scholarly journals Detection of intracellular histological abnormalities using cardiac magnetic resonance T1 mapping in patients with Danon disease: a case series

2021 ◽  
Vol 5 (5) ◽  
Author(s):  
Hideaki Suzuki ◽  
Yoshiaki Morita ◽  
Ryoko Saito ◽  
Shunsuke Tatebe ◽  
Tetsuya Niihori ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Extracellular Volume ◽  
Case Series ◽  
Left Ventricular ◽  
Musculoskeletal Symptoms ◽  
Danon Disease ◽  
Autophagic Vacuoles ◽  
Native T1

Abstract Background Danon disease is an X-linked dominant disorder with defects in the lysosome-associated membrane protein 2 (LAMP2) gene and is characterized histologically by intracellular autophagic vacuoles in skeletal and cardiac muscles. Cardiac magnetic resonance (CMR) T1 mapping potentially allows to differentiate intracellular and extracellular cardiac abnormalities with a combination of native T1 value and extracellular volume (ECV) fraction. Case summary We assessed CMR T1 mapping in two Danon disease patients (a 22-year-old man and his 48-year-old mother), who had a LAMP2 c.864G>A p. Val288Val mutation, and two blood relatives without Danon disease (his 47-year-old maternal aunt and 49-year-old father). The male patient underwent a left ventricular (LV) assist device implantation at 15 months after the image acquisition because he was inotrope dependent (INTERMACS profile 3) and had no noticeable psychological or musculoskeletal symptoms. His mother was in New York Heart Association Class II with mildly reduced LV ejection fraction (46%). The Danon group showed late gadolinium enhancement (LGE) in the anterior and posterolateral LV walls. In the interventricular wall, where evident LGE was not noted, the Danon group had high native T1 value, compared with the T1 value in the non-Danon group, and normal ECV fraction. Cardiac biopsy from the interventricular wall showed intracytoplasmic autophagic vacuoles, which are characteristics of Danon disease. Discussion This characteristic pattern of high native T1 and normal ECV fraction in the areas without LGE, which may reflect the existence of intracytoplasmic autophagic vacuoles, may support the differential diagnosis of Danon disease from other cardiomyopathies.

scholarly journals Multiparametric CMR Imaging of Myocardial Structure and Function Changes in Diabetic Mini-pigs With Preserved LV Function : A Preliminary Study

2021 ◽  
Author(s):  
Guozhu Shao ◽  
Yukun Cao ◽  
Yue Cui ◽  
Xiaoyu Han ◽  
Jia Liu ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Structure And Function ◽  
Lv Function ◽  
Myocardial Structure ◽  
And Function ◽  
Mini Pigs

Abstract Background: The purpose of this study is to dynamically monitor the myocardial structure and function changes in diabetic mini-pigs by 1.5T cardiac magnetic resonance. Methods: Cardiac magnetic resonance (CMR) T1 mapping was performed in three male streptozotocin-induced diabetic mini-pigs. T1-mapping and ECV-mapping were acquired at basal, mid and apical segments. CMR feature-tracking (CMR-FT) is used to quantify left ventricle global longitudinal (LVGLS), circumferential (LVGCS) and radial strain(LVGRS). Epicardial adipose tissue (EAT) was evaluated using a commercially available software.Results: Left ventricular mass (LVM), myocardial T1 value and extracellular volume (ECV) value increased gradually after 3, 4.5 and 6 months of modeling, while LVGLS decreased gradually after 3 months of modeling（Modeling 3M VS 1.5M：LVM，34.0 ± 1.9 VS 26.4 ± 1.3，P=0.027；T1，1012.3 ± 9.6 VS 1002.2 ± 11.4, P=0.014; ECV，24.3 ± 1.6 VS 22.4 ± 1.6，P=0.014；GLS：-20.8 ± 1.3 VS -23.0 ± 1.6，P=0.014；Modeling 4.5M VS 3M：LVM，37.5 ± 1.3 VS 34.0 ± 1.9，P=0.005；T1, 1017.8 ± 9.5 VS 1012.3 ± 9.6, P＜0.001；ECV，26.2 ± 1.5 VS 24.3 ± 1.6，P=0.037；GLS：-19.4 ± 1.4 VS -20.8 ± 1.3，P=0.016；Modeling 6M VS 4.5M：LVM，42.9 ± 1.6 ± 1.9 VS 37.5 ± 1.3，P=0.008；T1，1026.6 ± 10.2 VS 1017.8 ± 9.5, P=0.003；ECV，28.6 ± 1.8 VS 26.2 ± 1.5，P=0.016；GLS：-17.9 ± 1.1 VS -19.4 ± 1.4，P=0.019). EAT did not increase significantly until the sixth month (Modeling 6M VS 4.5M, EAT: 24.1 ± 3.1 VS 20.2 ± 2.4, P= 0.043).Conclusion: The progressive impairments in LV structure and myocardial deformation occurs in diabetic mini-pigs. T1 mapping and CMR-FT technology are promising to monitor abnormal changes of diabetic myocardium in early stage of diabetic cardiomyopathy.

