P5274Characterization of cardiac involvement in patients with MELAS syndrome in comparison to HCM patients by conventional LGE imaging and novel T1-mapping

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Bietenbeck ◽  
A R Florian ◽  
C Meier ◽  
V Holtstiege ◽  
G Chatzantonis ◽  
...  
Keyword(s):  
T1 Mapping ◽  
Cardiac Involvement ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Specific Pattern ◽  
Lv Function ◽  
Healthy Controls ◽  
Melas Syndrome ◽  
Native T1 ◽  
Lv Mass

Abstract Background MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes) is a rare clinical subtype of mitochondrial myopathy. Cardiovascular magnetic resonance (CMR) images of MELAS-patients at an advanced state of the disease often show characteristic patterns: septal pronounced left ventricular (LV) hypertrophy and distinct focal myocardial scars in late-gadolinium-enhancement (LGE) images. This specific pattern is different from those seen in patients with hypertrophic cardiomyopathy (HCM) due to sarcomere protein mutations. Besides LGE imaging another method for the assessment of myocardial integrity, T1-mapping, has been established recently. This is the first study comparing native and enhanced T1-mapping (T1-na/en) as well as extracellular volume (ECV) values in HCM and MELAS. Methods 12 patients with confirmed MELAS syndrome at different states of the disease, 13 HCM patients and 15 controls were included. All CMR studies were performed on a 1.5T MR scanner (Ingenia, Philips) using bSSFP cine sequences for the assessment of LV-function and MOLLI sequences for T1-na/en mapping. 15min after IV injection of 0.1mmol/kg BW Gadobutrol for LGE imaging, T1-en maps were acquired. For comparison of groups T1-na/en and ECV values in a basal short axis slice were used. Results Median and interquartile ranges of LV function, T1 and ECV are shown in the table. There was no difference in LV ejection fraction (LVEF) between the groups, but end-diastolic LV-mass was increased in HCM- and MELAS-patients vs. controls (p<0.001, p=0.028). In MELAS-patients, myocardial fibrosis was detected in focal spots clearly defined in both septum and free lateral wall. In HCM patients there was a rather diffuse LGE pattern in the hypertrophic septum and the RV insertion points. Here T1-na/en and ECV significantly differed from those measured in healthy controls (p<0.001, p<0.001, p=0.002). In the MELAS group T1-na values were significantly higher compared to controls (p=0.004) and significantly lower compared to HCM patients (p=0.003). LV-function and mapping parameters of compared groups LV-EF, % LV-EDV, ml/m2 LV-mass, g/m2 T1-na, ms T1-en, ms ECV, % MELAS 57 (44–63) 86 (81–122) 80 (50–131) 969 (940–1033) 409 (381–465) 27 (24–35) HCM 60 (57–65) 50 (73–94) 94 (72–102) 1041 (1021–1074) 362 (346–430) 31 (28–36) Controls 61 (58–65) 72 (67–89) 53 (42–60) 937 (894–951) 464 (446–513) 26 (24–27) Conclusion Compared to healthy controls and HCM-patients, cardiac involvement in MELAS-patients is not only reliably visualized by conventional LGE imaging but also detected without the use of contrast agent by native T1-mapping. MELAS vs. HCM-patients show a different pattern of LGE and higher native T1 values, respectively.

scholarly journals Effect of migalastat on cardiac involvement in Fabry disease: preliminary results from MAIORA study

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A Camporeale ◽  
F Bandera ◽  
M Pieroni ◽  
F Pieruzzi ◽  
A Bersano ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Functional Capacity ◽  
Cardiac Involvement ◽  
Extracellular Volume ◽  
Left Ventricular ◽  
Real World Data ◽  
Private Company ◽  
Native T1 ◽  
Lv Mass ◽  
Treatment Naïve

