Year: 2018
Type: article
Study design: Observational Study
Abstract: Triglyceride deposit cardiomyovasculopathy (TGCV) is a rare and intractable disease, first reported in Japanese patients with congestive heart failure (HF) requiring heart transplant. TGCV is characterized by the excessive accumulation of triglyceride (TG) in cardiomyocytes and vascular smooth muscle cells, which leads to coronary artery disease, HF, and arrhythmia. In TGCV, long-chain fatty acid (LCFA), a major energy source for the normal heart, accumulates as TG in cytoplasmic lipid droplets. In 2009, we launched the Japan TGCV study group to elucidate the pathophysiology of TGCV and have developed diagnostic procedures along with specific treatment. Single-photon emission computed tomography (SPECT) with iodine-123-β-methyl iodophenyl-pentadecanoic acid (BMIPP), a radioactive analogue for LCFA, is a useful diagnostic tool to detect impaired myocardial LCFA metabolism in TGCV. Since we posted the latest version of diagnostic criteria including the myocardial washout rate of BMIPP in SPECT in 2016, we have identified 138 patients with TGCV, 27 of whom have died. More recently, we developed a TGCV severity score consisting of specific questionnaires in order to assess symptoms and activities of daily living in patients with TGCV.
Source: Annals of Nuclear Cardiology
Authors: Hideyuki Miyauchi, Chikako Hashimoto, Yoshihiko Ikeda, Ming Li, Yusuke Nakano, Junji Kozawa, Eiryu Sai, Yasuyuki Nagasawa, Koichiro Sugimura, Shintaro Kinugawa, Katsuhiro Kawaguchi, Kazunori Shimada, Tomomi Ide, Tetsuya Amano, Masahiro Higashi, Tohru Inaba, Hiroshi Nakamura, Kunihisa Kobayashi, Ken-ichi Hirano
Institutions: Chiba University, The University of Osaka, National Cerebral and Cardiovascular Center, Aichi Medical University, Juntendo University, Hyogo Medical University, Tohoku University, Hokkaido University, Komaki City Hospital, Kyushu University, Osaka National Hospital, Kyoto Prefectural University of Medicine, Kure Medical Center, Fukuoka University
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W2888872893