Diagnostic Criteria and Severity Score for Triglyceride Deposit Cardiomyovasculopathy
-
- Miyauchi Hideyuki
- Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine These authors equally contributed to this work
-
- Hashimoto Chikako
- Laboratory of Cardiovascular Disease, Novel, Non-invasive, and Nutritional Therapeutics, Graduate School of Medicine, Osaka University These authors equally contributed to this work
-
- Ikeda Yoshihiko
- Department of Pathology, National Cerebral and Cardiovascular Center
-
- Li Ming
- Laboratory of Cardiovascular Disease, Novel, Non-invasive, and Nutritional Therapeutics, Graduate School of Medicine, Osaka University
-
- Nakano Yusuke
- Department of Cardiology, Aichi Medical University
-
- Kozawa Junji
- Department of Metabolic Medicine, Graduate School of Medicine, Osaka University
-
- Sai Eiryu
- Department of Cardiovascular Medicine, Juntendo University Graduate School of Medicine
-
- Nagasawa Yasuyuki
- Department of Internal Medicine, Division of Kidney and Dialysis, Hyogo College of Medicine
-
- Sugimura Koichiro
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
-
- Kinugawa Shintaro
- Department of Cardiovascular Medicine, Faculty of Medicine and Graduate School of Medicine, Hokkaido University
-
- Kawaguchi Katsuhiro
- Department of Cardiology, Komaki City Hospital
-
- Shimada Kazunori
- Department of Cardiovascular Medicine, Juntendo University Graduate School of Medicine
-
- Ide Tomomi
- Department of Cardiovascular Medicine, Graduate School of Medicine, Kyushu University
-
- Amano Tetsuya
- Department of Cardiology, Aichi Medical University
-
- Higashi Masahiro
- Department of Radiology, Osaka National Hospital, National Hospital Organization
-
- Inaba Tohru
- Department of Infection Control and Laboratory Medicine, Kyoto Prefectural University of Medicine
-
- Nakamura Hiroshi
- Department of General Medicine, Kure Medical Center and Chugoku Cancer Center
-
- Kobayashi Kunihisa
- Department of Endocrinology and Diabetes Mellitus, Fukuoka University Chikushi Hospital
-
- Hirano Ken-ichi
- Laboratory of Cardiovascular Disease, Novel, Non-invasive, and Nutritional Therapeutics, Graduate School of Medicine, Osaka University These authors equally contributed to this work The principal investigator for the Japan TGCV study group
この論文をさがす
抄録
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.
収録刊行物
-
- Annals of Nuclear Cardiology
-
Annals of Nuclear Cardiology 4 (1), 94-100, 2018
日本心臓核医学会