Cardiomyopathy in Duchenne muscular dystrophy (DMD) is associated with death in approximately 40% of patients. Echocardiography is routinely used to assess left ventricular (LV) function; however, it has limitations in these patients. Here, the authors compared echocardiographic measures of cardiac function assessment to cardiac MRI in children and young adults with DMD. Presence of late gadolinium enhancement (LGE) was assessed by MRI. Subjects were divided into two groups based on MRI left ventricular ejection fraction (LVEF): group I, LVEF ≥55% and group II, LVEF <55%. A total of 41 studies in 33 subjects was included, with 25 in group I and 16 in group II. Only 8 of 16 (50%) patients in group II had diminished function on echocardiogram. Echocardiographic images were suboptimal in 16 subjects (39%). Overall, echocardiographic parameters had weak correlation with MRI-derived ejection fraction percentage. MRI-derived myocardial strain assessment has better correlation with MRI ejection fraction as compared to echocardiography-derived strain parameters. Echocardiography-based ventricular functional assessment has weak correlation with MRI parameters in children and young adults with Duchenne muscular dystrophy. While this correlation improves in the subset of subjects with adequate echocardiographic image quality, it remains modest and potentially suboptimal for clinical management.
Buddhe S, Lewin M, Olson A, Ferguson M, Soriano BD. Comparison of left ventricular function assessment between echocardiography and MRI in Duchenne muscular dystrophy. Pediatr Radiol. 2016 May 12. [Epub ahead of print] http://www.ncbi.nlm.nih.gov/pubmed/27173979
