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.

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 Oedema-fibrosis in Duchenne Muscular Dystrophy: Role of cardiovascular magnetic resonance imaging

2017 ◽  
Vol 47 (12) ◽  
pp. e12843 ◽  
Author(s):  
Sophie Mavrogeni ◽  
Antigoni Papavasiliou ◽  
Katerina Giannakopoulou ◽  
George Markousis-Mavrogenis ◽  
Maria Roser Pons ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cardiovascular Magnetic Resonance ◽  
Duchenne Muscular Dystrophy ◽  
Magnetic Resonance ◽  
Muscular Dystrophy ◽  
Cardiovascular Magnetic Resonance Imaging ◽  
Resonance Imaging

scholarly journals Cardiovascular magnetic resonance for early detection of late cardiotoxicity in asymptomatic survivors of hodgkin and non-hodgkin lymphoma

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
N Van Der Velde ◽  
CPM Janus ◽  
DJ Bowen ◽  
HC Hassing ◽  
I Kardys ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Late Gadolinium Enhancement ◽  
Magnetic Resonance ◽  
Ejection Fraction ◽  
B Cell Lymphoma ◽  
Left Ventricular ◽  
Healthy Controls ◽  
Gadolinium Enhancement ◽  
Native T1 ◽  
Late Cardiotoxicity

Abstract Funding Acknowledgements Type of funding sources: None. Background Long-term survivors of Hodgkin (HL) and non-Hodgkin (NHL) lymphomas experience late adverse effects of mediastinal radiotherapy and/or anthracycline containing chemotherapy, which lead to premature cardiovascular morbidity and mortality. It is unknown whether early stages of myocardial dysfunction and heart failure in these survivors can be detected by cardiovascular magnetic resonance imaging (CMR). Purpose To identify early sensitive markers for the detection of subclinical late cardiotoxicity using CMR in asymptomatic survivors of HL and (primary mediastinal large B-cell lymphoma) NHL. Methods For this prospective observational study, we included 80 HL or selected NHL survivors, who have been free of disease for ≥5 years and were treated with mediastinal radiotherapy (RT) with/without chemotherapy. Patients with known cardiac disease were excluded. Included patients were compared to 40 age- and sex matched healthy controls. CMR included 1) cine imaging for assessment of left ventricular (LV) and right ventricular (RV) dimensions, systolic function and strain; 2) 2-dimensional late gadolinium enhancement (LGE) imaging; 3) T2 mapping and 4) pre- and post-contrast T1 mapping (MOLLI) for assessment of native T1 values and extracellular volume (ECV). Results Of the 80 patients, 78 (98%) had a history of HL and 2 (2%) of NHL with a mean age of 47 ± 11 years (46% male). All patients were treated with mediastinal RT which was combined with anthracycline containing chemotherapy in 68 (85%) patients. The median interval between diagnosis and CMR was 20 [14 – 26] years. Differences in CMR characteristics between patients and healthy controls are shown in the table. LV end-systolic volume was statistically significantly higher, but LV ejection fraction and mass were significantly lower in patients compared to healthy controls. RV volumes were significantly lower in patients, but RV ejection fraction was preserved. Strain parameters of the LV, i.e. global longitudinal strain, global circumferential strain and global radial strain, were slightly but significantly reduced in patients. No significant differences were found in myocardial T2 times and ECV; however, native myocardial T1 time was significantly higher in patients compared to healthy controls. LGE was detected in 25% of the patients and in the majority of patients with LGE this was classified as hinge point fibrosis. Conclusion Asymptomatic survivors of HL and NHL are not exempt of late cardiotoxicity, which can be detected by subtle changes in LV myocardial function, strain and native T1 value with CMR. Furthermore, late gadolinium enhancement was present in 25% of the patients. Further longitudinal studies are needed to assess the implication of these changes in relation to clinical outcome.

scholarly journals The reliability and feasibility of non-contrast adenosine stress cardiovascular magnetic resonance T1 mapping in patients on haemodialysis

