scholarly journals Subclinical myocardial inflammation and diffuse fibrosis are common in systemic sclerosis – a clinical study using myocardial T1-mapping and extracellular volume quantification

2014 ◽  
Vol 16 (1) ◽  
pp. 21 ◽  
Author(s):  
Ntobeko AB Ntusi ◽  
Stefan K Piechnik ◽  
Jane M Francis ◽  
Vanessa M Ferreira ◽  
Aitzaz BS Rai ◽  
...  
2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 702.2-702
Author(s):  
R. B. Dumitru ◽  
A. Goodall ◽  
D. Broadbent ◽  
A. Kidambi ◽  
S. Plein ◽  
...  

Background:Peripheral myositis in systemic sclerosis (SSc) is associated with poor prognosis and myocarditis1but non-inflammatory myopathy, which also represents a significant cause of disability in SSc remains poorly understood. Cardiovascular magnetic resonance (CMR) T1 mapping studies in asymptomatic SSc patients show increased extracellular volume (ECV), suggestive of diffuse fibrosis in both the myocardium and thoracic muscle2.Objectives:To evaluate the feasibility of T1 mapping MRI and determine ECV in peripheral muscle of SSc patients with and without myopathy and explore the association between cardiac and peripheral muscle T1 mapping in SSc.Methods:This was a hypothesis-generating pilot and feasibility study. SSc patients, fulfilling the 2013 ACR/EULAR criteria, with no cardiovascular disease or myositis but either minimal muscle symptoms (non-inflammatory myopathy) or no muscle involvement and healthy volunteers (HV) underwent peripheral muscle T1 mapping-MRI for native T1 and extracellular volume (ECV) quantification of the dominant thigh. Patients also had T1 mapping CMR, and creatine-kinase (CK) measured. Non-inflammatory myopathy was defined as current/history of minimally raised CK (<600 IU/l) +/- presence of clinical signs-symptoms (including proximal myasthenia and/or myalgia) +/- a Manual Muscle Testing (MMT) score <5 in the thighs.Results:12 SSc patients and 10 HV were recruited. SSc patients had a median (IQR) age of 52 (41,65) years, 9/12 had limited cutaneous SSc, 4/12 interstitial lung disease, 7/12 non-inflammatory myopathy. Higher skeletal muscle ECV was recorded in SSc patients compared to HV [mean (SD) 23(11)%, vs 11(4)% p=0.04]. Skeletal muscle native T1 values were comparable between the 2 groups although modestly higher in SSc patients [mean (SD) 1396ms (56) vs 1387ms (42)] (Figure 1A).Peripheral muscle ECV associated with CK (R2=0.307, rho=0.554, p=0.061) and was higher in SSc patients with evidence of myopathy compared to those with no myopathy [28 (10)% vs 15 (5)%, p=0.023] (Figure 1B). An ECV of 22% was determined to best identify myopathy with a sensitivity of 71% and a specificity of 80%.SSc patients had raised myocardial ECV and native T1 with means (SD) of 31 (3) % and 1287 (54) ms respectively (normal reference range ECV ≤29%, native T1 ≤1240ms). No clear association between myocardial and peripheral muscle ECV (rho=-0.485) or between myocardial ECV and peripheral muscle native T1 (rho=0.470) of SSc patients was observed.Conclusion:This pilot study to determine ECV-MRI of the peripheral muscle showed higher ECV in SSc patients compared to HV, suggesting the presence of diffuse fibrosis in the peripheral muscle of SSc patients. These data support further investigation to understand the pathophysiological involvement and relationship of peripheral and cardiac muscle in SSc.References:[1]Follansbee WP et al, Am Heart J. 1993[2]Barison A et al, Eur Heart J Cardiovasc Imaging. 2015Disclosure of Interests:Raluca-Bianca Dumitru: None declared, Alex Goodall: None declared, David Broadbent: None declared, Ananth Kidambi: None declared, Sven Plein: None declared, Francesco Del Galdo: None declared, Ai Lyn Tan: None declared, John Biglands: None declared, Maya H Buch Grant/research support from: Pfizer, Roche, and UCB, Consultant of: Pfizer; AbbVie; Eli Lilly; Gilead Sciences, Inc.; Merck-Serono; Sandoz; and Sanofi


2021 ◽  
Vol 76 (2) ◽  
pp. 162.e1-162.e8
Author(s):  
V. Bordonaro ◽  
D. Bivort ◽  
T. Dresselaers ◽  
E. De Langhe ◽  
J. Bogaert ◽  
...  

