Abstract 697: Dyssynchrony in Duchenne Muscular Dystrophy Demonstrated by Tagged MRI Analysis is Independent of Ejection Fraction
Patients with Duchenne Muscular Dystrophy (DMD), a dystrophinopathy, universally develop dilated cardiomyopathy, which is associated with abnormal myocardial strain, as well as heterogeneous development of fibrosis as demonstrated by pathology and more recently by cardiac MRI methods. We sought to determine the presence of systolic dyssynchrony in this population using cardiac MRI tagging methods. We analyzed tagged MRI images for the presence of dyssynchrony in 61 males (age 12.5 ± 4.4 y) with a dystrophin mutation undergoing clinical cardiac MRI. Tagged MRI images were analyzed using HARP® analysis of regional strain and strain-time curves. The mid-myocardial slice was specifically analyzed, by dividing it into 6 coronary perfusion regions. Dyssynchrony was defined by the presence of either of 2 previously published indexes modified for use with MRI data: 1) time difference of 1st to last regional peak strain > 100 ms; 2) standard deviation of time differences to peak strain for each of the six regions > 33 ms. Additional indexes evaluated included heart rate, LV ejection fraction, mid-myocardial composite circumferential strain, and presence of delayed myocardial hyperenhancement (MDE). Among the 61 subjects analyzed, 28 (46%) exhibited dyssynchrony indexes 1 and 2, while 8 additional subjects met dyssynchrony index 2 but not index 1. Only 4 subjects, all of whom met both dyssynchrony criteria, had positive MDE and abnormal EF <55%. The regions of slowest activation were highly dispersed and not clustered to the areas of positive MDE. One additional subject with dyssynchrony by either of the critieria also had abnormal EF but did not have MDE. There was no statistically significant difference between mean EF (61 vs 62%), age (12.58 vs 12.63 yrs), heart rate (105 vs 108 bpm) or mid-myocardial composite circumferential strain (−13.3 vs −12.9%) between those subjects with dyssynchrony (by either criteria) and those without. DMD patients frequently exhibit systolic dyssynchrony even in the presence of normal EF. However, the dispersed nature of the dyssynchrony suggests that resynchronization therapy once EF becomes abnormal is unlikely to be of benefit in DMD cardiomyopathy.