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Federica E Poli ◽  
Gaurav S Gulsin ◽  
Daniel S March ◽  
Ahmed MSEK Abdelaty ◽  
Kelly S Parke ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Intraclass Correlation ◽  
Adenosine Stress ◽  
Single Shot ◽  
Retest Reliability ◽  
Native T1 ◽  
Dialysis Vintage ◽  
Test Retest Reliability

Abstract Background Identifying coronary artery disease (CAD) in patients with end-stage renal disease (ESRD) is challenging. Adenosine stress native T1 mapping with cardiovascular magnetic resonance (CMR) may accurately detect obstructive CAD and microvascular dysfunction in the general population. This study assessed the feasibility and reliability of adenosine stress native T1 mapping in patients on haemodialysis. Methods The feasibility of undertaking rest and adenosine stress native T1 mapping using the single-shot Modified Look-Locker inversion recovery (MOLLI) sequence was assessed in 58 patients on maintenance haemodialysis using 3 T CMR. Ten patients underwent repeat stress CMR within 2 weeks for assessment of test-retest reliability of native T1, stress T1 and delta T1 (ΔT1). Interrater and intrarater agreement were assessed in 10 patients. Exploratory analyses were undertaken to assess associations between clinical variables and native T1 values in 51 patients on haemodialysis. Results Mean age of participants was 55 ± 15 years, 46 (79%) were male, and median dialysis vintage was 21 (8; 48) months. All patients completed the scan without complications. Mean native T1 rest, stress and ΔT1 were 1261 ± 57 ms, 1297 ± 50 ms and 2.9 ± 2.5%, respectively. Interrater and intrarater agreement of rest T1, stress T1 and ΔT1 were excellent, with intraclass correlation coefficients (ICC) > 0.9 for all. Test-retest reliability of rest and stress native T1 were excellent or good (CoV 1.2 and 1.5%; ICC, 0.79 and 0.69, respectively). Test-retest reliability of ΔT1 was moderate to poor (CoV 27.4%, ICC 0.55). On multivariate analysis, CAD, diabetes mellitus and resting native T1 time were independent determinants of ΔT1 (β = − 0.275, p = 0.019; β = − 0.297, p = 0.013; β = − 0.455; p < 0.001, respectively). Conclusions Rest and adenosine stress native T1 mapping is feasible and well-tolerated amongst patients with ESRD on haemodialysis. Although rater agreement of the technique is excellent, test-retest reliability of ΔT1 is moderate to poor. Prospective studies should evaluate the relationship between this technique and established methods of CAD assessment and association with outcomes.

Abstract 13897: Native T1-mapping by Cardiac MRI as a Biomarker of Remodeling in Boys With Duchenne Muscular Dystrophy

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Seraina A Dual ◽  
Nyasha G Maforo ◽  
Doff B McElhinney ◽  
Ashley Prosper ◽  
Holden Wu ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
T1 Mapping ◽  
Lateral Wall ◽  
Inferior Wall ◽  
Thin Wall ◽  
Native T1 ◽  
The Difference ◽  
Average Size ◽  
The Right