scholarly journals Changes of Cardiac Magnetic Resonance T1 and ECV Values in Healthy Adults of Different Gender and Age

2021 ◽  
Author(s):  
Shumei Huang ◽  
Meng Zhang ◽  
Kanghui Yu ◽  
Xiaofen Ma ◽  
Chunlong Li ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Healthy Adult ◽  
Extracellular Volume ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Healthy Adults ◽  
Left Ventricular Myocardium ◽  
3T Mri

Abstract BackgroundThe correlation between T1 and ECV value and myocardial fibrosis has been confirmed. In healthy adult, many disease may change T1 and ECV value. The purpose of this reseach is to clarify the T1 mapping and extracellular volume (ECV) value of healthy adults in 3 Tesla (T) magnetic resonance imaging (MRI), and to study the relationship between the values with age and gender.MethodsWe recruited 87 healthy adult subjects (mean age 38 ± 11 years; 61% were females) for cardiac magnetic resonance examination at 3T MRI. The left ventricular function parameters were obtained from functional imaging. T1 mapping was performed using an improved Look-Locker reversal recovery sequence after motion correction. ResultsUnder 3T field strength, T1 value of the myocardium of healthy adults was 1,261 ± 52, and the ECV value was 28.3% ± 2.9%. T1 value and ECV values of female myocardium were higher than those of males (1,274 ± 56 vs. 1,241 ± 40, P = 0.003; 29.6% ± 2.6% vs. 26.2% ± 2.1%, P < 0.001). Only gender (Beta = 0.311, P = 0.003) was independently related to the native T1 mapping of left ventricular myocardium, while gender (Beta = 0.572, P < 0.001) and age (Beta = 0.501, P = 0.003) were related to the ECV value of left ventricular myocardium. T1 value (P = 0.002) and ECV value (P = 0.013) increased significantly from the base to the apex. There were significant differences in the T1 and ECV value between each segment (P < 0.001) in the base of the left ventricle. Significant differences were observed between the middle segments of T1 value (P = 0.001) but not between the middle of the ECV value of each segment (P = 0.068). Lastly, there was no statistical difference between each segment in the apical of the T1 (P = 0.756) and ECV value (P = 0.344).ConclusionsUnder 3T MRI, the T1 value of the myocardium of healthy adults was 1,261 ± 52, and the ECV value was 28.3% ± 2.9%. T1 and ECV values of females were significantly higher than those of males. The ECV values of males increased with age, while T1 value in males and T1 and ECV values in females had no significant relationship with age.

scholarly journals Changes of Cardiac Magnetic Resonance T1 and ECV Values in Healthy Adults of Different Gender and Age

2021 ◽  
Author(s):  
Shumei Huang ◽  
Meng Zhang ◽  
Kanghui Yu ◽  
Chunlong Li ◽  
Zhihong Lan ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Healthy Adult ◽  
Extracellular Volume ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Healthy Adults ◽  
Left Ventricular Myocardium ◽  
3T Mri