Abstract Background Fabry Disease (FD) is a rare X-linked lysosomal storage disorder. Since 2016, pharmacological chaperone Migalastat has been approved for treatment of FD patients with amenable mutations to stabilize defective forms of the enzyme α-galactosidase A. A small but significant reduction in left ventricular (LV) mass after 18 months of Migalastat treatment has been previously reported by echocardiography. However, an integrated assessment of the effect of Migalastat on cardiac involvement, combining LV morphology and tissue composition by CMR with exercise capacity by cardiopulmonary test, is lacking. Purpose To determine the effects of 18 month treatment with Migalastat on LV mass, native T1 value and functional capacity in naïve patients with genetically confirmed FD cardiomyopathy. Methods Sixteen treatment naïve FD patients (4 females, mean age 46.4±16.2) with amenable mutations and signs of cardiac involvement underwent CMR with T1 mapping and cardio-pulmonary testing before and after 18 months of migalastat therapy as a part of MAIORA Study. Cardiac involvement was defined as presence of reduced native T1 values at CMR (a surrogate of myocardial glycosphingolipid storage) and/or LV hypertrophy (LVH). Nine patients (56%, 2 females, mean age 56.4±12.7 years) had LVH at baseline. Results Migalastat treatment was well tolerated in all patients, with no serious adverse event. No change in LV mass was detected at 18 months compared to baseline (95.2 (66.0–184.0) vs 103.0 (71.0–182.0) g/m2; p=0.5516). The same result was found after stratifying patients according to presence/absence of Late Gadolinium Enhancement (LGE) (LGE+ n=8, 2 females, mean age 56.2±13.1 years). There was a trend towards an increased native septal T1 value (870.0 (848–882) vs 860.0 (833.0–875.0) ms at baseline; p 0.056) with unchanged extracellular volume (ECV) (0.26 (0.23–0.028) vs 0.26 (0.22–0.29) at baseline; p 0.276) in the overall cohort. An improvement in functional capacity with a trend towards an increase in percent-predicted peak VO2 (72.0 (61.25–80.75) vs 67.0 (45.2–79.2) at baseline; p 0.056) and a significant increase in VO2 at anaerobic threshold (14.8 (12.6–20.0) vs 13.10 (6.8–18.6) ml/kg/min at baseline; p 0.004) was reported in the total population. Conclusion In treatment naïve FD patients with amenable mutations and signs of early or overt cardiac involvement, 18-month treatment with Migalastat stabilized LV mass both in patients with and without LGE and was associated with an improvement in exercise tolerance. The trend towards an increase in T1 value associated with unchanged ECV suggests partial clearance of cardiomyocyte glycoshingolipid storage. These real-world data are consistent with a beneficial impact of migalastat on the progression of cardiac involvement in FD. FUNDunding Acknowledgement Type of funding sources: Private company. Main funding source(s): Amicus Therapeutics

scholarly journals Left ventricular fibrosis and hypertrophy are associated with mortality in heart failure with preserved ejection fraction

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pankaj Garg ◽  
Hosamadin Assadi ◽  
Rachel Jones ◽  
Wei Bin Chan ◽  
Peter Metherall ◽  
...  
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
T1 Mapping ◽  
Left Ventricular ◽  
Preserved Ejection Fraction ◽  
Native T1 ◽  
Lv Mass ◽  
All Cause Mortality ◽  
Ventricular Fibrosis