Diagnostics ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. 335
Author(s):  
George Markousis-Mavrogenis ◽  
Loukia Koutsogeorgopoulou ◽  
Gikas Katsifis ◽  
Theodoros Dimitroulas ◽  
Genovefa Kolovou ◽  
...  

Aims: T1-mapping is considered a surrogate marker of acute myocardial inflammation. However, in diffuse cutaneous systemic sclerosis (dcSSc) this might be confounded by coexisting myocardial fibrosis. We hypothesized that T1-based indices should not by themselves be considered as indicators of myocardial inflammation in dcSSc patients. Methods/Results: A cohort of 59 dcSSc and 34 infectious myocarditis patients was prospectively evaluated using a 1.5-Tesla system for an indication of suspected myocardial inflammation and was compared with 31 healthy controls. Collectively, 33 (97%) and 57 (98%) of myocarditis and dcSSc patients respectively had ≥1 pathologic T2-based index. However, 33 (97%) and 45 (76%) of myocarditis and dcSSc patients respectively had ≥1 pathologic T2-based index. T2-signal ratio was significantly higher in myocarditis patients compared with dcSSc patients (2.5 (0.6) vs. 2.1 (0.4), p < 0.001). Early gadolinium enhancement, late gadolinium enhancement and T2-mapping did not differ significantly between groups. However, both native T1-mapping and extracellular volume fraction were significantly lower in myocarditis compared with dcSSc patients (1051.0 (1027.0, 1099.0) vs. 1120.0 (1065.0, 1170.0), p < 0.001 and 28.0 (26.0, 30.0) vs. 31.5 (30.0, 33.0), p < 0.001, respectively). The original Lake Louise criteria (LLc) were positive in 34 (100%) myocarditis and 40 (69%) dcSSc patients, while the updated LLc were positive in 32 (94%) and 44 (76%) patients, respectively. Both criteria had good agreement with greater but nonsignificant discordance in dcSSc patients. Conclusions: ~25% of dcSSc patients with suspected myocardial inflammation had no CMR evidence of acute inflammatory processes. T1-based indices should not be used by themselves as surrogates of acute myocardial inflammation in dcSSc patients.


Cardiology ◽  
2017 ◽  
Vol 138 (4) ◽  
pp. 207-217 ◽  
Author(s):  
Sophie Mavrogeni ◽  
Dimitris Apostolou ◽  
Panayiotis Argyriou ◽  
Stella Velitsista ◽  
Lilika Papa ◽  
...  

The increasing use of cardiovascular magnetic resonance (CMR) is based on its capability to perform biventricular function assessment and tissue characterization without radiation and with high reproducibility. The use of late gadolinium enhancement (LGE) gave the potential of non-invasive biopsy for fibrosis quantification. However, LGE is unable to detect diffuse myocardial disease. Native T1 mapping and extracellular volume fraction (ECV) provide knowledge about pathologies affecting both the myocardium and interstitium that is otherwise difficult to identify. Changes of myocardial native T1 reflect cardiac diseases (acute coronary syndromes, infarction, myocarditis, and diffuse fibrosis, all with high T1) and systemic diseases such as cardiac amyloid (high T1), Anderson-Fabry disease (low T1), and siderosis (low T1). The ECV, an index generated by native and post-contrast T1 mapping, measures the cellular and extracellular interstitial matrix (ECM) compartments. This myocyte-ECM dichotomy has important implications for identifying specific therapeutic targets of great value for heart failure treatment. On the other hand, T2 mapping is superior compared with myocardial T1 and ECM for assessing the activity of myocarditis in recent-onset heart failure. Although these indices can significantly affect the clinical decision making, multicentre studies and a community-wide approach (including MRI vendors, funding, software, contrast agent manufacturers, and clinicians) are still missing.


Author(s):  
Alexander Brendel ◽  
Jens Kübler ◽  
Sebastian Gassenmaier ◽  
Florian Hagen ◽  
Jan Michael Brendel ◽  
...  

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Kjellstad Larsen ◽  
J Duchenne ◽  
E Galli ◽  
J M Aalen ◽  
E Kongsgaard ◽  
...  