Abstract purposeThe correlation between T1 and ECV value and myocardial fibrosis has been confirmed. In healthy adult, many disease may change T1 and ECV value. The purpose of this reseach is to clarify the T1 mapping and extracellular volume (ECV) value of healthy adults in 3 Tesla (T) magnetic resonance imaging (MRI), and to study the relationship between the values with age and gender.Methods We recruited 87 healthy adult subjects (mean age 38 ± 11 years; 61% were females) for cardiac magnetic resonance examination at 3T MRI. The left ventricular function parameters were obtained from functional imaging. T1 mapping was performed using an improved Look-Locker reversal recovery sequence after motion correction. resultsUnder 3T field strength, T1 value of the myocardium of healthy adults was 1,261 ± 52, and the ECV value was 28.3% ± 2.9%. T1 value and ECV values of female myocardium were higher than those of males (1,274 ± 56 vs. 1,241 ± 40, P = 0.003; 29.6% ± 2.6% vs. 26.2% ± 2.1%, P < 0.001). Only gender (Beta = 0.311, P = 0.003) was independently related to the native T1 mapping of left ventricular myocardium, while gender (Beta = 0.572, P < 0.001) and age (Beta = 0.501, P = 0.003) were related to the ECV value of left ventricular myocardium. T1 value (P = 0.002) and ECV value (P = 0.013) increased significantly from the base to the apex. There were significant differences in the T1 and ECV value between each segment (P < 0.001) in the base of the left ventricle. Significant differences were observed between the middle segments of T1 value (P = 0.001) but not between the middle of the ECV value of each segment (P = 0.068). Lastly, there was no statistical difference between each segment in the apical of the T1 (P = 0.756) and ECV value (P = 0.344).ConclusionsUnder 3T MRI, the T1 value of the myocardium of healthy adults was 1,261 ± 52, and the ECV value was 28.3% ± 2.9%. T1 and ECV values of females were significantly higher than those of males. The ECV values of males increased with age, while T1 value in males and T1 and ECV values in females had no significant relationship with age.

Myocardial Fibrosis in Pediatric Patients With Ebstein's Anomaly

2021 ◽  
Author(s):  
Safwat Aly ◽  
Mike Seed ◽  
Shi-Joon Yoo ◽  
Christopher Lam ◽  
Lars Grosse-Wortmann
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Children And Adolescents ◽  
Myocardial Fibrosis ◽  
T1 Mapping ◽  
Severity Index ◽  
Left Ventricular ◽  
Ventricular Volumes ◽  
Native T1

Background: Left ventricular dysfunction in Ebstein's anomaly (EA) is associated with higher mortality. The health of the left ventricular myocardium in children and adolescents with EA has not been investigated in detail. Methods: Patients with unrepaired EA who had undergone cardiac magnetic resonance imaging including T1 mapping were retrospectively reviewed. Patients were compared with age- and sex-matched controls. EA severity index was calculated using volumetric measurements at end diastole ([right atrial+atrialized right ventricular volumes]/[functional right ventricular+left atrial+left ventricular volumes]). Global circumferential and radial strain and as well as strain rate were examined using cardiac magnetic resonance feature tracking. Results: Twelve EA patients and an equal number of controls were included. Functional and atrialized right ventricular end-diastolic volumes were 84±15 and 21±13 mL/m 2 , respectively. Late gadolinium enhancement, confined to the right ventricle, was found in 2 patients (16%). Left ventricular native T1 values and extracellular volume fractions were higher in patients compared with controls (1026±47 versus 956±40 ms, P =0.0004 and 28.5±3.4% versus 22.5±2.6%, P <0.001, respectively). Native T1 times correlated inversely with patients’ age, body surface area, and O 2 saturations (r=−0.63, −0.62, and −0.91, respectively; P =0.02, P =0.02, and P <0.0001, respectively). EA severity index ranged between 0.15 and 0.94 and correlated with T1 values (r=0.76, P =0.003). Native T1 correlated with global circumferential strain (r=0.58, P =0.04) but not ejection fraction (EF). EA patients had reduced maximum oxygen uptake (V o 2 max). V o 2 max correlated inversely with T1 values (r=−0.79, P =0.01). Conclusions: Children and adolescents with EA experience an abnormal degree of diffuse myocardial fibrosis. Its association with O 2 saturation points toward a role of hypoxemia in the pathogenesis of fibrosis. Larger and prospective studies are needed to evaluate the value of T1 mapping for risk stratification and monitoring in EA.

