1 40 1 Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month November 2017 List Notes 1524-4571 Lee, Teresa M Hsu, Daphne T Kantor, Paul Towbin, Jeffrey A Ware, Stephanie M Colan, Steven D Chung, Wendy K Jefferies, John L Rossano, Joseph W Castleberry, Chesney D Addonizio, Linda J Lal, Ashwin K Lamour, Jacqueline M Miller, Erin M Thrush, Philip T Czachor, Jason D Razoky, Hiedy Hill, Ashley Lipshultz, Steven E Journal Article Review United States Circ Res. 2017 Sep 15;121(7):855-873. doi: 10.1161/CIRCRESAHA.116.309386. Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Pediatric Cardiomyopathies Publisher An entity responsible for making the resource available Circulation Research Date A point or period of time associated with an event in the lifecycle of the resource 2017 Subject The topic of the resource Epidemiology; Genetics; Pediatrics Creator An entity primarily responsible for making the resource Lee TM; Hsu DT; Kantor P; Towbin JA; Ware SM; Colan SD; Chung WK; Jefferies JL; Rossano JW; Castleberry CD; Addonizio LJ; Lal AK; Lamour JM; Miller EM; Thrush PT; Czachor JD; Razoky H; Hill A; Lipshultz SE Description An account of the resource Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent. Pediatric cardiomyopathies often occur in the absence of comorbidities, such as atherosclerosis, hypertension, renal dysfunction, and diabetes mellitus; as a result, they offer insights into the primary pathogenesis of myocardial dysfunction. Large international registries have characterized the epidemiology, cause, and outcomes of pediatric cardiomyopathies. Although adult and pediatric cardiomyopathies have similar morphological and clinical manifestations, their outcomes differ significantly. Within 2 years of presentation, normalization of function occurs in 20% of children with dilated cardiomyopathy, and 40% die or undergo transplantation. Infants with hypertrophic cardiomyopathy have a 2-year mortality of 30%, whereas death is rare in older children. Sudden death is rare. Molecular evidence indicates that gene expression differs between adult and pediatric cardiomyopathies, suggesting that treatment response may differ as well. Clinical trials to support evidence-based treatments and the development of disease-specific therapies for pediatric cardiomyopathies are in their infancy. This compendium summarizes current knowledge of the genetic and molecular origins, clinical course, and outcomes of the most common phenotypic presentations of pediatric cardiomyopathies and highlights key areas where additional research is required. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifiers: NCT02549664 and NCT01912534. Identifier An unambiguous reference to the resource within a given context 10.1161/circresaha.116.309386 Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). 2017 Addonizio LJ Castleberry CD Chung WK Circulation Research Colan SD Czachor JD Epidemiology Genetics Hill A Hsu DT Jefferies JL Kantor P Lal AK Lamour JM Lee TM Lipshultz SE Miller EM November 2017 List Pediatrics Razoky H Rossano JW Thrush PT Towbin JA Ware SM