{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T14:17:25Z","timestamp":1785161845595,"version":"3.55.0"},"reference-count":59,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,7,23]],"date-time":"2021-07-23T00:00:00Z","timestamp":1626998400000},"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. Pediatr."],"abstract":"<jats:p>Paediatric cardiomyopathies are a heterogenous group of rare disorders, characterised by mechanical and electrical abnormalities of the heart muscle. The overall annual incidence of childhood cardiomyopathies is estimated at about 1 per 100,000 children and is significantly higher during the first 2 years of life. Dilated cardiomyopathies account for approximately half of the cases. Hypertrophic cardiomyopathies form the second largest group, followed by the less common left ventricular non-compaction and restrictive phenotypes. Infectious, metabolic, genetic, and syndromic conditions account for the majority of cases. Congestive heart failure is the typical manifestation in children with dilated cardiomyopathy, whereas presenting symptoms are more variable in other phenotypes. The natural history is largely influenced by the type of cardiomyopathy and its underlying aetiology. Results from a national population-based study revealed 10-year transplant-free survival rates of 80, 62, and 48% for hypertrophic, dilated and left ventricular non-compaction cardiomyopathies, respectively. Long-term survival rates of children with a restrictive phenotype have largely been obscured by early listing for heart transplantation. In general, the majority of adverse events, including death and heart transplantation, occur during the first 2 years after the initial presentation. This review provides an overview of childhood cardiomyopathies with a focus on epidemiology, natural history, and outcomes.<\/jats:p>","DOI":"10.3389\/fped.2021.708732","type":"journal-article","created":{"date-parts":[[2021,7,23]],"date-time":"2021-07-23T05:58:42Z","timestamp":1627019922000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":43,"title":["Overview of Cardiomyopathies in Childhood"],"prefix":"10.3389","volume":"9","author":[{"given":"Anika","family":"Rath","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Weintraub","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,7,23]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"855","DOI":"10.1161\/CIRCRESAHA.116.309386","article-title":"Pediatric cardiomyopathies","volume":"121","author":"Lee","year":"2017","journal-title":"Circ Res."},{"key":"B2","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1161\/CIR.0000000000000682","article-title":"Cardiomyopathy in children: classification and diagnosis: a scientific statement from the american heart association","volume":"140","author":"Lipshultz","year":"2019","journal-title":"Circulation"},{"key":"B3","doi-asserted-by":"publisher","first-page":"813","DOI":"10.1016\/S0140-6736(14)61282-4","article-title":"Left ventricular non-compaction cardiomyopathy","volume":"386","author":"Towbin","year":"2015","journal-title":"Lancet"},{"key":"B4","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":"B5","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1016\/j.hfc.2010.05.005","article-title":"Restrictive cardiomyopathy in childhood","volume":"6","author":"Denfield","year":"2010","journal-title":"Heart Fail Clin."},{"key":"B6","doi-asserted-by":"publisher","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":"B7","doi-asserted-by":"publisher","first-page":"1647","DOI":"10.1056\/NEJMoa021715","article-title":"The incidence of pediatric cardiomyopathy in two regions of the United States","volume":"348","author":"Lipshultz","year":"2003","journal-title":"N Engl J Med"},{"key":"B8","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1161\/CIRCULATIONAHA.117.028895","article-title":"Long-term outcomes of hypertrophic cardiomyopathy diagnosed during childhood: results from a national population-based study","volume":"138","author":"Alexander","year":"2018","journal-title":"Circulation"},{"key":"B9","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1001\/jamacardio.2018.0789","article-title":"Long-term outcomes of pediatric-onset hypertrophic cardiomyopathy and age-specific risk factors for lethal arrhythmic events","volume":"3","author":"Maurizi","year":"2018","journal-title":"JAMA Cardiol"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1237","DOI":"10.1161\/CIRCULATIONAHA.112.104638","article-title":"Outcomes of restrictive cardiomyopathy in childhood and the influence of phenotype: a report from the pediatric cardiomyopathy