P598T1 mapping and myocardial extracellular volume assessed by cardiac magnetic resonance in diabetic patients with stable coronary artery disease

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G A B Boros ◽  
W Hueb ◽  
P C Rezende ◽  
F F Ribas ◽  
A R Dallazen ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Diabetic Patients ◽  
Myocardial Tissue ◽  
Syntax Score ◽  
Post Contrast ◽  
Native T1

Abstract Background T1 mapping is a quantitative technique of cardiac magnetic resonance (CMR) increasingly used for characterization of the myocardium. Type 2 diabetes mellitus (T2DM) may impact myocardial tissue structure, however studies that assessed this association using non-invasive methods have conflicting results. Purpose We sought to compare the tissue characteristics of the non-infarcted myocardium of patients with and without diabetes with multivessel CAD. Methods Patients with stable multivessel CAD and preserved left ventricular ejection fraction (LVEF), included in the MASS V trial, underwent contrast-enhanced CMR before revascularization procedures. Patients were stratified according to the T2DM diagnosis at baseline. Values of myocardial native T1, post-contrast T1 and extracellular volume fraction (ECV) were compared between diabetic and non-diabetic patients. Only myocardial tissue without late gadolinium enhancement were assessed. Results Of 155 patients studied, 67 (43%) were diabetic and 88 (57%) non-diabetic. Baseline characteristics were similiar between groups (age 70±10 vs 69±11; 69% vs 68% males; LVEF 65±13 vs 67±9). Mean Syntax score was 21.2±8.5 and 20.4±8.5 (p=0.52) in diabetic and non-diabetic, respectively. Myocardial native T1 values showed no diference in diabetic and non-diabetic (1013±67.9 vs 1015±61.4, p=0.72). However, in diabetic patients values of post-contrast T1 were significantly lower (482.2±43.8 vs 499.4±47.2, p=0.024) and ECV were higher (29.62±6.61 vs 27.08. ± 4.22, p=0.004). Multivariable analyses adjusted for age, sex, BMI, hypertension and Syntax score showed no differences in the results. Figure1 Conclusion In this study, T2DM was associated with higher ECV and lower post-contrast T1 values in the myocardial tissue. These findings suggest an increase in the myocardial intersticial matrix in patients with diabetes and stable multivessel CAD.

scholarly journals Proteoglycan remodeling is accelerated in women with angina pectoris and diffuse myocardial fibrosis: the iPOWER Study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N.D Mygind ◽  
S Holm Nielsen ◽  
M Mide Michelsen ◽  
A Pena ◽  
D Bechsgaard Frestad ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Angina Pectoris ◽  
Myocardial Fibrosis ◽  
T1 Mapping ◽  
Obstructive Coronary Artery Disease ◽  
Extracellular Volume ◽  
Diffuse Myocardial Fibrosis ◽  
Public Institution ◽  
Funding Source

