{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:26Z","timestamp":1784557046036,"version":"3.55.0"},"reference-count":10,"publisher":"Bioscientifica","issue":"2","license":[{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,4,1]]},"abstract":"\n Summary<\/jats:title>\n \n Triglyceride deposit cardiomyovasculopathy (TGCV) is an intractable disease characterized by massive triglyceride (TG) accumulation in the myocardium and coronary arteries caused by genetic or acquired dysfunction of adipose TG lipase (ATGL). A phase IIa trial has been conducted involving patients with idiopathic TGCV using CNT-01 (tricaprin\/trisdecanoin) by the Japan TGCV study group, which showed that CNT-01 improved myocardial lipolysis as demonstrated by iodine-123-beta-methyl iodophenyl-pentadecanoic acid (BMIPP) scintigraphy. We evaluated changes in myocardial TG content using proton magnetic resonance spectroscopy (\n 1<\/jats:sup>\n H-MRS) before\/after CNT-01. This report describes a male patient with hypertension, diabetes, angina pectoris, repeated percutaneous coronary intervention, chest pain, and exertional dyspnea that persisted despite standard medications and nitroglycerin. Idiopathic TGCV was diagnosed based on a remarkably reduced washout rate (WR) for BMIPP scintigraphy, high myocardial TG content on\n 1<\/jats:sup>\n H-MRS, and no ATGL mutation. After an 8-week, 1.5 g\/day CNT-01 administration, the WR of BMIPP increased from 5.1 to 13.3% and the myocardial TG content decreased from 8.4 to 5.9%, with no adverse effects. CNT-01 corrected myocardial lipolysis and subsequently reduced TG content in idiopathic TGCV as evaluated using\n 1<\/jats:sup>\n H-MRS, which may be a useful, noninvasive evaluation of therapeutic efficacy.\n <\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Triglyceride deposit cardiomyovasculopathy (TGCV) is an intractable disease characterized by massive triglyceride accumulation in the myocardium and coronary arteries, caused by genetic or acquired dysfunction of adipose triglyceride lipase.<\/jats:p>\n <\/jats:list-item>\n \n Japan TGCV Study Group developed a specific treatment for idiopathic TGCV using CNT-01 (tricaprin\/trisdecanoin), a type of medium-chain fatty acid.<\/jats:p>\n <\/jats:list-item>\n \n CNT-01 corrected myocardial lipolysis and reduced TG content in idiopathic TGCV using proton magnetic resonance spectroscopy, which may be a useful noninvasive evaluation of therapeutic efficacy.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-22-0370","type":"journal-article","created":{"date-parts":[[2023,4,21]],"date-time":"2023-04-21T10:00:35Z","timestamp":1682071235000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":1,"title":["1H-MRS to evaluate improved triglyceride accumulation in idiopathic triglyceride deposit cardiomyovasculopathy after CNT-01 (tricaprin\/trisdecanoin) administration"],"prefix":"10.1530","volume":"2023","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1656-2660","authenticated-orcid":true,"given":"Tatsuro","family":"Aikawa","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2588-3797","authenticated-orcid":true,"given":"Eiryu","family":"Sai","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayako","family":"Kudo","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko O","family":"Kawaguchi","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhisa","family":"Takamura","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"},{"name":"Department of Cardiovascular Medicine, Juntendo University Urayasu Hospital, Tomioka, Urayasu-shi, Chiba, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makoto","family":"Hiki","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayuki","family":"Yokoyama","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tetsuro","family":"Miyazaki","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"},{"name":"Department of Cardiovascular Medicine, Juntendo University Urayasu Hospital, Tomioka, Urayasu-shi, Chiba, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinichiro","family":"Fujimoto","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazunori","family":"Shimada","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"},{"name":"Sportology Center, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken-ichi","family":"Hirano","sequence":"additional","affiliation":[{"name":"Laboratory of Cardiovascular Disease, Novel, Non-invasive, and Nutritional Therapeutics and Triglyceride Research Center (TGRC), Department of Triglyceride Science, Graduate School of Medicine, Osaka University, Furuedai, Suita, Osaka, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Daida","sequence":"additional","affiliation":[{"name":"Faculty of Health Science, Juntendo University, Hongo, Bunkyo-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tohru","family":"Minamino","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan"},{"name":"Japan Agency for Medical Research and Development-Core Research for Evolutionary Medical Science and Technology (AMED-CREST), Japan Agency for Medical Research and Development, Otemachi, Chiyoda-ku, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"New England Journal of Medicine","first-page":"2396","article-title":"Triglyceride deposit cardimyovasculopathy","volume":"359","author":"Hirano","year":"2008","unstructured":"Hirano KIkeda YZaima NSakata Y & Matsumiya G. 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