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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  • Arakawa Kenichiro
    Department of Cardiology, Faculty of Medical Sciences, University of Fukui
  • Kudo Takashi
    Biomedical Imaging Research Center, Faculty of Medical Sciences, University of Fukui
  • Ikawa Masamichi
    Department of Neurology, Faculty of Medical Sciences, University of Fukui
  • Morikawa Norihiro
    Department of Cardiology, Faculty of Medical Sciences, University of Fukui
  • Kawai Yasuyuki
    Department of Cardiology, Kanazawa Medical University
  • Sahashi Ko
    Department of Neurology, Aichi Medical University School of Medicine
  • Lee Jong-Dae
    Department of Cardiology, Faculty of Medical Sciences, University of Fukui
  • Kuriyama Masaru
    Department of Neurology, Faculty of Medical Sciences, University of Fukui
  • Miyamori Isamu
    Department of Endocrinology, Faculty of Medical Sciences, University of Fukui
  • Okazawa Hidehiko
    Biomedical Imaging Research Center, Faculty of Medical Sciences, University of Fukui
  • Yoneda Makoto
    Department of Neurology, Faculty of Medical Sciences, University of Fukui

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  • – C-11 Acetate Kinetics Revealed by Positron Emission Tomography –

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Abstract

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)<br>

Journal

  • Circulation Journal

    Circulation Journal 74 (12), 2702-2711, 2010

    The Japanese Circulation Society

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