{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T19:06:58Z","timestamp":1790276818026,"version":"4.1.0"},"reference-count":27,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,11,4]],"date-time":"2022-11-04T00:00:00Z","timestamp":1667520000000},"content-version":"vor","delay-in-days":307,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatrics International"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Mitochondrial fatty acid oxidation disorders (FAODs) cause impairment in energy metabolism and can lead to a spectrum of cardiac pathologies including cardiomyopathy and arrhythmias. The frequency of underlying cardiac pathologies and the response to recommended treatment in FAODs was investigated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Sixty\u2010eight children (35 males, 33 females) with the diagnosis of a FAOD were included in the study. Cardiac function was evaluated with 12\u2010lead standard electrocardiography, echocardiography, and 24 h Holter monitoring.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010five patients (66%) were diagnosed after disease symptoms developed and 23 patients (34%) were diagnosed in the pre\u2010symptomatic period. Among symptomatic patients (<jats:italic>n<\/jats:italic>: 45), cardiovascular findings were detected in 18 (40%) patients, including cardiomyopathy in 14 (31.1%) and conduction abnormalities in 4 (8.8%) patients. Cardiac symptoms were more frequently detected in primary systemic carnitine deficiency (57.1%). Patients with multiple acyl\u2010CoA dehydrogenase, long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase, and mitochondrial trifunctional protein deficiencies also had an increased frequency of cardiac symptoms. Patients with medium\u2010chain acyl\u2010CoA dehydrogenase, very long\u2010chain acyl\u2010CoA dehydrogenase, and carnitine palmitoyltransferase I deficiencies had a lower prevalence of cardiac symptoms both during admission and during clinical follow up. Cardiomyopathy resolved completely in 8\/14 (57%) patients and partially in 2\/14 (14.3%) patients with treatment. Two patients with cardiomyopathy died in the newborn period; cardiomyopathy persisted in 1 (7.1%) patient with very long\u2010chain acyl\u2010CoA dehydrogenase deficiency.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Early diagnosis, treatment and follow up made a significant contribution to the improvement of cardiac symptoms of patients with FAODs.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ped.15317","type":"journal-article","created":{"date-parts":[[2022,8,2]],"date-time":"2022-08-02T16:56:12Z","timestamp":1659459372000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Cardiologic evaluation of Turkish mitochondrial fatty acid oxidation disorders"],"prefix":"10.1111","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3384-8679","authenticated-orcid":false,"given":"Mehmet Cihan","family":"Balci","sequence":"first","affiliation":[{"name":"Division of Pediatric Nutrition and Metabolism Istanbul Medical Faculty Children's Hospital, Istanbul University  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0662-7344","authenticated-orcid":false,"given":"Meryem","family":"Karaca","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nutrition and Metabolism Istanbul Medical Faculty Children's Hospital, Istanbul University  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8814-0435","authenticated-orcid":false,"given":"Yakup","family":"Ergul","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology Istanbul Medical Faculty Children's Hospital, Istanbul University  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rukiye Eker","family":"Omeroglu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology Istanbul Medical Faculty Children's Hospital, Istanbul University  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mubeccel","family":"Demirkol","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nutrition and Metabolism Istanbul Medical Faculty Children's Hospital, Istanbul University  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gulden Fatma","family":"Gokcay","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nutrition and Metabolism Istanbul Medical Faculty Children's Hospital, Istanbul University  Istanbul Turkey"},{"name":"Department of Rare Diseases, Institute of Child Health Istanbul University  Istanbul 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