scholarly journals Non-contrast cardiovascular magnetic resonance detection of myocardial fibrosis in Duchenne muscular dystrophy

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Frank J. Raucci ◽  
Meng Xu ◽  
Kristen George-Durrett ◽  
Kimberly Crum ◽  
James C. Slaughter ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Duchenne Muscular Dystrophy ◽  
Magnetic Resonance ◽  
Muscular Dystrophy ◽  
Myocardial Fibrosis ◽  
Severity Score ◽  
T1 Mapping ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Native T1

Abstract Background Duchenne 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. Objectives We hypothesized that native T1 mapping and/or circumferential (εcc) and longitudinal (εls) strain can detect myocardial fibrosis. Methods 156 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. Results Mean 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). Conclusions Pre-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.

scholarly journals Global, segmental and layer specific analysis of myocardial involvement in Duchenne muscular dystrophy by cardiovascular magnetic resonance native T1 mapping

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Ke Xu ◽  
Hua-yan Xu ◽  
Rong Xu ◽  
Lin-jun Xie ◽  
Zhi-gang Yang ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Duchenne Muscular Dystrophy ◽  
Magnetic Resonance ◽  
Muscular Dystrophy ◽  
T1 Mapping ◽  
Healthy Controls ◽  
Native T1 ◽  
Specific Analysis ◽  
Epicardial Layer ◽  
Myocardial Involvement

Abstract Background Progressive cardiomyopathy accounts for almost all mortality among Duchenne muscular dystrophy (DMD) patients.‍ Thus, our aim was to comprehensively characterize myocardial involvement by investigating the heterogeneity of native T1 mapping in DMD patients using global and regional (including segmental and layer-specific) analysis across a large cohort. Methods We prospectively enrolled 99 DMD patients (8.8 ± 2.5 years) and 25 matched male healthy controls (9.5 ± 2.5 years). All subjects underwent cardiovascular magnetic resonance (CMR) with cine, T1 mapping and late gadolinium enhancement (LGE) sequences. Native T1 values based on the global and regional myocardium were measured, and LGE was defined. Results LGE was present in 49 (49%) DMD patients. Global native T1 values were significantly longer in LGE-positive (LGE +) patients than in healthy controls, both in basal slices (1304 ± 55 vs. 1246 ± 27 ms, p < 0.001) and in mid-level slices (1305 ± 57 vs. 1245 ± 37 ms, p < 0.001). No significant difference in global native T1 was found between healthy controls and LGE-negative (LGE−) patients. In segmental analysis, LGE + patients had significantly increased native T1 in all analyzed segments compared to the healthy control group. Meanwhile, the comparison between LGE− patients and healthy controls showed significantly elevated values only in the basal anterolateral segment (1273 ± 62 vs. 1234 ± 40 ms, p = 0.034). Interestingly, the epicardial layer had a significantly higher native T1 in LGE− patients than in healthy controls (p < 0.05), whereas no such pattern was noticed in the global myocardium. Epicardial layer native T1 resulted in the highest diagnostic performance for distinguishing between healthy controls and DMD patients in receiver operating curve analyses (area under the curve [AUC] 0.84 for basal level and 0.85 for middle level) when compared to global native T1 and endocardial layer native T1. Conclusions Myocardial regional native T1, particularly epicardial native T1, seems to have potential as a novel robust marker of very early cardiac involvement in DMD patients. Trial registration: Chinese Clinical Trial Registry (http://www.chictr.org.cn/index.aspx) ChiCTR1800018340, 09/12/2018, Retrospectively registered.

Comprehensive evaluation of structural and functional myocardial impairments in Becker muscular dystrophy using quantitative cardiac magnetic resonance imaging

2018 ◽  
Vol 20 (8) ◽  
pp. 906-915 ◽  
Author(s):  
Benjamin Marty ◽  
Raymond Gilles ◽  
Marcel Toussaint ◽  
Anthony Béhin ◽  
Tanya Stojkovic ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Muscular Dystrophy ◽  
Comprehensive Evaluation ◽  
Becker Muscular Dystrophy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Functional Impairments ◽  
Ventricular Ejection Fraction ◽  
Native T1

Abstract Aims Becker muscular dystrophy (BMD) is a genetic neuromuscular disease characterized by an alteration of the dystrophin protein. Myocardial involvement is frequent, eventually progressing to a dilated cardiomyopathy, and represents the most common cause of death for this pathology. We performed a comprehensive evaluation of myocardial functional and structural alterations encountered in a large cohort of BMD patients using quantitative cardiac magnetic resonance (CMR) imaging. Methods and results Eighty-eight BMD patients and 26 age-matched volunteers underwent standard cine and tag imaging to assess myocardial function and dyssynchrony, while native T1, T2, and extracellular volume fraction (ECV) were measured for tissue characterization. The left ventricular ejection fraction (LV-EF) was significantly reduced in 26% of the BMD patients. Patients exhibited higher dyssynchrony index than controls (6.94 ± 3.17 vs. 5.09 ± 1.25, P = 0.005). Diastolic dyssynchrony also exists in patients where systolic function was normal. BMD subjects, compared with controls, had significantly higher native T1, T2, and ECV (1183 ± 60 ms vs. 1164 ± 22 ms, 47.5 ± 4.5 ms vs. 45.6 ± 3.4 ms, 0.282 ± 0.050 vs. 0.231 ± 0.027, respectively, P < 0.05). Native T1, T2, and ECV correlated with LV-EF (R = −0.79, −0.70, and −0.71, respectively, P < 0.001) and N-terminal-pro brain natriuretic peptide (R = 0.51, 0.58, and 0.44, respectively, P < 0.001). Conclusion Quantitative CMR represents a powerful tool to evaluate structural and functional impairments in the myocardium of BMD subjects. Native T1, T2, and ECV provided quantitative biomarkers related to inflammation and fibrosis, and could stratify disease severity.

