Abstract
BackgroundDuchenne muscular dystrophy (DMD) leads to progressive cardiomyopathy. Detection of myocardial fibrosis with late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) is critical for clinical management. Due to concerns of brain deposition of gadolinium, non-contrast methods for detecting and monitoring myocardial fibrosis would be beneficial.ObjectivesWe hypothesized that native T1 mapping and/or circumferential (εcc) and longitudinal (εls) strain can detect myocardial fibrosis.Methods156 CMRs with gadolinium were performed in 66 DMD boys and included: (1) left ventricular ejection fraction (LVEF), (2) LGE, (3) native T1 mapping and myocardial tagging (εcc-tag measured using harmonic phase analysis). LGE was graded as: (1) presence/absence by segment, slice, and globally; (2) global severity from 0 (no LGE) to 4 (severe); (3) percent LGE using full width half maximum (FWHM). εls and εcc measured using feature tracking. Regression models to predict LGE included native T1 and either εcc-tag or εls and εcc measured at each segment, slice, and globally.ResultsMean age and LVEF at first CMR were 14 years and 54%, respectively. Global εls and εcc strongly predicted presence or absence of LGE (OR 2.6 [1.1, 6.0], p = 0.029, and OR 2.3 [1.0, 5.1], p = 0.049, respectively) while global native T1 did not. Global εcc, εls, and native T1 predicted global severity score (OR 2.6 [1.4, 4.8], p = 0.002, OR 2.6 [1.4, 6.0], p = 0.002, and OR 1.8 [1.1, 3.1], p = 0.025, respectively). εls correlated with change in LGE by severity score (n = 33, 3.8 [1.0, 14.2], p = 0.048) and εcc-tag correlated with change in percent LGE by FWHM (n = 34, OR 0.2 [0.1, 0.9], p = 0.01).ConclusionsPre-contrast sequences predict presence and severity of LGE, with εls and εcc being more predictive in most models, but there was not an observable advantage over using LVEF as a predictor. Change in LGE was predicted by εls (global severity score) and εcc-tag (FWHM). While statistically significant, our results suggest these sequences are currently not a replacement for LGE and may only have utility in a very limited subset of DMD patients.
Highlights
Duchenne muscular dystrophy (DMD) leads to progressive skeletal and cardiac myopathy and affects up to 1 in 4700 live male births [1]
Εls, and native T1 predicted global severity score. εls correlated with change in late gadolinium enhancement (LGE) by severity score (n = 33, 3.8 [1.0, 14.2], p = 0.048) and εcc-tag correlated with change in percent LGE by full width half maximum (FWHM) (n = 34, OR 0.2 [0.1, 0.9], p = 0.01)
Change in LGE was predicted by εls and εcc-tag (FWHM)
Summary
Duchenne muscular dystrophy (DMD) leads to progressive skeletal and cardiac myopathy and affects up to 1 in 4700 live male births [1]. Some boys with DMD have earlier onset of cardiovascular disease or more rapid progression, requiring either earlier initiation or more rapid intensification of therapy, resulting in early heart failure and death. While transthoracic echocardiography has been the mainstay of cardiovascular evaluation in DMD, the field has increasingly shifted to cardiovascular magnetic resonance (CMR) [5]. This is partially because CMR provides superior image quality, a significant advantage in a patient population with challenging acoustic windows [6]. Detection of myocardial fibrosis with late gadolinium enhancement (LGE) by cardiovascular magnetic resonance (CMR) is critical for clinical management. Due to concerns of brain deposition of gadolinium, non-contrast methods for detecting and monitor‐ ing myocardial fibrosis would be beneficial
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