{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T17:30:25Z","timestamp":1791394225942,"version":"4.3.3"},"reference-count":38,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,9,4]],"date-time":"2017-09-04T00:00:00Z","timestamp":1504483200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2017,9,4]],"date-time":"2017-09-04T00:00:00Z","timestamp":1504483200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"funder":[{"DOI":"10.13039\/100000038","name":"U.S. Food and Drug Administration","doi-asserted-by":"publisher","award":["FD038950"],"award-info":[{"award-number":["FD038950"]}],"id":[{"id":"10.13039\/100000038","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Observational reports suggest that supplementation that increases citric acid cycle intermediates via anaplerosis may have therapeutic advantages over traditional medium\u2010chain triglyceride (MCT) treatment of long\u2010chain fatty acid oxidation disorders (LC\u2010FAODs) but controlled trials have not been reported. The goal of our study was to compare the effects of triheptanoin (C7), an anaplerotic seven\u2010carbon fatty acid triglyceride, to trioctanoin (C8), an eight\u2010carbon fatty acid triglyceride, in patients with LC\u2010FAODs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A double blinded, randomized controlled trial of 32 subjects with LC\u2010FAODs (carnitine palmitoyltransferase\u20102, very long\u2010chain acylCoA dehydrogenase, trifunctional protein or long\u2010chain 3\u2010hydroxy acylCoA dehydrogenase deficiencies) who were randomly assigned a diet containing 20% of their total daily energy from either C7 or C8 for 4 months was conducted. Primary outcomes included changes in total energy expenditure (TEE), cardiac function by echocardiogram, exercise tolerance, and phosphocreatine recovery following acute exercise. Secondary outcomes included body composition, blood biomarkers, and adverse events, including incidence of rhabdomyolysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients in the C7 group increased left ventricular (LV) ejection fraction by 7.4% (<jats:italic>p<\/jats:italic> = 0.046) while experiencing a 20% (<jats:italic>p<\/jats:italic> = 0.041) decrease in LV wall mass on their resting echocardiogram. They also required a lower heart rate for the same amount of work during a moderate\u2010intensity exercise stress test when compared to patients taking C8. There was no difference in TEE, phosphocreatine recovery, body composition, incidence of rhabdomyolysis, or any secondary outcome measures between the groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>C7 improved LV ejection fraction and reduced LV mass at rest, as well as lowering heart rate during exercise among patients with LC\u2010FAODs.<\/jats:p><jats:p>Clinical Trial Registration: <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/clinicaltrials.gov\">Clinicaltrials.gov<\/jats:ext-link> NCT01379625.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s10545-017-0085-8","type":"journal-article","created":{"date-parts":[[2017,9,4]],"date-time":"2017-09-04T11:14:43Z","timestamp":1504523683000},"page":"831-843","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":108,"title":["Triheptanoin versus trioctanoin for long\u2010chain fatty acid oxidation disorders: a double blinded, randomized controlled trial"],"prefix":"10.1002","volume":"40","author":[{"given":"Melanie B.","family":"Gillingham","sequence":"first","affiliation":[{"name":"Department of Molecular and Medical Genetics, Graduate Programs in Human Nutrition Oregon Health &amp; Science University 3181 SW Sam Jackson Park Rd Portland OR 97239 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen B.","family":"Heitner","sequence":"additional","affiliation":[{"name":"Knight Cardiovascular Institute Oregon Health &amp; Science University Portland OR USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Martin","sequence":"additional","affiliation":[{"name":"Department of Molecular and Medical Genetics, Graduate Programs in Human Nutrition Oregon Health &amp; Science University 3181 SW Sam Jackson Park Rd Portland OR 97239 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