AbstractCardiac magnetic resonance (CMR) is emerging as an important tool in the assessment of heart failure with preserved ejection fraction (HFpEF). This study sought to investigate the prognostic value of multiparametric CMR, including left and right heart volumetric assessment, native T1-mapping and LGE in HFpEF. In this retrospective study, we identified patients with HFpEF who have undergone CMR. CMR protocol included: cines, native T1-mapping and late gadolinium enhancement (LGE). The mean follow-up period was 3.2 ± 2.4 years. We identified 86 patients with HFpEF who had CMR. Of the 86 patients (85% hypertensive; 61% males; 14% cardiac amyloidosis), 27 (31%) patients died during the follow up period. From all the CMR metrics, LV mass (area under curve [AUC] 0.66, SE 0.07, 95% CI 0.54–0.76, p = 0.02), LGE fibrosis (AUC 0.59, SE 0.15, 95% CI 0.41–0.75, p = 0.03) and native T1-values (AUC 0.76, SE 0.09, 95% CI 0.58–0.88, p < 0.01) were the strongest predictors of all-cause mortality. The optimum thresholds for these were: LV mass > 133.24 g (hazard ratio [HR] 1.58, 95% CI 1.1–2.2, p < 0.01); LGE-fibrosis > 34.86% (HR 1.77, 95% CI 1.1–2.8, p = 0.01) and native T1 > 1056.42 ms (HR 2.36, 95% CI 0.9–6.4, p = 0.07). In multivariate cox regression, CMR score model comprising these three variables independently predicted mortality in HFpEF when compared to NTproBNP (HR 4 vs HR 1.65). In non-amyloid HFpEF cases, only native T1 > 1056.42 ms demonstrated higher mortality (AUC 0.833, p < 0.01). In patients with HFpEF, multiparametric CMR aids prognostication. Our results show that left ventricular fibrosis and hypertrophy quantified by CMR are associated with all-cause mortality in patients with HFpEF.

scholarly journals Cardiovascular magnetic resonance-determined left ventricular myocardium impairment is associated with C-reactive protein and ST2 in patients with paroxysmal atrial fibrillation

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Lei Zhao ◽  
Songnan Li ◽  
Chen Zhang ◽  
Jie Tian ◽  
Aijia Lu ◽  
...  
Keyword(s):  
Cardiovascular Disease ◽  
Healthy Subjects ◽  
T1 Mapping ◽  
Circumferential Strain ◽  
Myocardial Strain ◽  
Left Ventricular ◽  
Healthy Controls ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Native T1

Abstract Background Myocardial strain assessed with cardiovascular magnetic resonance (CMR) feature tracking can detect early left ventricular (LV) myocardial deformation quantitatively in patients with a variety of cardiovascular diseases, but this method has not yet been applied to quantify myocardial strain in patients with atrial fibrillation (AF) and no coexistent cardiovascular disease, i.e., the early stage of AF. This study sought to compare LV myocardial strain and T1 mapping indices in AF patients and healthy subjects, and to investigate the associations of a portfolio of inflammation, cardiac remodeling and fibrosis biomarkers with LV myocardial strain and T1 mapping indices in AF patients with no coexistent cardiovascular disease. Methods The study consisted of 80 patients with paroxysmal AF patients and no coexistent cardiovascular disease and 20 age- and sex-matched healthy controls. Left atrial volume (LAV), LV myocardial strain and native T1 were assessed with CMR, and compared between the AF patients and healthy subjects. Biomarkers of C-reactive protein (CRP), transforming growth factor beta-1 (TGF-β1), collagen III N-terminal propeptide (PIIINP), and soluble suppression of tumorigenicity 2 (sST2) were obtained with blood tests, and compared between the AF patients and healthy controls. Associations of these biomarkers with those CMR-measured parameters were analyzed for the AF patients. Results For the CMR-measured parameters, the AF patients showed significantly larger LAV and LV end-systolic volume, and higher native T1 than the healthy controls (max P = 0.027). The absolute values of the LV peak systolic circumferential strain and its rate as well as the LV diastolic circumferential strain rate were all significantly reduced in the AF patients (all P < 0.001). For the biomarkers, the AF patients showed significantly larger CRP (an inflammation biomarker) and sST2 (a myocardium stiffness biomarker) than the controls (max P = 0.007). In the AF patients, the five CMR-measured parameters of LAV, three LV strain indices and native T1 were all significantly associated with these two biomarkers of CRP and sST2 (max P = 0.020). Conclusions In patients with paroxysmal AF and no coexistent cardiovascular disease, LAV enlargement and LV myocardium abnormalities were detected by CMR, and these abnormalities were associated with biomarkers that reflect inflammation and myocardial stiffness.

