{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T22:36:02Z","timestamp":1785191762054,"version":"3.55.0"},"reference-count":59,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2024,1,12]],"date-time":"2024-01-12T00:00:00Z","timestamp":1705017600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by the progressive replacement of the normal myocardium by fibroadipocytic tissue. The importance of an early diagnosis is supported by a higher risk of sudden cardiac death in the pediatric population. We reviewed the literature on diagnosis, risk stratification, and prognosis in the pediatric population with ARVC. In case reports which analyzed children with ARVC, the most common sign was ventricular tachycardia, frequently presenting as dizziness, syncope, or even cardiac arrest. Currently, there is no gold standard for diagnosing ARVC in children. Nevertheless, genetic analysis may provide a proper diagnosis tool for asymptomatic cases. Although risk stratification is recommended in patients with ARVC, a validated prediction model for risk stratification in children is still lacking; thus, it is a matter of further research. In consequence, even though ARVC is a relatively rare condition in children, it negatively impacts the survival and clinical outcomes of the patients. Therefore, appropriate and validated diagnostic and risk stratification tools are crucial for the early detection of children with ARVC, ensuring a prompt therapeutic intervention.<\/jats:p>","DOI":"10.3390\/diagnostics14020175","type":"journal-article","created":{"date-parts":[[2024,1,12]],"date-time":"2024-01-12T07:47:16Z","timestamp":1705045636000},"page":"175","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4140-0492","authenticated-orcid":false,"given":"Stefana Maria","family":"Moisa","sequence":"first","affiliation":[{"name":"Pediatrics Department, Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"},{"name":"\u201cSfanta Maria\u201d Clinical Emergency Hospital for Children, 700309 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7015-0085","authenticated-orcid":false,"given":"Elena Lia","family":"Spoiala","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3239-0704","authenticated-orcid":false,"given":"Eliza","family":"Cinteza","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Faculty of Medicine, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 700115 Bucharest, Romania"},{"name":"\u201cMarie Curie\u201d Clinical Emergency Hospital for Children, 41451 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7163-8528","authenticated-orcid":false,"given":"Radu","family":"Vatasescu","sequence":"additional","affiliation":[{"name":"Cardio-Thoracic Department, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 020021 Bucharest, Romania"},{"name":"Clinical Emergency Hospital, 050098 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6713-4244","authenticated-orcid":false,"given":"Lacramioara Ionela","family":"Butnariu","sequence":"additional","affiliation":[{"name":"\u201cSfanta Maria\u201d Clinical Emergency Hospital for Children, 700309 Iasi, Romania"},{"name":"Genetics Department, Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6253-3203","authenticated-orcid":false,"given":"Crischentian","family":"Brinza","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"},{"name":"Institute of Cardiovascular Diseases \u201cProf. Dr. George I.M. Georgescu\u201d, 700503 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3424-1588","authenticated-orcid":false,"given":"Alexandru","family":"Burlacu","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"},{"name":"Institute of Cardiovascular Diseases \u201cProf. Dr. George I.M. Georgescu\u201d, 700503 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,1,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1146\/annurev-pathol-011110-130151","article-title":"The pathobiology of arrhythmogenic cardiomyopathy","volume":"6","author":"Saffitz","year":"2011","journal-title":"Annu. Rev. Pathol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1038\/s41569-020-0428-2","article-title":"Epidemiology of the inherited cardiomyopathies","volume":"18","author":"McKenna","year":"2021","journal-title":"Nat. Rev. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"e008509","DOI":"10.1161\/CIRCEP.120.008509","article-title":"Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy: A Multinational Collaboration","volume":"14","author":"Bosman","year":"2021","journal-title":"Circ. Arrhythm. 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