Cardiac magnetic resonance (CMR) is an important tool to assess cardiac disease progression in boys with Duchenne muscular dystrophy (DMD), with native (pre-contrast) myocardial T1-mapping considered a possible biomarker of fibrosis. Due to its thin wall and highly trabeculated structure, the right ventricle (RV) remains understudied in DMD despite pre-clinical evidence of RV involvement. After determining the most robust method of obtaining RV-T1, we assessed the hypothesis that RV-T1 distinguishes healthy controls from boys with DMD. Boys with DMD (N=27) and age-matched healthy control boys (N=17) were prospectively enrolled for a 3T CMR exam (Skyra, Siemens). The CMR exam included standard functional imaging, LGE imaging, and native T1 maps acquired at diastasis. Native RV-T1 was evaluated in four regions of interest (ROIs): 1 pixel (px) along the RV centerline, 3px dilated RV centerline, a segment within the RV lateral wall, and the RV inferior wall (Figure 1). Robustness was assessed in controls only and was defined as an acceptable number of pixels, coefficient of variation (COV) per ROI, percentage of readable images, and lowest inter-observer variability across three observers. Subsequently, a t-test assessed the difference between boys with DMD and controls using the most robust RV ROI. Comparing the four ROIs, we found an average size of the ROI of 65, 196, 23, 25 px; a COV of 13.4%, 16.6%, 6.0%, 9.9%; and 75%, 75%, 93%, 81% of readable images. Intra-class correlation (0.75) was highest and both mean bias (20ms) and limits of agreement (100ms) were lowest for the RV 1px ROI. Using the 1px ROI, the RV-T1 was higher and more variable in boys with DMD compared to controls (1526 [1457,1650] ms vs. 1432 [1390,1486] ms; p<0.0001). Native RV-T1 is elevated in boys with DMD, if measured along a 1px centerline ROI. The methods described herein may enable using native RV-T1 as a biomarker of fibrosis in DMD and other pathologies. Correlation with functional indices is pending.

Abstract 14877: Native T1-based Cardiovascular Magnetic Resonance for Characterizing Chronic Myocardial Infarctions: Proof-of-Concept Study in Canines

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Avinash Kali ◽  
Ivan Cokic ◽  
Richard L Tang ◽  
Hsin J Yang ◽  
Behzad Sharif ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Infarct Size ◽  
T1 Mapping ◽  
Ischemia Reperfusion Injury ◽  
Ex Vivo ◽  
Ischemia Reperfusion ◽  
Infarcted Myocardium ◽  
Native T1 ◽  
Replacement Fibrosis

Introduction: Gadolinium infusion required for Late Gadolinium Enhancement (LGE) Cardiovascular Magnetic Resonance (CMR) imaging is contraindicated in nearly 20% of myocardial infarction (MI) patients due to chronic end-stage kidney disease. Hypothesis: Using a canine model of MI, we investigated whether native T1 mapping at 3T could be an alternative to LGE CMR for characterizing chronic MIs (CMIs). Methods: Canines (n=29) were subjected to ischemia-reperfusion injury. Native T1 maps, native T2 maps and LGE images were acquired at 7 days (acute, AMI) and 4 months (CMI) post-MI at 1.5T and 3T. Infarct location, size and transmurality, measured using Mean + 5 Standard Deviations criterion, were compared between T1 maps and LGE images. Native T2 maps were used to examine the resolution of edema between AMI and CMI. Following the CMR studies, animals were euthanized and ex-vivo histology was performed. Results: T1 maps and LGE images were not different for measuring infarct size (p=0.61) and transmurality (p=0.81) in CMI at 3T. In AMI at 3T, T1 maps overestimated both infarct size (p=0.007) and transmurality (p=0.007) relative to LGE images. At 1.5T, T1 maps underestimated both infarct size and transmurality relative to LGE images in both AMI and CMI (p<0.001 for all cases). Relative to the remote territories, T1 of the infarcted myocardium was elevated in AMI (3T: p<0.001; 1.5T: p<0.001) and CMI (3T: p<0.001; 1.5T: p=0.037). T2 of the infarcted myocardium was elevated in AMI (p<0.001 at both 3T and 1.5T), but not in CMI (3T: p=0.19, 1.5T: p=0.55) indicating that myocardial edema resolved by 4 months post-MI. Masson’s trichrome staining showed extensive replacement fibrosis within CMIs. Sensitivity and specificity of T1 maps to detect CMI were 95% and 97% respectively at 3T, and 58% and 78% respectively at 1.5T. Conclusions: Native T1 mapping at 3T can characterize CMIs with high diagnostic accuracy. T1 elongations in CMI appear to arise predominantly from replacement fibrosis.

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