Abstract 16030: Myocardial Abnormalities in Competitive Athletes on T1 and T2 Parametric Mapping by Cardiovascular Magnetic Resonance Imaging

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Marc Lee ◽  
Richard Lafountain ◽  
Juliet Varghese ◽  
Christopher Hummel ◽  
James Borchers ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
T2 Mapping ◽  
Sports Participation ◽  
Myocardial Edema ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Native T1 ◽  
Competitive Athletes ◽  
T1 And T2

Introduction: Athletic cardiac adaptation is associated with structural changes that can overlap with disease states, unnecessarily limiting sports participation. Cardiovascular magnetic resonance (CMR) is useful in athlete’s heart and provides myocardial tissue characterization by T1 and T2 mapping. Hypothesis: CMR in competitive athletes will show abnormal T1 and T2 mapping due to intense exercise induced myocardial edema that can overlap with myocarditis. Methods: CMR data including left ventricular ejection fraction (LVEF) and T1/T2 maps were collected using standardized protocols on a 1.5 T scanner and compared between competitive athletes (N = 18, 83% male, median age 20 years), clinical myocarditis (N = 42, 71% male, median age 23 years) and controls (N = 35, 86% male, median age 22 years) between 2016-2020. T2 values of <59 ms and native T1 <1080 ms were defined as normal per institutional data. Extracellular volume fraction (ECV) and late gadolinium enhancement (LGE) were compared between athlete and myocarditis groups. Results: Figure 1 (panel A) shows participating sport and indications for CMR in athletes. There were 11 athletes (61%) with elevated T2 values (>59 ms), of which 9 (82%) were without clinical myocarditis. Average T2, native T1, ECV, and LVEF are shown in panels B-E. T2 values were highest in myocarditis, followed by athletes and controls (p = 0.001). ECV was higher in myocarditis compared to athletes (p = 0.002). LGE was present in 8/18 athletes and 41/42 myocarditis patients. 6 athletes had follow-up CMR after a period of deconditioning, with 3 (50%) demonstrating an improvement in T2 values and LGE. Conclusions: To conclude, we demonstrate abnormalities on T2 mapping in athletes consistent with myocardial edema or inflammation. Changes in T2 may be related to intense training. Additional studies are required to prospectively evaluate athletes for normative T1 and T2 mapping values, relationship to training, and their correlation with LGE.

Abstract 12450: Myocardial Fibrosis Burden Predicts Left Ventricular Ejection Fraction and is Modified by Age and Steroid Treatment Duration in Duchenne Muscular Dystrophy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Animesh Tandon ◽  
Chet R Villa ◽  
Kan N Hor ◽  
John L Jefferies ◽  
Zhiqian Gao ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Myocardial Fibrosis ◽  
Cardiac Dysfunction ◽  
Treatment Duration ◽  
Left Ventricular ◽  
Steroid Treatment ◽  
Left Ventricular Ejection ◽  
Patient Age ◽  
Patient Âgé

Background: Patients with Duchenne muscular dystrophy (DMD) typically exhibit progressive cardiac and skeletal muscle dysfunction, and are commonly treated with corticosteroids to prolong ambulation. Myocardial fibrosis and steroid treatment may modulate the progression of cardiac dysfunction in DMD patients. We aimed to longitudinally characterize the impact of myocardial fibrosis and steroid treatment on the progression of cardiac dysfunction using cardiac magnetic resonance (CMR) in a large DMD cohort. Methods: Serial CMR studies performed on DMD patients were reviewed for LVEF and late gadolinium enhancement (LGE) status, a marker for myocardial fibrosis. LVEF was modeled by examining effects of patient age, steroid treatment duration, LGE status, and myocardial fibrosis burden, as assessed by the number of LGE positive (LGE+) LV segments. Results: We analyzed 469 CMR studies from 99 DMD patients with ≥4 CMRs. Patient age at time of CMR ranged from 6.6 to 29.4 (median 12.3) years. There were 146 (31.1%) LGE+ studies and 59 studies (12.6%) that demonstrated depressed LVEF (LVEF<55%). An age-only model demonstrated that LVEF declined 0.58±0.10%/yr (p<0.0001, r 2 =0.067). Univariate modeling showed significant associations between LVEF and steroid treatment duration, presence of LGE, and number of LGE+ LV segments; multivariate modeling showed that LVEF declined by 0.93±0.09% for each LGE+ LV segment (p<0.0001, r 2 =0.171), while age and steroid treatment duration were no longer significant. The number of LGE+ LV segments increased with age by 1.2 segments/year (95% confidence interval 1.1-1.2), and steroid treatment partially mitigated this increase (interaction term β=-0.01±0.005, p=0.010). Conclusions: Progressive myocardial fibrosis, as imaged by LGE on CMR, is a strong marker for the decline in LVEF in DMD patients. Steroid treatment partially attenuates the age-related increase in myocardial fibrosis burden.

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