"],"issn-type":[{"value":"2297-055X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,17]]},"article-number":"1160089"}}