Abstract Background Women with angina and no obstructive coronary artery disease (CAD) have an unfavourable prognosis, possibly due to coronary microvascular disease and diffuse myocardial fibrosis (DMF). In DMF myocardial extracellular matrix (ECM) proteins are actively remodeled by matrix metalloproteinase (MMP). Purpose We investigated MMP-mediated degradation of the protegoglycans biglycan and versican in women with angina pectoris and possible DMF assessed by cardiac magnetic resonance T1 mapping. Methods Seventy-one women with angina pectoris and no obstructive CAD were included. Asymptomatic age-matched women served as controls (n=32). Versican and biglycan were measured in serum by specific competitive enzyme-linked immunosorbent assays. T1 mapping was performed by cardiac magnetic resonance with gadolinium measuring T1 and extracellular volume (ECV). Results Both biglycan and versican levels were higher in symptomatic women compared with controls; 31.4 ng/mL vs. 16.4 ng/mL (p&lt;0.001) and 2.1 ng/mL vs. 1.8 ng/mL (p&lt;0.001), respectively (Figure 1) and were moderately correlated to global ECV (r2=0.38, p&lt;0.001 and r2=0.26, p=0.015 respectively). Conclusion Turnover of biglycan and versican was increased in symptomatic compared to asymptomatic women and associated to ECV, supporting a link between angina with no obstructive CAD and fibrotic cardiac remodeling. The examined biomarkers may prove to be suitable for monitoring active ECM remodeling. Figure 1. Levels of BGM and VCANM Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): This work was supported by The Danish Heart Foundation, the Danish Research Fund (Den Danske Forskningsfond) and by University of Copenhagen.

scholarly journals Multicenter Study on the Diagnostic Performance of Native-T1 Cardiac Magnetic Resonance of Chronic Myocardial Infarctions at 3T

2020 ◽  
Vol 13 (6) ◽  
Author(s):  
Guan Wang ◽  
Sang-Eun Lee ◽  
Qi Yang ◽  
Vignesh Sadras ◽  
Suraj Patel ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Myocardial Infarctions ◽  
Native T1 ◽  
Medical Centers ◽  
Diagnostic Capacity ◽  
Highly Correlated ◽  
Sensitivity Specificity ◽  
High Degree

Background: Preclinical studies and pilot patient studies have shown that chronic infarctions can be detected and characterized from cardiac magnetic resonance without gadolinium-based contrast agents using native-T1 maps at 3T. We aimed to investigate the diagnostic capacity of this approach for characterizing chronic myocardial infarctions (MIs) in a multi-center setting. Methods: Patients with a prior MI (n=105) were recruited at 3 different medical centers and were imaged with native-T1 mapping and late gadolinium enhancement (LGE) at 3T. Infarct location, size, and transmurality were determined from native-T1 maps and LGE. Sensitivity, specificity, receiver-operating characteristic metrics, and inter- and intraobserver variabilities were assessed relative to LGE. Results: Across all subjects, T1 of MI territory was 1621±110 ms, and remote territory was 1225±75 ms. Sensitivity, specificity, and area under curve for detecting MI location based on native-T1 mapping relative to LGE were 88%, 92%, and 0.93, respectively. Native-T1 maps were not different for measuring infarct size (native-T1 maps: 12.1±7.5%; LGE: 11.8±7.2%, P =0.82) and were in agreement with LGE ( R 2 =0.92, bias, 0.09±2.6%). Corresponding inter- and intraobserver assessments were also highly correlated (interobserver: R 2 =0.90, bias, 0.18±2.4%; and intraobserver: R 2 =0.91, bias, 0.28±2.1%). Native T1 maps were not different for measuring MI transmurality (native-T1 maps: 49.1±15.8%; LGE: 47.2±19.0%, P =0.56) and showed agreement ( R 2 =0.71; bias, 1.32±10.2%). Corresponding inter- and intraobserver assessments were also in agreement (interobserver: R 2 =0.81, bias, 0.1±9.4%; and intraobserver: R 2 =0.91, bias, 0.28±2.1%, respectively). While the overall accuracy for detecting MI with native-T1 maps at 3T was high, logistic regression analysis showed that MI location was a prominent confounder. Conclusions: Native-T1 mapping can be used to image chronic MI with high degree of accuracy, and as such, it is a viable alternative for scar imaging in patients with chronic MI who are contraindicated for LGE. Technical advancements may be needed to overcome the imaging confounders that currently limit native-T1 mapping from reaching equivalent detection levels as LGE.

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