scholarly journals Association between Native Myocardial T1 Mapping and Cardiac Function and Macroscopic Fibrosis in Thalassemia Major

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 26-27
Author(s):  
Alessia Pepe ◽  
Nicola Martini ◽  
Antonio De Luca ◽  
Vincenzo Positano ◽  
Laura Pistoia ◽  
...  
Keyword(s):  
Iron Overload ◽  
Myocardial Fibrosis ◽  
T1 Mapping ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Lv Function ◽  
Myocardial Iron ◽  
Native T1 ◽  
Molli Sequence ◽  
Lv Volume

Background.Cardiovascular magnetic resonance (CMR) is the only available technique for the non-invasive quantification of MIO. The native T1 mapping has recently been proposed as an alternative to the universally adopted T2* technique, due to the higher sensitivity for detection of changes associated with mild or early iron overload. Objective.To study the association between T1 values and left ventricular (LV) function in thalassemia major (TM) and to evaluate for the first time if T1 measurements quantifying MIO are influenced by macroscopic myocardial fibrosis. Methods.146 TM patients (87 females, 38.7±11.1 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network underwent CMR. Native T1 values were obtained by Modified Look-Locker Inversion recovery (MOLLI) sequence in all 16 myocardial segments and the global value was the mean. LV function parameters were quantified by cine images. Late gadolinium enhancement (LGE) technique was used to detect macroscopic myocardial fibrosis. Results.No correlation was detected between global heart T1 values and LV volume indexes, LV mass index, or LV ejection fraction. Foourteen (9.6%) patients had an abnormal LV motion (13 hypokinesia and 1 dyskinesia) and they showed significantly lower global heart T1 values than patients without LV motion abnormalities (883.8±139.7 ms vs 959.0±91.3 ms; P=0.049). LGE images were acquired in 88 patients (60.3%) and macroscopic myocardial fibrosis was detected in 36 patients (40.9%). The 72.2% of patients had two or more foci of fibrosis. Patients with macroscopic myocardial fibrosis had significantly lower global heart T1 values (921.3±100.3 ms vs 974.5±72.7 ms; P=0.027) (Figure 1A). Data about the LGE was present for 1408 segments (88 patients x 16 segments) and 105 (7.5%) were positive. Segments with LGE had significantly lower T1 values than segments LGE-negative (905.6±110.6 ms vs 956.9±103.8 ms; P&lt;0.0001) (Figure 1B). Conclusion.No correlation between T1 values and LV function parameters was detected, probably because the majority of the patients had normal or mild abnormal LV parameters. TM patients with macroscopic myocardial fibrosis showed significantly lower T1 values suggesting that T1 measurements for quantifying MIO are not influenced by macroscopic myocardial fibrosis and an association between myocardial iron and macroscopic fibrosis, previously detected only in pediatric TM patients. Figure Disclosures Pepe: Chiesi Farmaceutici S.p.A.:Other: no profit support and speakers' honoraria;Bayer:Other: no profit support;ApoPharma Inc.:Other: no profit support.Pistoia:Chiesi Farmaceutici S.p.A.:Other: speakers' honoraria.Meloni:Chiesi Farmaceutici S.p.A.:Other: speakers' honoraria.

Insight form cardiovascular magnetic resonance on cardiac sarcoidosis: a single centre experience

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Alderighi ◽  
A Baritussio ◽  
O Ozden Tok ◽  
M Perazzolo Marra ◽  
S Iliceto ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Cardiac Sarcoidosis ◽  
T2 Mapping ◽  
Left Ventricular ◽  
Lv Function ◽  
Post Contrast ◽  
Lv Volumes

