Cardiac Involvement in Sporadic Inclusion-Body Myositis

W. Utz, S. Schmidt, J. Schulz-Menger, F. Luft, S. Spuler
2010 Circulation  
A 36-year-old man came to the emergency department with acute onset of exertional chest pain. He had had no recent infections, and no cardiovascular risk factors were present. However, the patient had used a wheelchair since his mid-20s because of sporadic inclusion-body myositis, as established by muscle biopsy. There were no other physical findings. Inflammatory markers and troponin T were normal, and the creatine kinase was elevated 2-fold with a significant muscle brain fraction. Chest
more » ... genogram was normal, but ECG showed a normal sinus rhythm at 62 bpm with deep Q waves, tall R waves in the right precordial leads, interventricular conduction delay, and T wave inversion in the left lateral leads (Figure 1 ). Echocardiography did not reveal any abnormalities. Cardiovascular magnetic resonance (CMR) examination was next performed. Two-chamber (Movie I of the online-only Data Supplement) and 4-chamber (Movie II of the online-only Data Supplement) cine images were obtained by balanced, steady-state free precession cine sequences. Hypokinetic wall motion was detected in the midventricular to apical region of the lateral and anterior walls of a left ventricle (LV) of normal size. High spatial resolution turbo spin echo imaging revealed massive fatty replacement of skeletal musculature of trunk which was expectable from upper leg muscle biopsy. Furthermore, extensive epicardial fat was found with regional fatty replacement of subepicardial Figure 1. Twelve-lead electrocardiogram demonstrating deep Q waves, tall R waves in the right precordial leads, interventricular conduction delay, and T inversion in the left lateral leads. Figure 2. Turbo spin echo images showing extensive epicardial fat with fatty replacement of subepicardial layers of the myocardium (large arrows) and suspected intramural fat in the septum (small arrow) in the 2-chamber view (A) and a midventricular short axis (B).
doi:10.1161/circulationaha.109.866178 pmid:20142463 fatcat:j6727dqamjbo5jgyo5rf4l3gi4