{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,26]],"date-time":"2025-09-26T13:05:53Z","timestamp":1758891953587,"version":"3.41.2"},"reference-count":38,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,11,30]],"date-time":"2022-11-30T00:00:00Z","timestamp":1669766400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Although ventricular failure is a late finding in adults with AC, we hypothesize that this is a presenting symptom in pediatric heart failure patients who undergo HT and that their ventricular arrhythmia burden could differentiate AC from other cardiomyopathies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed a single\u2010center retrospective cohort study reviewing 457 consecutive pediatric (\u226418\u2009years) HT recipients at our institution. Explanted hearts were examined to establish the primary diagnosis, based on pathologic findings. Demographic and clinical variables were compared between AC versus non\u2010HCM cardiomyopathy cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010five percent (<jats:italic>n<\/jats:italic>\u00a0=\u2009205\/457) had non\u2010HCM cardiomyopathies as the underlying primary diagnosis. Ten cases (10\/205\u00a0=\u00a04.9%) were diagnosed with AC. All 10 had biventricular disease. In 8\/10 patients (80%), AC diagnosis was unrecognized pre\u2010HT. Compared with non\u2010AC cardiomyopathies, the AC group was older at diagnosis (9.3\u2009years vs. 4.3\u2009years, <jats:italic>p<\/jats:italic>\u00a0=\u2009.012) and transplant (11.1\u2009years vs. 6.5\u2009years, <jats:italic>p<\/jats:italic>\u00a0=\u2009.010), had more ventricular arrhythmias (80.0% vs 32.8%, <jats:italic>p<\/jats:italic>\u00a0=\u2009.003), and required more anti\u2010arrhythmic use (80.0% vs 32.3%, <jats:italic>p<\/jats:italic>\u00a0=\u2009.001). Genetic testing yielded causative pathogenic variants in all tested individuals (<jats:italic>n<\/jats:italic>\u00a0=\u20095\/5, 100%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>AC is often an unrecognized cardiomyopathy pretransplant in children who undergo HT. Pediatric non\u2010HCM phenotypes with heart failure who have a significant ventricular arrhythmia burden should be investigated for AC.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14442","type":"journal-article","created":{"date-parts":[[2022,12,1]],"date-time":"2022-12-01T12:16:24Z","timestamp":1669896984000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Arrhythmogenic cardiomyopathy is under\u2010recognized in end\u2010stage pediatric heart failure: A 36\u2010year single\u2010center experience"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2348-9262","authenticated-orcid":false,"given":"Hanna J.","family":"Tadros","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Lille Frank Abercrombie Division of Pediatric Cardiology Texas Children's Hospital, Baylor College of Medicine  Houston Texas 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