Abnormal Myocardial Energy-Production State in Mitochondrial Cardiomyopathy and Acute Response to L-Arginine Infusion : C-11 Acetate Kinetics Revealed by Positron Emission Tomography
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概要
- 論文の詳細を見る
Background: Cardiomyopathy is a life-threatening condition in patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (known as MELAS). However, no effective therapy has been available until now. In the present study cardiac energetics and acute effects of L-arginine (Arg) were evaluated in MELAS patients. Methods and Results: The 6 patients with MELAS (M-group) and 6 volunteers (C-group) underwent dynamic C-11 acetate positron emission tomography (PET) imaging. TCA-cycle metabolic rate (kmono), myocardial efficiency (double product (DP)/kmono), and myocardial blood flow (MBF) were determined before and after L-Arg administration. Baseline kmono showed a lower value in the M-group than in the C-group (0.051±0.013 vs 0.070±0.019min-1, P=0.055). On the other hand, baseline DP/kmono was significantly greater in the M-group (1.69±5.9 vs 0.95±1.2×105, P=0.004). After L-Arg administration, 4 patients showed significant elevation of kmono. No relationship was observed between the distribution of kmono elevation and the increase in MBF. Conclusions: The TCA cycle metabolic rate is markedly suppressed in MELAS patients, indicating a shift in energy production to the anaerobic pathway, leading to a paradoxical increase in myocardial efficiency. L-Arg can enhance TCA-cycle metabolism, regardless of its vasodilatation effect, and can be used as a treatment for patients with mitochondrial cardiomyopathy. (Circ J 2010; 74: 2702-2711)
- 社団法人 日本循環器学会の論文
- 2010-11-25
著者
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LEE Jong-Dae
First Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui
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Kawai Yasuyuki
Department Of Cardiology Kanazawa Medical University
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Miyamori Isamu
Department Of Endocrinology Faculty Of Medical Sciences University Of Fukui
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Lee Jong-dae
京都大学 医学研究科循環器内科学
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Lee Jong-dae
First Department Of Internal Medicine Faculty Of Medical Sciences University Of Fukui
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Lee Jong-dae
Department Of Cardiovascular Medicine University Of Fukui Faculty Of Medical Sciences
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KUDO Takashi
Biomedical Imaging Research Center, University of Fukui
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OKAZAWA Hidehiko
Biomedical Imaging Research Center, University of Fukui
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Okazawa Hidehiko
Biomedical Imaging Research Center Faculty Of Medical Sciences University Of Fukui
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Morikawa Norihiro
Department of Cardiovascular Medicine, University of Fukui Hospital
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Arakawa Kenichiro
Department of Cardiovascular Medicine, University of Fukui Hospital
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Lee Jong-dae
Departments Of First Internal Medicine Fukui Medical University
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Kudo Tadashi
Biomedical Imaging Research Center Faculty Of Medical Sciences University Of Fukui
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Kudo Takashi
Biomedical Imaging Research Center University Of Fukui
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Yoneda Makoto
Second Department Of Internalmedicine Faculty Of Medical Sciences Univerisity Of Fukui
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Yoneda Makoto
Department Of Gene Therapy Research Gifu International Institute Of Biotechnology
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Arakawa Kenichiro
Third Department Of Internal Medicine Faculty Of Medical Sciences University Of Fukui
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Morikawa Norihiro
Department Of Cardiovasucular Medicine Fukui University Hospital
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Sahashi Ko
Department of neurology, Aichi medical university
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Kuriyama M
Second Department Of Internal Medicine Faculty Of Medical Science University Of Fukui
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Kuriyama Masaru
Department Of Internal Medicine Fukui Medical University Matsuoka
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Igawa Masamichi
Second Department Of Internalmedicine Faculty Of Medical Sciences Univerisity Of Fukui
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Sahashi Ko
Department Of Internal Medicine Division Of Neurology Aichi Medical University School Of Medicine
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Sahashi Ko
Department Of Neurology Aichi Medical University
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Miyamori Isamu
Third Department Of Internal Medicine University Of Fukui Faculty Of Medical Sciences
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Kuriyama Masaru
Second Department Of Internal Medicine Faculty Of Medical Science University Of Fukui
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Lewis J
Washington Univ. Medical Center St. Louis Usa
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Arakawa Kenichiro
Department Of Cardiology Faculty Of Medical Sciences University Of Fukui
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IKAWA Masamichi
Department of Neurology, Faculty of Medical Sciences, University of Fukui
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Lee Jong-dae
Department Of Cardiology Faculty Of Medical Sciences University Of Fukui
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Kumano Takanori
Biomedical Imaging Research Center University Of Fukui
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Morikawa Norihiro
Department Of Cardiology Faculty Of Medical Sciences University Of Fukui
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Kobayashi Yasuyuki
Department Of Cardiology Kanazawa Medical University
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Kawai Yasuyuki
Department Of Cardiology Faculty Of Medical Sciences Kanazawa University School Of Medicine
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Kudo Takashi
Biomedical Imaging Research Center (birc) University Of Fukui
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