Evaluation of epicardial and intra-myocardial fat in a patient with mitochondrial cardiomyopathy

Abstract

Mitochondrial cardiomyopathy is one of the main manifestations in patients with primary mitochondrial respiratory chain disease characterized by mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and carries a similar prognosis. The phenotype frequently includes hypertrophic cardiomyopathy; dilated cardiomyopathy and left ventricular (LV) non-compaction are rarely present [ [1] Bates M.G. Bourke J.P. Giordano C. d'Amati G. Turnbull D.M. Taylor R.W. Cardiac involvement in mitochondrial DNA disease: clinical spectrum, diagnosis, and management. Eur Heart J. 2012; 33: 3023-3033 Crossref PubMed Scopus (147) Google Scholar ]. Cardiac magnetic resonance imaging (MRI) with gadolinium-based contrast agents has been widely used for evaluating the morphology and function of the left ventricle and injured myocardial tissue [ [2] Okayama S. Uemura S. Soeda T. et al. Clinical significance of papillary muscle late enhancement detected via cardiac magnetic resonance imaging in patients with single old myocardial infarction. Int J Cardiol. 2011; 146: 73-79 Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar ]. Cine imaging with a standard steady state free precession sequence has been validated for accurate evaluation of epicardial fat [ [3] Nelson A.J. Worthley M.I. Psaltis P.J. et al. Validation of cardiovascular magnetic resonance assessment of pericardial adipose tissue volume. J Cardiovasc Magn Reson. 2009; 5: 15 Crossref Scopus (81) Google Scholar ], which is associated with coronary atherosclerosis and LV systolic dysfunction [ [4] Khawaja T. Greer C. Chokshi A. et al. Epicardial fat volume in patients with left ventricular systolic dysfunction. Am J Cardiol. 2011; 108: 397-401 Abstract Full Text Full Text PDF PubMed Scopus (60) Google Scholar ]. Recent advances in magnetic resonance spectroscopy (MRS) have enabled noninvasive measurement of intra-myocardial triglyceride content [ [5] Nakae I. Mitsunami K. Yoshino T. et al. Clinical features of myocardial triglyceride in different types of cardiomyopathy assessed by proton magnetic resonance spectroscopy: comparison with myocardial creatine. J Card Fail. 2010; 16: 812-822 Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar ], which is increased and associated with LV diastolic dysfunction in patients with diabetes mellitus [ 6 McGavock J.M. Lingvay I. Zib I. et al. Cardiac steatosis in diabetes mellitus: a 1H-magnetic resonance spectroscopy study. Circulation. 2007; 116: 1170-1175 Crossref PubMed Scopus (464) Google Scholar , 7 Rijzewijk L.J. van der Meer R.W. Smit J.W. et al. Myocardial steatosis is an independent predictor of diastolic dysfunction in type 2 diabetes mellitus. J Am Coll Cardiol. 2008; 52: 1793-1799 Abstract Full Text Full Text PDF PubMed Scopus (423) Google Scholar ].