The purpose of this study is to assess the safety and to examine the index of the efficacy after repeated dosing of CNT-01 in patients with idiopathic triglyceride deposit cardiomyovasculopathy.

Inclusion Criteria:

• Have a definite diagnosis of idiopathic triglyceride deposit cardiomyovasculopathy
• After receiving a sufficient explanation upon participation in this study, on a full understanding, patients who obtained the written informed consent from each patient
• More than 20 years old at the time of informed consent
• Is able to oral intake
• Must be willing to comply with protocol-required examination such as BMIPP myocardial scintigraphy

Exclusion Criteria:

• Have diabetic ketoacidosis
• Have a malignancy that is being treated by anticancer drug or anticipated survival is less than three years
• Female with pregnant or lactating
• Is not able to agree to using intrauterine device, oral contraceptive, condom or having sexual intercourse from the start of taking investigational product to 30 days after the end of the study
• Have a New York Heart Association functional classification IV
• Have a known history of drug dependence
• Is allergic to any component of the investigational product
• Is allergic to BMIPP or iodine
• Have a known history of clinically significant drug allergy
• Have a severe liver dysfunction (Child classification B and C)
• Participated in other clinical study within the past 3 months and received an investigational agent including placebo
• Being treated with diet containing medium chain fatty acid
• Is considered unfit for the study by the Investigator's medical decision