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Fatty acids released from adipose tissues during fasting accumulate in various organs predisposing to their impaired function.[5][6] Accumulation of fat in the liver causes steatosis and impairment of ketone body production.[5][6][8] In the heart and skeletal muscles, this abnormal accumulation results in cardiomyopathy and myopathy respectively.[5][6] Heart derives two-thirds of its energy from free fatty acids and this predisposes patients with impaired carnitine metabolism to the development of cardiomyopathy.[6] Additionally, impaired lipid metabolism can affect the electrical rhythm of the heart resulting in arrhythmias.[6] The brain utilizes ketone bodies as an alternate energy source in fasting states

Sowers JR