Abstract Background Clinically manifest cardiac sarcoidosis (CS) has a prevalence of 5%, but is more frequent in autoptic series (25%). Diagnosis is multiparametric and relies on clinical criteria and imaging findings, although a certain diagnosis, especially in the case of isolated CS (ICS), can only be based on endomyocardial biopsy. Cardiovascular magnetic resonance (CMR) has a comprehensive role in the assessment of CS: left ventricular (LV) dysfunction and extent of late gadolinium enhancement (LGE)are important predictors of prognosis, T2 mapping provides information on disease activity and global longitudinal strain (GLS) analysis can uncover subclinical LV impairment. Purpose To assess the prevalence of CS by CMR in patients with biopsy-proven extracardiacsarcoidosis (ECS); to describe the imaging characteristics of patients with ECS and those with high clinical suspicionof ICS; to investigate the contribution of more recent techniques to the diagnosis of CS alongside traditional LGE assessment. Methods We retrospectively enrolled 84 patients (66% males, mean age 59±13 years) referred to our centreforsuspected CS (biopsy-proven ECS, n=61; clinical presentation suggestive of CS,, n=23). CMR was performed on a 1.5T scanner, with a protocol comprehensive of biventricular functional assessment and post-contrast images; T2-STIR images (n=30), native myocardial T1 mapping (n=24) and T2 mapping (n=19) were also performed in selected patients. Tissue tracking analysis was perfomed in all patients using a dedicated software. Results Based on CMR findings, 35 patients (42%) with ECS did not show cardiac involvement (SS), 26 (31%) showed both cardiac and systemic involvement (CS-SS) and 23 (27%) had evidence of ICS (ICS). 43% of patients had history of arrhythmias, but life-threatening tachyarrhythmiaswere more frequent in patients with CS (p=0.02).Patients with CS had significantly lower LVEF (p&lt;0,01), larger LV volumes (p&lt;0,01) and greater LV mass (p&lt;0,01). GLS values were impaired in all the groups but significantly more in patients with CS (p&lt;0,01). With regards to LGE distribution, ICS patients showed a higher number of segments involved (p=0,011) as compared to CS patients. T2-STIRimages were positive in 3 out of 30 patients; T2 mapping detected myocardial oedema in 1 patient with negative T2- STIR and was positive in 7 who did not undergo traditional oedema evaluation. T1 mapping mainly confirmed the results provided by LGE, but was altered in 1 patient who could not receive gadolinium. Conclusions CMR findings consistent with CS were found in 49 patients referred for suspected CS. Patients with cardiac involvement, particularly if isolated, had significantly lower LVEF, greater LV volumes and more impaired GLS. Patients with SS, despite a normal LV function, showed mildly impaired GLS, subtending subclinical cardiac involvement. Funding Acknowledgement Type of funding source: None

