Annals of Nuclear Cardiology
Online ISSN : 2424-1741
Print ISSN : 2189-3926
ISSN-L : 2189-3926
Guidelines/Recommendations/Statements
The Diagnostic Criteria 2020 for Triglyceride Deposit Cardiomyovasculopathy
Kunihisa KobayashiYasuhiko SakataHideyuki MiyauchiYoshihiko IkedaYasuyuki NagasawaKenichi NakajimaKazunori ShimadaJunji KozawaHiroyuki HaoTetsuya AmanoHiroshi YoshidaTohru InabaChikako HashimotoKen-ichi Hirano for the Japan TGCV Study Group
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2020 Volume 6 Issue 1 Pages 99-104

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Abstract

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.

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© 2020 The Japanese Society of Nuclear Cardiology
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