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It is unknown whether latent cardiac abnormalities exist in adult patients. To investigate cardiac involvement in LCFAOD, we used proton magnetic resonance imaging (MRI) and spectroscopy (<jats:sup>1<\/jats:sup>H\u2010MRS) to quantify heart function, myocardial tissue characteristics, and myocardial lipid content in 14 adult patients (two with long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency (LCHADD); four with carnitine palmitoyltransferase II deficiency (CPT2D); and eight with very long\u2010chain acyl\u2010CoA dehydrogenase deficiency (VLCADD)) and 14 gender\u2010, age\u2010, and BMI\u2010matched control subjects. Examinations included cine MRI, MR tagging, native myocardial T<jats:sub>1<\/jats:sub> and T<jats:sub>2<\/jats:sub> mapping, and localized <jats:sup>1<\/jats:sup>H\u2010MRS at 3 Tesla. Left ventricular (LV) myocardial mass (<jats:italic>P<\/jats:italic> = .011) and the LV myocardial mass\u2010to\u2010volume ratio (<jats:italic>P<\/jats:italic> = .008) were higher in patients, while ejection fraction (EF) was normal (<jats:italic>P<\/jats:italic> = .397). LV torsion was higher in patients (<jats:italic>P<\/jats:italic> = .026), whereas circumferential shortening was similar compared with controls (<jats:italic>P<\/jats:italic> = .875). LV hypertrophy was accompanied by high myocardial T<jats:sub>1<\/jats:sub> values (indicative of diffuse fibrosis) in two patients, and additionally a low EF in one case. Myocardial lipid content was similar in patients and controls. We identified subclinical morphological and functional differences between the hearts of LCFAOD patients and matched control subjects using state\u2010of\u2010the\u2010art MR methods. Our results suggest a chronic cardiac disease phenotype and hypertrophic LV remodeling of the heart in LCFAOD, potentially triggered by a mild, but chronic, energy deficiency, rather than by lipotoxic effects of accumulating lipid metabolites.<\/jats:p>","DOI":"10.1002\/jimd.12266","type":"journal-article","created":{"date-parts":[[2020,5,28]],"date-time":"2020-05-28T13:13:51Z","timestamp":1590671631000},"page":"969-980","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Subclinical effects of long\u2010chain fatty acid \u03b2\u2010oxidation deficiency on the adult heart: A case\u2010control magnetic resonance study"],"prefix":"10.1002","volume":"43","author":[{"given":"Suzan J. G.","family":"Knottnerus","sequence":"first","affiliation":[{"name":"Department of Metabolic Diseases Wilhelmina Children's Hospital, University Medical Center Utrecht  Utrecht The Netherlands"},{"name":"Laboratory Genetic Metabolic Diseases Amsterdam University Medical Centers, University of Amsterdam, Amsterdam Gastroenterology and Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeannette C.","family":"Bleeker","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases Wilhelmina Children's Hospital, University Medical Center Utrecht  Utrecht The Netherlands"},{"name":"Laboratory Genetic Metabolic Diseases Amsterdam University Medical Centers, University of Amsterdam, Amsterdam Gastroenterology and Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sacha","family":"Ferdinandusse","sequence":"additional","affiliation":[{"name":"Laboratory Genetic Metabolic Diseases Amsterdam University Medical Centers, University of Amsterdam, Amsterdam Gastroenterology and Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riekelt H.","family":"Houtkooper","sequence":"additional","affiliation":[{"name":"Laboratory Genetic Metabolic Diseases Amsterdam University Medical Centers, University of Amsterdam, Amsterdam Gastroenterology and Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirjam","family":"Langeveld","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Metabolism Amsterdam University Medical Centers, University of Amsterdam  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aart J.","family":"Nederveen","sequence":"additional","affiliation":[{"name":"Department of Radiology and Nuclear Medicine Amsterdam University Medical Centers, University of Amsterdam  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gustav J.","family":"Strijkers","sequence":"additional","affiliation":[{"name":"Biomedical Engineering and Physics, Amsterdam University Medical Centers University of Amsterdam  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gepke","family":"Visser","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases Wilhelmina Children's Hospital, University Medical Center Utrecht  Utrecht The Netherlands"},{"name":"Laboratory Genetic Metabolic Diseases Amsterdam University Medical Centers, University of Amsterdam, Amsterdam Gastroenterology and Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald J. 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