P5273Trabecular complexity as a subclinical structural alteration in Fabry cardiomyopathy: a cardiac magnetic resonance study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
D Lazzeroni ◽  
A Camporeale ◽  
F Moroni ◽  
S Garibaldi ◽  
S Pica ◽  
...  
Keyword(s):  
Fractal Dimension ◽  
Cardiac Magnetic Resonance ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Fractal Dimensions ◽  
Healthy Controls ◽  
Lv Mass ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Background Heart involvement represents the main cause of death in Fabry Disease (FD), thus its early detection is important to define the optimal therapeutic strategy. Recently, a disproportionate increase in myocardial trabeculation has been described in FD by cardiac magnetic resonance (CMR), even in early (prehypertrophic) stage of the disease. In addition, CMR with T1 mapping can identity the presence of myocardial sphingolipid storage (causing lowering of native T1 values) in more than 50% of FD patients with no LVH. However, it is not clear whether a relationship exists between trabecular complexity and sphingolipid storage in FD. Aim To explore the association between myocardial trabecular complexity, quantified by endocardial border fractal analysis, and sphingolipid storage, described by CMR T1 mapping, in different stages of Fabry cardiomyopathy. Methods Study population included 60 subjects: 15 FD patients with no detectable signs of cardiac involvement (no LVH, normal T1; 2 M, age 30.6±14; Group 1); 15 FD patients with early sphingolipid storage (no LVH, low T1; 9 M, age 33±9.6; Group 2); 15 FD patients with LVH (11 M, age 53.5±9.6; Group 3); 15 healthy controls (9 M, age 34±10). Patients and controls underwent CMR with T1 mapping; disease severity was quantified using Mainz Severity Score Index (MSSI). Myocardial trabecular fractal dimension was evaluated, blinded to patients'characteristics, on short axis cine images using the Image J dedicated plug-in FracLac, deriving the following parameters: total, basal, mid-ventricular and apical fractal dimensions. Results Total fractal dimension was higher in all Fabry groups compared to controls. Indeed, a gradient of total fractal dimension was observed, with this parameter gradually increasing from healthy controls to Groups 3 (1.27±0.02 in controls vs 1.29±0.02 in Group 1 vs 1.30±0.02 in Group 2 vs 1.34±0.02 in Group 3; p<0.001) (Figure 1A). Interestingly, both total and basal fractal dimensions were significantly higher in Group 1 compared to controls (1.27±0.02 vs 1.29±0.02, p=0.044 and 1.26±0.04 vs 1.30±0.03; p=0.007, respectively). Moreover, considering the total population, fractal dimension showed significant correlations with: i) T1 values (r=−0.567; p<0.001 - Figure 1B); ii) LV mass (r=0.674, p<0.001); iii) trabecular mass expressed as percentage of global LV mass (r=0.611; p<0.001); iv) MSSI (r=0.535; p<0.001). Conclusion Cardiac involvement in FD is characterized by a progressive increase in fractal dimension of endocardial trabeculae (Figure 1C). Both total and basal myocardial trabeculation are increased in Fabry patients even before the presence of detectable sphingolipid storage, thus representing a very early sign of cardiac involvement.

scholarly journals P0254MYOCARDIAL TISSUE CHARACTERIZATION IN LIVING KIDNEY DONORS 5 YEARS AFTER NEPHRECTOMY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Anna Price ◽  
Victoria Stoll ◽  
Ravi Vijapurapu ◽  
Kirsty McGee ◽  
Roman Wesolowski ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
T1 Mapping ◽  
Cardiac Fibrosis ◽  
Left Ventricular ◽  
Healthy Controls ◽  
Gadolinium Enhancement ◽  
Post Contrast ◽  
Kidney Donors ◽  
Living Kidney Donors ◽  
Lv Mass

Abstract Background and Aims Uraemic cardiomyopathy is characterized by left ventricular (LV) hypertrophy, diastolic dysfunction and myocardial fibrosis. Diffuse interstitial fibrosis of the myocardium, assessed using cardiac magnetic resonance imaging (CMR) techniques of native T1-mapping and extracellular volume (ECV), has been demonstrated in end stage chronic kidney disease (CKD) and in patients with stage 3 CKD and normal left ventricular mass. In living kidney donors estimated glomerular filtration rate (eGFR) declines by a third after donation, with 60% having a resultant eGFR comparable with stage 3 CKD. Recent studies have demonstrated small increases in LV mass at 12 months after donation as well as functional sequelae of reduced global circumferential strain and apical torsion. We sought to establish whether living kidney donors had CMR evidence of diffuse interstitial cardiac fibrosis which might contribute to observed functional correlates. Method A cross sectional blinded study of living kidney donors (n=50) and healthy controls (n=45) who underwent 3 Tesla CMR and blood samples for biomarkers of fibrosis. A modified look-locker inversion recovery (MOLLI) 5(3)3 sampling scheme was used for T1 mapping followed by T2 mapping sequences at the mid LV slice. Five minutes after the administration of gadolinium (Gadovist®) contrast (0.15mmol/kg), standard T1-weighted gradient echo inversion recovery images were repeated for the assessment of late gadolinium enhancement (assessment for focal fibrosis). Post contrast MOLLI images were acquired using identical slice positions as native images using a 4(1)3(1)2 sampling scheme 15 minutes after the administration of gadolinium. Native and post contrast T1 time was used to calculate ECV. Results There were no differences in demographics between groups; age (donors 54 ± 12yrs vs. healthy controls 50 ± 13yrs, p=0.128), ethnicity (89% Caucasian) or male gender (37%). LV mass and volumes were not significantly different. Forty four donors and 34 controls consented to receive gadolinium contrast. Native T1 in the septal mid LV slice was not significantly different between groups (donors 1223 ± 35ms vs. controls 1210 ± 36ms, p=0.102). There was also no difference in T2 time of the septal mid LV slice (donors 40 ± 2ms vs. controls 40 ± 4ms vs. p=0.455). Late gadolinium enhancement was seen in five living kidney donors in a right ventricular insertion point pattern but there was no mid wall or ischaemic pattern enhancement. There was no difference in septal ECV at the mid LV slice (donors 25 ± 2% vs. controls 25 ± 2%, p=0.896). There was also no corresponding difference in fibroblast growth factor-23 (RU/ml) in donors 74 [58-105] vs. controls 59 [47-75], p=0.081 or soluble α-klotho (pg/ml) in donors 610 [503-810] vs. controls 703 [550-955], p=0.061. Conclusion Septal T1 times and ECV in living kidney donors at 5 years from donation are no different from healthy controls. Biomarkers of cardiac fibrosis are also comparable to healthy controls in this small cohort. We found no CMR evidence of the ultrastructural changes reported in uraemic cardiomyopathy in the hearts of living kidney donors at 5 years from donation.

