Evaluation of respiratory chain failure in mitochondrial cardiomyopathy by assessments of 99mTc-MIBI washout and 123I-BMIPP/99mTc-MIBI mismatch

Abnormal Myocardial Energy-Production State in Mitochondrial Cardiomyopathy and Acute Response to L-Arginine Infusion - C-11 Acetate Kinetics Revealed by Positron Emission Tomography -

“…23 123 I-BMIPP is a tracer that has been used for assessing myocardial fatty acid metabolism and is associated with the triglyceride (TG) pool. 24 Therefore, on the basis of both our previous 9 and present studies, we suggest that: (1) the decreased rate of 99m Tc-MIBI uptake and its increased washout rate reflected the lower mitochondrial membrane potential created by respiratory chain failure; 9 (2) the lower value of kmono assessed by C-11 acetate PET suggested impairment of the TCA cycle metabolic rate, indicating a shift of energy production to the anaerobic (glycolytic) pathway, thus supporting the lactic acidosis of MELAS; (3) the increased rate of 123 I-BMIPP uptake was influenced by excess in the TG pool, through an amelioration process by reducing dihydroacetone phosphate to glycerol-3-phosphate in order to oxidize superfluous nicotinamide adenine dinucleotide [NADH] to [NAD + ], the excess glycerol-3-phosphate being utilized for synthesis of TG. 9 In this context, we were able to evaluate the energy production state of mitochondrial cardiomyopathy non-invasively and obtain supportive experimental evidence in vivo.…”

“…9 Those findings suggest that oxidative metabolism changes from aerobic to anaerobic, resulting in excessive reduction in energy and impairment of left ventricular (LV) function. As the tricarboxylic acid (TCA) cycle is a series of enzymecatalyzed chemical reactions of central importance in the oxidative metabolism pathway, it is important to evaluate TCA cycle metabolism in order to clarify the energy state of the heart in patients with mitochondrial cardiomyopathy.…”

Fleming-Harrington Redistribution Wash-in Washout (FHRWW): The Platinum Standard for Nuclear Cardiology

“…Furthermore, comparisons of the initial 5-minute imaging with the 60-minute imaging demonstrated different results from the simple "uptake/retention" rest-stress model. Other investigators have noted similar differences between 5 minute and 60 minute imaging of the heart (Hurwitz 1993, Saha 1994, Giubbini 1995, Pace 2005) allowing for enhanced detection of congestive heart failure (Hurwitz 1998, Kumita 2002, Sugiura 2006, Matsuo 2007, cardiomyopathies (Meissner 2002, Ikawa 2007), Prinzmetal 's angina (Ono 2002(Ono , 2003 and underlying coronary artery disease (Meerdink 1990, Richter 1995, Shin 1995, Takeishi 1996, Takahashi 1996, Fujiwara 1998, Hurwitz 1998, Ayalew 2000, Liu 2001, Kumita 2002, Tanaka 2006, Fukushima 2007, VanBrocklin 2007 including evidence of wash-in (Meerdink 1990, Richter 1995 indicative of critical lesions not detected by conventional MPI. In the same way that thrombolysis in myocardial infarction (TIMI) flow is used to look for changes (sometimes subtle, sometimes not) in coronary blood flow in the catch lab, multiple imaging following pharmacologic or EST allows us to take a more advanced look at the physiologic function of the heart, unmasking ischemic heart disease missed by rest-stress imaging.…”

“…This misinformation continued to drive the market reducing study accuracy despite multiple studies reporting that Sestamibi did in fact redistribute. By using this redistribution property, investigators have been better able to define heart failure (Hurwitz 1993, Saha 1994, Giubbini 1995 www.intechopen.com (Matsuo 2007, Ikawa 2007, Meissner 2002) and coronary artery vasospasm (Ono 2002(Ono , 2003. Perhaps the most compelling work was published by Maublant (1988), prior to the introduction of Sestamibi into the United States.…”

Energy States in Mitochondrial Cardiomyopathy

“…11 Our study demonstrated an increased washout rate and decreased uptake of 99m Tc-MIBI, and increased uptake of 123 I-BMIPP (ie, 123 I-BMIPP/ 99m Tc-MIBI mismatch) in patients with severe cardiac involvement. 9 These findings indicated that respiratory chain failure and suppressed oxidative metabolism lead to cardiac dysfunction in patients with mitochondrial cardiomyopathy.…”