Abstract Funding Acknowledgements The study was supported by Center for Cardiological Innovation Background Myocardial scar burden (focal fibrosis) is associated with poor response to cardiac resynchronization therapy (CRT), and should preferably be detected prior to device implantation. Late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is considered reference standard for scar detection, but is not available in renal failure. Diffuse fibrosis is assessed by T1 mapping CMR with or without calculation of extracellular volume fraction (ECV). The method is vulnerable to partial volume effects, thus subendocardial tissue is most often not included in mapping analyses. Whether the contrast-free native T1mapping could replace LGE in the preoperative evaluation of patients referred for CRT is unknown. Purpose To investigate if native T1 mapping and calculation of ECV can adequately detect scar in patients referred for CRT. Methods Scar was quantified as percentage segmental LGE in 45 patients (age 65 ± 10 years, 71% male, QRS-width 165 ± 17ms) referred for CRT. In total 720 segments were analyzed, and LGE≥50% was considered transmural scar. T1-mapping before and after contrast agent injection was performed in all patients. ECV was calculated based on the ratio between tissue T1 relaxation change and blood T1 relaxation change after contrast agent injection, corrected for the haematocrit level. The agreement between native T1/ECV and scar was evaluated with receiver operating characteristic (ROC) curves with calculation of area under the curve (AUC) and 95% confidence interval (CI). Results LGE was present in 255 segments, 465 segments were without LGE. Average native T1 in segments with LGE was 1028 ± 88 ms, and 1040 ± 60 ms in segments without LGE (p = 0.16). The corresponding numbers for ECV were 38.7 ± 10.9% and 30.0 ± 4.7%, p &lt; 0.001. Native T1 showed poor agreement to scar independent of scar size (AUC = 0.532, 95% CI 0.485-0.578 for scars of all sizes, and AUC = 0.572, 95% CI 0.495-0.650 for transmural scars). ECV, on the other hand, showed reasonable agreement with scar of all sizes (AUC = 0.777, 95% CI 0.739-0.815), and good agreement with transmural scars (AUC = 0.856, 95% CI 0.811-0.902). (Figure) Conclusion The contrast-free CMR technique T1 mapping does not adequately detect scars in patients referred for CRT. Adding post contrast T1 measurements and calculating ECV improves accuracy, especially for transmural scars. Future studies should investigate if diffuse fibrosis could be predictive of CRT response. Abstract P1585 Figure. Detection of transmural scars


Author(s):  
Fabian aus dem Siepen ◽  
Sebastian Buss ◽  
Florian Andre ◽  
Marius Keller ◽  
Sebastian A Seitz ◽  
...  

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
R Everett ◽  
T Treibel ◽  
M Fukui ◽  
H Lee ◽  
M Rigolli ◽  
...  

Abstract Background The development of myocardial fibrosis is a key mechanism in the transition from compensated hypertrophy to heart failure in aortic stenosis (AS). Focal and diffuse fibrosis can be quantified using cardiac magnetic resonance (CMR) imaging late gadolinium-enhanced (LGE) and T1 mapping techniques. Purpose To assess T1 mapping measures of fibrosis in patients with severe AS referred for aortic valve intervention, and determine their associations with clinical characteristics, disease severity and long-term clinical outcome. Methods In this international prospective cohort study, patients with severe AS underwent contrast enhanced CMR with T1 mapping and LGE prior to aortic valve intervention. Image analysis was performed by a single core laboratory and the extracellular volume fraction [ECV%] calculated from T1 mapping images. The presence of LGE was determined visually and quantified using the full-width-at-half-maximum technique. Results Four-hundred and forty patients (70±10 years, 59% male) from ten international centres were enrolled. Aortic valve intervention was performed 15 [4 to 58] days following CMR. Within a follow-up of 3.8 [2.8 to 4.6] years, 52 patients died. ECV% (mean 27.7±3.6%) correlated with increasing age, Society of Thoracic Surgeons Predicted Risk of Mortality score, known coronary artery disease, lower peak aortic-jet velocity, larger left ventricular (LV) mass, lower LV ejection fraction, and presence of LGE (P<0.05 for all). Following adjustment for all demographic and clinical variables, ECV% remained associated with age (P=0.028), LV ejection fraction (P<0.001) and presence of LGE (P=0.035). Univariable predictors of all-cause mortality included age, male sex, impaired LV ejection fraction and presence of LGE (all P<0.05). A progressive increase in all-cause mortality was seen across tertiles of ECV% (17.3, 31.6 and 52.7 deaths per 1000 patient-years; log-rank test, P=0.009). ECV% was independently associated with all-cause mortality following adjustment for age, sex, impaired LV ejection fraction and presence of LGE (HR per unit increase in ECV: 1.10, 95%, (1.02–1.19), P=0.013). ECV440 abstract iamge Conclusion In patients with severe aortic stenosis scheduled for aortic valve intervention, extracellular volume-based T1 mapping correlates with LV decompensation. ECV% is a strong independent predictor of late all-cause mortality and is a potential therapeutic target.


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