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 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&gt;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 Assessment of Cardiac Involvement in Fabry Disease (FD) with Native T1 Mapping

2019 ◽  
Vol 191 (10) ◽  
pp. 932-939
Author(s):  
Fritz Christian Roller ◽  
Sven Fuest ◽  
Marco Meyer ◽  
Sebastian Harth ◽  
Dursun Gündüz ◽  
...  
Keyword(s):  
Fabry Disease ◽  
T1 Mapping ◽  
Cardiac Involvement ◽  
Morphological Changes ◽  
Tissue Level ◽  
Therapeutic Benefit ◽  
Left Ventricular ◽  
Imaging Biomarker ◽  
Native T1 ◽  
Native T1 Time

Purpose Fabry disease (FD) is an X-linked multi-organ disorder of lysosomal metabolism with cardiac disease being the leading cause of death. Identifying early FD-specific pathologies is important in the context of maximum therapeutic benefit in these stages. Therefore, the aim of this study was to investigate the value of quantitative cardiac T1 mapping as a potential disease-specific surrogate. Methods 16 consecutive FD patients (9 female, 7 male; median age: 54 years, IQR 17) and 16 control patients (9 female, 7 male; median age: 52 years, IQR 20) were investigated at 1.5 Tesla. Native T1 mapping was performed using a modified look locker inversion recovery sequence (MOLLI) and native T1 times were measured within the septal myocardium at the midventricular short-axis section. Also functional parameters, left ventricular morphology, presence of late-gadolinium enhancement, cTnI- and Lyso-Gb3-Levels were evaluated. Results The median native septal T1 time for FD was 889.0 ms and 950.6 ms for controls (p < 0.003). LGE and positive cTnI values (0.26 ± 0.21) were present in 5 FD patients (31.25 %), and left ventricular hypertrophy (LVH) was present in 4 FD patients (25.00 %). The 4 cTnI and 8 Lyso-Gb3 positive FD patients had significantly lower native T1 values (p < 0.05, respectively p < 0.01). Assuming a T1 cut-off value of 900 ms for the identification of increased cardiac lipid deposit, 9 patients with FD (56.25 %) had pathologic values (4 patients cTnI and 8 patients Lyso-Gb3 positive). Moreover, native septal T1 showed a good negative correlation to Lyso-Gb3 (r = – 0.582; p = 0.018). Conclusion A pathologic cardiac native T1 time obviously reflects cardiac involvement in the scope of FD at tissue level. In the future native T1 mapping as an imaging biomarker might allow identification of early stages of cardiac involvement in FD before morphological changes are obvious. Key Points:  Citation Format

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