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The Diagnostic Criteria 2020 for Triglyceride Deposit Cardiomyovasculopathy

  • Kobayashi Kunihisa
    Department of Endocrinology and Diabetes Mellitus, Fukuoka University Chikushi Hospital, Chikushino, Fukuoka, Japan
  • Sakata Yasuhiko
    Department of Cardiovascular Medicine, Graduate School of Medicine, Tohoku University, Sendai, Miyagi, Japan
  • Miyauchi Hideyuki
    Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, Chiba, Japan
  • Ikeda Yoshihiko
    Department of Pathology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
  • Nagasawa Yasuyuki
    Department of Internal Medicine, Division of Kidney and Dialysis, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
  • Nakajima Kenichi
    Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan
  • Shimada Kazunori
    Department of Functional Imaging and Artificial Intelligence, Kanazawa University, Ishikawa, Japan
  • Kozawa Junji
    Department of Metabolic Medicine, Graduate School of Medicine, Osaka University, Osaka, Japan
  • Hao Hiroyuki
    Division of Human Pathology, Department of Pathology and Microbiology, Nihon University School of Medicine, Tokyo, Japan
  • Amano Tetsuya
    Department of Cardiology, Aichi Medical University, Aichi, Japan
  • Yoshida Hiroshi
    Department of Laboratory Medicine, The Jikei University Kashiwa Hospital, Chiba, Japan
  • Inaba Tohru
    Department of Infection Control and Laboratory Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan
  • Hashimoto Chikako
    Laboratory of Cardiovascular Disease, Novel, Non-invasive, and Nutritional Therapeutics (CNT) and Triglyceride Research Center (TGRC), Graduate School of Medicine, Osaka University, Suita, Osaka, Japan
  • Hirano Ken-ichi
    Laboratory of Cardiovascular Disease, Novel, Non-invasive, and Nutritional Therapeutics (CNT) and Triglyceride Research Center (TGRC), Graduate School of Medicine, Osaka University, Suita, Osaka, Japan

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Abstract

<p>Triglyceride deposit cardiomyovasculopathy (TGCV) is a newly identified disease that was discovered in individuals who required cardiac transplantation in Japan in 2008. Defective intracellular lipolysis causes triglyceride (TG) accumulation in the myocardium and coronary artery vascular smooth muscle cells, which results in severe heart failure and coronary artery disease with poor prognosis. A known cause of TGCV is a genetic deficiency of adipose triglyceride lipase (ATGL), a rate-limiting enzyme in the intracellular hydrolysis of TG. TGCV is classified into primary TGCV with ATGL mutations and idiopathic TGCV without ATGL mutations. Since its discovery, the Japan TGCV Study Group has attempted to elucidate its pathophysiology, develop diagnostic procedures, and specific treatment. Myocardial scintigraphy with iodine-123-β-methyl iodophenyl-pentadecanoic acid (123I-BMIPP) is a unique imaging modality for evaluating myocardial lipolysis in vivo. The washout rate of 123I-BMIPP is an essential indicator for the diagnosis of TGCV. Along with our efforts to provide awareness of and insights into this disease concept, we found that the cumulative number of clinically diagnosed patients has reached >200 and the cases are distributed throughout Japan. In addition, we successfully completed three investigator-initiated clinical trials of a potential therapeutic agent (CNT-01) for TGCV, which was assigned by the Ministry of Health, Labour, and Welfare, Japan, under the SAKIGAKE Designation System in June 2020. Here, we provide the Diagnostic Criteria 2020 for TGCV in order to further promote this “rare and intractable disease” project.</p>

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