registry","volume":"126","author":"Webber","year":"2012","journal-title":"Circulation"},{"key":"B11","doi-asserted-by":"publisher","first-page":"501","DOI":"10.1016\/S0002-9149(02)02522-5","article-title":"Outcome of idiopathic restrictive cardiomyopathy in children","volume":"90","author":"Weller","year":"2002","journal-title":"Am J Cardiol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"877","DOI":"10.1016\/j.cardfail.2015.06.381","article-title":"Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: results from the pediatric cardiomyopathy registry","volume":"21","author":"Jefferies","year":"2015","journal-title":"J Card Fail"},{"key":"B13","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1161\/CIRCULATIONAHA.117.032262","article-title":"Long-term outcomes of childhood left ventricular noncompaction cardiomyopathy: Results from a national population-based study","volume":"138","author":"Shi","year":"2018","journal-title":"Circulation"},{"key":"B14","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1016\/j.ppedcard.2007.11.006","article-title":"The pediatric cardiomyopathy registry: 1995-2007","volume":"25","author":"Wilkinson","year":"2008","journal-title":"Prog Pediatr Cardiol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1016\/j.hfc.2010.05.002","article-title":"The pediatric cardiomyopathy registry and heart failure: key results from the first 15 years","volume":"6","author":"Wilkinson","year":"2010","journal-title":"Heart Fail Clin"},{"key":"B16","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1161\/JAHA.116.003450","article-title":"Natural history of dilated cardiomyopathy in children","volume":"5","author":"Puggia","year":"2016","journal-title":"J Am Heart Assoc"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1639","DOI":"10.1056\/NEJMoa021737","article-title":"The epidemiology of childhood cardiomyopathy in Australia","volume":"348","author":"Nugent","year":"2003","journal-title":"N Engl J Med"},{"key":"B18","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1093\/oxfordjournals.aje.a009291","article-title":"Epidemiology of idiopathic cardiomyopathies in children and adolescents: a nationwide study in Finland","volume":"146","author":"Arola","year":"1997","journal-title":"Am J Epidemiol"},{"key":"B19","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1161\/CIRCULATIONAHA.106.671735","article-title":"New-onset heart failure due to heart muscle disease in childhood: a prospective study in the United Kingdom and Ireland","volume":"117","author":"Andrews","year":"2008","journal-title":"Circulation"},{"key":"B20","doi-asserted-by":"publisher","first-page":"1867","DOI":"10.1001\/jama.296.15.1867","article-title":"Incidence, causes, and outcome of dilated cardiomyopathy in children","volume":"296","author":"Towbin","year":"2006","journal-title":"JAMA Cardiol."},{"key":"B21","doi-asserted-by":"publisher","first-page":"2671","DOI":"10.1161\/CIRCULATIONAHA.106.635128","article-title":"Clinical features and outcomes of childhood dilated cardiomyopathy: results from a national population-based study","volume":"114","author":"Daubeney","year":"2006","journal-title":"Circulation"},{"key":"B22","doi-asserted-by":"publisher","first-page":"2039","DOI":"10.1161\/CIRCULATIONAHA.113.002767","article-title":"Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy","volume":"128","author":"Alexander","year":"2013","journal-title":"Circulation"},{"key":"B23","doi-asserted-by":"publisher","first-page":"773","DOI":"10.1161\/CIRCULATIONAHA.106.621185","article-title":"Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the pediatric cardiomyopathy registry","volume":"115","author":"Colan","year":"2007","journal-title":"Circulation"},{"key":"B24","doi-asserted-by":"publisher","first-page":"1332","DOI":"10.1161\/CIRCULATIONAHA.104.530303","article-title":"Clinical features and outcomes of childhood hypertrophic cardiomyopathy: results from a national population-based study","volume":"112","author":"Nugent","year":"2005","journal-title":"Circulation"},{"key":"B25","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":"B26","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 function","volume":"113","author":"Maron","year":"2006","journal-title":"Circulation"},{"key":"B27","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1017\/S1047951115001201","article-title":"Nomenclature and systems of classification for cardiomyopathy in children","volume":"25","author":"Konta","year":"2015","journal-title":"Cardiol Young"},{"key":"B28","doi-asserted-by":"publisher","first-page":"731","DOI":"10.1161\/CIRCRESAHA.116.309396","article-title":"Dilated cardiomyopathy: genetic determinants and mechanisms","volume":"121","author":"McNally","year":"2017","journal-title":"Circ Res"},{"key":"B29","doi-asserted-by":"publisher","first-page":"1287","DOI":"10.1093\/cvr\/cvy122","article-title":"Complex roads from genotype to phenotype in dilated cardiomyopathy: scientific update from the working group of myocardial function of the european society of cardiology","volume":"114","author":"Bondue","year":"2018","journal-title":"Cardiovasc Res"},{"key":"B30","doi-asserted-by":"publisher","first-page":"1152","DOI":"10.1016\/S1053-2498(01)00334-5","article-title":"Clinical, electrocardiographic, and histologic correlations in children with dilated cardiomyopathy","volume":"20","author":"Nugent","year":"2001","journal-title":"J Heart Lung Transplant"},{"key":"B31","doi-asserted-by":"publisher","first-page":"1653","DOI":"10.1016\/j.jacc.2017.01.043","article-title":"Autosomal recessive cardiomyopathy presenting as acute myocarditis","volume":"69","author":"Belkaya","year":"2017","journal-title":"J Am Coll Cardiol."},{"key":"B32","doi-asserted-by":"publisher","first-page":"2913","DOI":"10.1016\/j.jacc.2015.10.049","article-title":"A genetically vulnerable myocardium may predispose to myocarditis","volume":"66","author":"Campuzano","year":"2015","journal-title":"J Am Coll Cardiol"},{"key":"B33","doi-asserted-by":"publisher","first-page":"3076","DOI":"10.1093\/eurheartj\/ehm456","article-title":"The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology","volume":"28","author":"Cooper","year":"2007","journal-title":"Eur Heart J."},{"key":"B34","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1161\/CIRCULATIONAHA.113.001372","article-title":"Diagnosis and treatment of myocarditis in children in the current era","volume":"129","author":"Canter","year":"2014","journal-title":"Circulation"},{"key":"B35","doi-asserted-by":"publisher","first-page":"672","DOI":"10.1016\/j.cardfail.2013.08.006","article-title":"Characteristics and outcomes of heart failure-related intensive care unit admissions in children with cardiomyopathy","volume":"19","author":"Shamszad","year":"2013","journal-title":"J Card Fail"},{"key":"B36","first-page":"2","article-title":"Heart diseases leading to pediatric transplantation: cardiomyopathies and congenital heart diseases","author":"Hsu","year":"2008"},{"key":"B37","doi-asserted-by":"publisher","first-page":"1405","DOI":"10.1016\/j.jacc.2013.11.059","article-title":"Recovery of echocardiographic function in children with idiopathic dilated cardiomyopathy: results from the pediatric cardiomyopathy registry","volume":"63","author":"Everitt","year":"2014","journal-title":"J Am Coll Cardiol"},{"key":"B38","doi-asserted-by":"publisher","first-page":"1723","DOI":"10.1016\/j.amjcard.2016.08.053","article-title":"Usefulness of serial N-terminal pro-B-type natriuretic peptide measurements to predict cardiac death in acute and chronic dilated cardiomyopathy in children","volume":"118","author":"den Boer","year":"2016","journal-title":"Am J Cardiol"},{"key":"B39","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1016\/j.jacc.2011.10.878","article-title":"Incidence of and risk factors for sudden cardiac death in children with dilated cardiomyopathy: a report from the pediatric cardiomyopathy registry","volume":"59","author":"Pahl","year":"2012","journal-title":"J Am Coll Cardiol"},{"key":"B40","doi-asserted-by":"publisher","first-page":"2663","DOI":"10.1016\/j.jacc.2017.09.1089","article-title":"Survival without cardiac transplantation among children with dilated cardiomyopathy","volume":"70","author":"Singh","year":"2017","journal-title":"J Am Coll Cardiol"},{"key":"B41","doi-asserted-by":"publisher","first-page":"1988","DOI":"10.1093\/eurheartj\/ehab148","article-title":"Clinical characteristics and outcomes in childhood-onset hypertrophic cardiomyopathy","volume":"42","author":"Marston","year":"2021","journal-title":"Eur Heart J"},{"key":"B42","doi-asserted-by":"publisher","first-page":"442","DOI":"10.1016\/j.ahj.2012.04.018","article-title":"Outcomes in children with noonan syndrome and hypertrophic cardiomyopathy: a study from the pediatric cardiomyopathy registry","volume":"164","author":"Wilkinson","year":"2012","journal-title":"Am Heart J"},{"key":"B43","doi-asserted-by":"publisher","first-page":"1220","DOI":"10.1177\/2047487317702519","article-title":"Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: a systematic review and meta-analysis","volume":"24","author":"Norrish","year":"2017","journal-title":"Eur J Prev Cardiol"},{"key":"B44","doi-asserted-by":"publisher","first-page":"918","DOI":"10.1001\/jamacardio.2019.2861","article-title":"Development of a novel risk prediction model for sudden cardiac death in childhood hypertrophic cardiomyopathy (HCM