Thallium-201 single photon emission computed tomography (SPECT) in patients with Duchenne's progressive muscular dystrophy - A histopathologic correlation study

The pathomorphologic mechanism responsible for abnormal perfusion imaging d uring thallium-201 myocardial single photon emission computed tomography (T l-201-SPECT) in patients with Duchenne's progressive muscular dystrophy (DM D) was investigated. Hearts from 7 patients with DMD were evaluated histopa thologically at autopsy and the results correlated with findings on initial and delayed resting Tl-201-SPECT images. The location of segments with per fusion defects correlated with the histopathologically abnormal segments in the hearts. Both the extent and degree of myocardial fibrosis were severe, especially in the posterolateral segment of the left ventricle. Severe tra nsmural fibrosis and severe fatty infiltration were common in segments with perfusion defects. In areas of redistribution, the degree of fibrosis appe ared to be greater than in areas of normal perfusion; and intermuscular ede ma was prominent. Thus, the degree and extent of perfusion defects detected by Tl-201-SPECT were compatible with the histopathology. The presence of t he redistribution phenomenon may indicate ongoing fibrosis. Initial and del ayed resting Tl-201-SPECT images can predict the site and progress of myoca rdial degeneration in patients with DMD.