Tutorial: Triheptanoin and Nutrition Management for Treatment of Long-Chain Fatty Acid Oxidation Disorders.
Marie K NorrisAnna I ScottSarah SullivanIrene J ChangChristina LamAngela SunSihoun HahnJenny M ThiesMelissa GunnarsonKelly N McKeanJ Lawrence MerrittPublished in: JPEN. Journal of parenteral and enteral nutrition (2020)
Triheptanoin can ameliorate acute cardiomyopathy and increase survival in patients with severe LC-FAOD. Substituting triheptanoin for traditional MCT-based treatment improves clinical outcomes. MCT oil might be less effective in carnitine-acylcarnitine translocase deficiency patients compared with other FAODs and needs further investigation.