risk-kids)","volume":"4","author":"Norrish","year":"2019","journal-title":"JAMA Cardiol"},{"key":"B45","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1161\/CIRCULATIONAHA.120.047235","article-title":"A validated model for sudden cardiac death risk prediction in pediatric hypertrophic cardiomyopathy","volume":"142","author":"Miron","year":"2020","journal-title":"Circulation"},{"key":"B46","doi-asserted-by":"publisher","first-page":"1462","DOI":"10.1016\/j.hrthm.2019.04.040","article-title":"Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator: an international multicenter study","volume":"16","author":"Balaji","year":"2019","journal-title":"Hear Rhythm"},{"key":"B47","doi-asserted-by":"publisher","first-page":"858","DOI":"10.1161\/01.CIR.102.8.858","article-title":"Epidemiology of hypertrophic cardiomyopathy\u2013related death","volume":"102","author":"Maron","year":"2000","journal-title":"Circulation"},{"key":"B48","doi-asserted-by":"publisher","first-page":"1478","DOI":"10.1136\/hrt.2007.134684","article-title":"Idiopathic restrictive cardiomyopathy in children is caused by mutations in cardiac sarcomere protein genes","volume":"94","author":"Kaski","year":"2008","journal-title":"Heart"},{"key":"B49","doi-asserted-by":"publisher","first-page":"694","DOI":"10.1007\/s00246-018-2043-0","article-title":"Contemporary outcomes of pediatric restrictive cardiomyopathy: a single-center experience","volume":"40","author":"Wittekind","year":"2019","journal-title":"Pediatr Cardiol."},{"key":"B50","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1056\/NEJM199701233360407","article-title":"Restrictive cardiomyopathy","volume":"336","author":"Kushwaha","year":"1997","journal-title":"N Engl J Med"},{"key":"B51","doi-asserted-by":"publisher","first-page":"876","DOI":"10.1161\/01.CIR.102.8.876","article-title":"Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy","volume":"102","author":"Rivenes","year":"2000","journal-title":"Circulation"},{"key":"B52","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1161\/CIRCHEARTFAILURE.111.964395","article-title":"Conduction abnormalities in pediatric patients with restrictive cardiomyopathy","volume":"5","author":"Walsh","year":"2012","journal-title":"Circ Heart Fail"},{"key":"B53","doi-asserted-by":"publisher","first-page":"1199","DOI":"10.1136\/hrt.2004.043869","article-title":"Idiopathic restrictive cardiomyopathy in children","volume":"91","author":"Russo","year":"2005","journal-title":"Heart."},{"key":"B54","doi-asserted-by":"publisher","first-page":"1266","DOI":"10.1016\/j.amjcard.2018.01.045","article-title":"Idiopathic restrictive cardiomyopathy in children and young adults","volume":"121","author":"Anderson","year":"2018","journal-title":"Am J Cardiol"},{"key":"B55","doi-asserted-by":"publisher","first-page":"2157","DOI":"10.1016\/j.jacc.2016.08.054","article-title":"Left ventricular noncompaction: anatomical phenotype or distinct cardiomyopathy?","volume":"68","author":"Weir-McCall","year":"2016","journal-title":"J Am Coll Cardiol"},{"key":"B56","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1016\/j.jacc.2017.12.019","article-title":"Genetics, clinical features, and long-term outcome of noncompaction cardiomyopathy","volume":"71","author":"van Waning","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"B57","doi-asserted-by":"publisher","first-page":"2202","DOI":"10.1161\/CIRCULATIONAHA.113.002511","article-title":"Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center","volume":"127","author":"Brescia","year":"2013","journal-title":"Circulation"},{"key":"B58","doi-asserted-by":"publisher","first-page":"722","DOI":"10.1161\/CIRCRESAHA.117.309711","article-title":"Classification, epidemiology, and global burden of cardiomyopathies","volume":"121","author":"McKenna","year":"2017","journal-title":"Circ Res"},{"key":"B59","doi-asserted-by":"publisher","first-page":"185","DOI":"10.1016\/S2352-4642(19)30402-X","article-title":"Global, regional, and national burden of congenital heart disease, 1990\u20132017: a systematic analysis for the global burden of disease study 2017","volume":"4","author":"Zimmerman","year":"2020","journal-title":"Lancet Child Adolesc Heal"}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2021.708732\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,23]],"date-time":"2021-07-23T05:58:46Z","timestamp":1627019926000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2021.708732\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7,23]]},"references-count":59,"alternative-id":["10.3389\/fped.2021.708732"],"URL":"https:\/\/doi.org\/10.3389\/fped.2021.708732","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,7,23]]},"article-number":"708732"}}