FRI0236 FIRST PILOT STUDY OF T1 MAPPING MRI WITH ECV MEASUREMENT OF PERIPHERAL MUSCLE IN SYSTEMIC SCLEROSIS PATIENTS SHOWS DIFFUSE FIBROSIS

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

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T1 and T2 Mapping in Cardiology: “Mapping the Obscure Object of Desire”

Cardiology ◽

10.1159/000478901 ◽

2017 ◽

Vol 138 (4) ◽

pp. 207-217 ◽

Cited By ~ 26

Author(s):

Sophie Mavrogeni ◽

Dimitris Apostolou ◽

Panayiotis Argyriou ◽

Stella Velitsista ◽

Lilika Papa ◽

...

Keyword(s):

Heart Failure ◽

T1 Mapping ◽

Clinical Decision Making ◽

Volume Fraction ◽

T2 Mapping ◽

Extracellular Volume ◽

Diffuse Fibrosis ◽

Post Contrast ◽

Cardiac Amyloid ◽

Native T1

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.

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P1585 Cardiac magnetic resonance estimated extracellular volume fraction, but not native T1 mapping, detects scar in patients referred for cardiac resynchronization therapy

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.1005 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

C Kjellstad Larsen ◽

J Duchenne ◽

E Galli ◽

J M Aalen ◽

E Kongsgaard ◽

...

Keyword(s):

T1 Mapping ◽

Volume Fraction ◽

Extracellular Volume ◽

Extracellular Volume Fraction ◽

Cardiac Resynchronization ◽

Diffuse Fibrosis ◽

Resynchronization Therapy ◽

Native T1 ◽

T1 Relaxation ◽

Contrast Agent Injection

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 < 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

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

Journal of Cardiovascular Magnetic Resonance ◽

10.1186/1532-429x-16-21 ◽

2014 ◽

Vol 16 (1) ◽

pp. 21 ◽

Cited By ~ 125

Author(s):

Ntobeko AB Ntusi ◽

Stefan K Piechnik ◽

Jane M Francis ◽

Vanessa M Ferreira ◽

Aitzaz BS Rai ◽

...

Keyword(s):

Systemic Sclerosis ◽

Clinical Study ◽

T1 Mapping ◽

Extracellular Volume ◽

Diffuse Fibrosis ◽

Myocardial Inflammation ◽

Myocardial T1

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REFERENCE RANGES OF NATIVE T1-MAPPING AND EXTRACELLULAR VOLUME FOR CARDIAC AMYLOIDOSIS - A META-ANALYSIS

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(21)02670-x ◽

2021 ◽

Vol 77 (18) ◽

pp. 1312

Author(s):

Tom Kai Ming Wang ◽

Maria Vega Brizneda ◽

Deborah Kwon ◽

Zoran Popovic ◽

Scott Flamm ◽

...

Keyword(s):

Cardiac Amyloidosis ◽

T1 Mapping ◽

Meta Analysis ◽

Extracellular Volume ◽

Reference Ranges ◽

Native T1

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RETRACTED ARTICLE: Prognostic value of heart failure in hemodialysis-dependent end-stage renal disease patients with myocardial fibrosis quantification by extracellular volume on cardiac magnetic resonance imaging

BMC Cardiovascular Disorders ◽

10.1186/s12872-019-01313-2 ◽

2020 ◽

Vol 20 (1) ◽

Cited By ~ 3

Author(s):

Hua-yan Xu ◽

Zhi-gang Yang ◽

Yi Zhang ◽

Wan-lin Peng ◽

Chun-chao Xia ◽

...

Keyword(s):

Heart Failure ◽

Myocardial Fibrosis ◽

Prognostic Value ◽

T1 Mapping ◽

Extracellular Volume ◽

Cardiac Events ◽

Native T1 ◽

Stage Renal Disease ◽

End Stage ◽

Esrd Patients

Abstract Background End-stage renal disease (ESRD) patients are at high cardiovascular risk, and myocardial fibrosis (MF) accounts for most of their cardiac events. The purpose of this study is to investigate the prognostic value and risk stratification of MF as measured by extracellular volume (ECV) on cardiac magnetic resonance (CMR) for heart failure (HF) in patients with hemodialysis-dependent ESRD. Methods Sixty-six hemodialysis ESRD patients and 25 matched healthy volunteers were prospectively enrolled and underwent CMR to quantify multiple parameters of MF by T1 mapping and late gadolinium enhancement (LGE). All ESRD patients were followed up for 11–30 months, and the end-point met the 2016 ESC guidelines for the definition of HF. Results Over a median follow-up of 18 months (range 11–30 months), there were 26 (39.39%) guideline-diagnosed HF patients in the entire cohort of ESRD subjects. The native T1 value was elongated, and ECV was enlarged in the HF cohort relative to the non-HF cohort and normal controls (native T1, 1360.10 ± 50.14 ms, 1319.39 ± 55.44 ms and 1276.35 ± 56.56 ms; ECV, 35.42 ± 4.42%, 31.85 ± 3.01% and 26.97 ± 1.87%; all p<0.05). In the cardiac strain analysis, ECV was significantly correlated with global radial strain (GRS) (r = − 0.501, p = 0.009), global circumferential strain (GCS) (r = 0.553, p = 0.005) and global longitudinal strain (GLS) (r = 0.507, p = 0.008) in ESRD patients with HF. Cox proportional hazard regression models revealed that ECV (hazard ratio [HR] = 1.160, 95% confidence interval: 1.022 to 1.318, p = 0.022) was the only independent predictor of HF in ESRD patients. It also had a higher diagnostic accuracy for detecting MF (area under the curve [AUC] = 0.936; 95% confidence interval: 0.864 to 0.976) than native T1 and post T1 (all p ≤ 0.002). Kaplan-Meier analysis revealed that the high-ECV group had a shorter median overall survival time than the low-ECV group (18 months vs. 20 months, log-rank p = 0.046) and that ESRD patients with high ECV were more likely to have HF. Conclusions Myocardial fibrosis quantification by ECV on CMR T1 mapping was shown to be an independent risk factor of heart failure, providing incremental prognostic value and risk stratification for cardiac events in ESRD patients. Trial registration Chinese Clinical Trial Registry ChiCTR-DND-17012976, 13/12/2017, Retrospectively registered.

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