scholarly journals Myocardial fibrosis imaging based on T1-mapping and extracellular volume fraction (ECV) measurement in muscular dystrophy patients: diagnostic value compared with conventional late gadolinium enhancement (LGE) imaging

2014 ◽  
Vol 15 (9) ◽  
pp. 1004-1012 ◽  
Author(s):  
A. Florian ◽  
A. Ludwig ◽  
S. Rosch ◽  
H. Yildiz ◽  
U. Sechtem ◽  
...  
2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Azuma ◽  
S Kato ◽  
S Kodama ◽  
K Hayakawa ◽  
M Kagimoto ◽  
...  

Abstract Background The Catheter Ablation versus Standard Conventional Therapy in Patients with Left Ventricular Dysfunction and Atrial Fibrillation (CASTLE-AF) trial has shown that the catheter ablation (CA) for atrial fibrillation (AF) significantly reduced the risk of death and hospitalization for heart failure in patients with non-ischemic dilated cardiomyopathy (NIDCM) and AF (N Engl J Med 2018; 378:417–27). In addition, the Catheter Ablation Versus Medical Rate Control in Atrial Fibrillation and Systolic Dysfunction (CAMERA-MRI) study demonstrated that the absence of myocardial fibrosis on late gadolinium enhanced (LGE) magnetic resonance imaging (MRI) is associated with improvement of left ventricular systolic function after CA in NIDCM patients with AF (J Am Coll Cardiol 2017; 70:1949–61). Extracellular volume fraction (ECV) by T1 mapping has emerges as a non-invasive mean to quantify diffuse myocardial fibrosis. Purpose The aim of this study was to compare the predictive value of LGE-MRI and ECV by T1 mapping for the prediction of improvement of LVEF after CA in NIDCM patients. Methods A total of twenty-eight patients with NIDCM and AF (age: 67±10 years; 25 (89%) male; LVEF: 34.1±8.8%) were studied. Using a 1.5T MR scanner and 32 channel cardiac coils, cine MRI, LGE-MRI, pre- and post- T1 mapping images of LV wall at mid-ventricular level (modified Look-Locker inversion recovery sequence) were acquired. Myocardial fibrosis on LGE was defined as area with >5SD signal intensity of normal myocardium. ECV from six segments of mid ventricular level were averaged for each patient. All patients underwent CA for AF, and the improvement of LVEF before and after CA were evaluated by echocardiography. Results All patients restored sinus rhythm after CA at the time of echocardiography. The mean LVEF was 34.1±8.8% before CA and 49.1±12.0% after CA (p<0.001), resulting an improvement of 15.0±11.8%. Significant correlation was found between improvements in LVEF and amount of fibrosis on LGE-MRI (r=−0.40, p=0.034), improvement of LVEF and ECV (r=−0.55, p=0.008). In the ROC analysis, ECV had a higher discriminative ability for the improvement of LVEF after CA compared with amount of fibrosis on LGE-MRI (AUC 0.885 vs 0.650) (Figure). Conclusions In NIDCM patients with AF, ECV by T1 mapping had better predictive ability for improvement of LVEF after CA in comparison to LGE-MRI. ROC curves of ECV and LGE-MRI Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Hyemoon Chung ◽  
Yoonjung Kim ◽  
Chul-Hwan Park ◽  
Jong-Youn Kim ◽  
Pil-Ki Min ◽  
...  

Abstract Background Myocardial fibrosis is an important prognostic factor in hypertrophic cardiomyopathy (HCM). However, the contribution from a wide spectrum of genetic mutations has not been well defined. We sought to investigate effect of sarcomere and mitochondria-related mutations on myocardial fibrosis in HCM. Methods In 133 HCM patients, comprehensive genetic analysis was performed in 82 nuclear DNA (33 sarcomere-associated genes, 5 phenocopy genes, and 44 nuclear genes linked to mitochondrial cardiomyopathy) and 37 mitochondrial DNA. In all patients, cardiovascular magnetic resonance (CMR) was performed, including 16-segmental thickness, late gadolinium enhancement (LGE), native and post-T1, extracellular volume fraction (ECV), and T2, along with echo-Doppler evaluations. Results Patients with sarcomere mutation (SM, n = 41) had higher LGE involved segment, % LGE mass, ECV and lower post-T1 compared to patients without SM (n = 92, all p < 0.05). When classified into, non-mutation (n = 67), only mitochondria-related mutation (MM, n = 24), only-SM (n = 36) and both SM and MM (n = 5) groups, only-SM group had higher ECV and LGE than the non-mutation group (all p < 0.05). In non-LGE-involved segments, ECV was significantly higher in patients with SM. Within non-SM group, patients with any sarcomere variants of uncertain significance had higher echocardiographic Doppler E/e’ (p < 0.05) and tendency of higher LGE amount and ECV (p > 0.05). However, MM group did not have significantly higher ECV or LGE amount than non-mutation group. Conclusions SMs are significantly related to increase in myocardial fibrosis. Although, some HCM patients had pathogenic MMs, it was not associated with an increase in myocardial fibrosis.


2020 ◽  
Vol 57 (5) ◽  
pp. 958-964
Author(s):  
Julia Schleihauf ◽  
Julie Cleuziou ◽  
Christian Meierhofer ◽  
Karin Klingel ◽  
Moritz Jesinghaus ◽  
...  

Abstract OBJECTIVES The aim of this study was to assess the effect of surgical septal myectomy performed during early childhood for severe, drug-refractory hypertrophic cardiomyopathy with left ventricular outflow tract obstruction on the extent of septal myocardial extracellular volume fraction and the potential risk of developing atrioventricular cardiac conduction system disease. METHODS In this retrospective study, data from 30 patients with a confirmed diagnosis of childhood-onset hypertrophic cardiomyopathy were reviewed including cardiovascular magnetic resonance (CMR) with myocardial T1 mapping and late gadolinium enhancement, histopathology of myocardial specimens, transthoracic echocardiography, electrocardiography, 24-h Holter and cardiopulmonary exercise testing. Eighteen patients without were compared to 12 patients with prior septal myectomy performed during childhood (non-operated versus myectomy patients). RESULTS Late gadolinium enhancement on CMR as a correlate for focal myocardial fibrosis was found in 53% of patients, predominantly located in the septal region, with no difference between groups. As compared to non-operated patients, those after myectomy showed a similar amount of total and septal extracellular volume fraction, as calculated from pre- and post-contrast CMR T1 mapping, which is a correlate for diffuse interstitial myocardial fibrosis. PQ-intervals or the occurrence of higher degree conduction system disease were equal between the 2 groups. CONCLUSIONS Data from CMR and electrocardiography suggest that surgical septal myectomy performed during early childhood for severe obstructive hypertrophic cardiomyopathy does not cause an increased septal extracellular volume fraction or delayed atrioventricular conduction time on long-term follow-up.


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.


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