Myocardial T1 Values Research Articles

Exploring cardiovascular involvement in IgG4-related disease: a case series approach with cardiovascular magnetic resonance

BackgroundIgG4-related disease (IgG4-RD) is a relapsing–remitting, fibroinflammatory, multisystem disorder. Cardiovascular involvement from IgG4-RD has not been systematically characterised. In this study, we sought to evaluate consecutive patients with IgG4-RD using...

Early Detection of Myocardial Involvement in Thalassemia Intermedia Patients: Multiparametric Mapping by Magnetic Resonance Imaging.

No study has assessed myocardial T1 and T2 values in patients with beta-thalassemia intermedia (β-TI). To assess the prevalence of myocardial involvement in β-TI patients by T2* relaxometry and native T1 and T2 mapping and to determine the correlation of myocardial relaxation times with demographic and clinical parameters. Prospective matched-cohort study. 42 β-TI patients (27 females, 39.65 ± 12.32 years), enrolled in the Extension-Myocardial Iron Overload in Thalassaemia Network, and 42 age- and sex-matched healthy volunteers (27 females, 40.01 ± 11.36 years) and thalassemia major (TM) patients (27 females, 39.27 ± 11.57 years). 1.5 T/multi-echo gradient echo, modified Look-Locker inversion recovery, multi-echo fast-spin-echo, cine balanced steady-state-free precession, and late gadolinium enhancement (LGE) sequences. Hepatic, pancreatic, and left ventricular (LV) T2* values, LV native T1 and T2 values, biventricular ejection fractions and volumes, and presence and extent of replacement myocardial fibrosis. Comparisons between two groups were performed with two-sample t tests, Wilcoxon's signed rank tests, or χ2 testing. Correlation analysis was performed using Pearson's or Spearman's test. P < 0.05 was considered statistically significant. β-TI patients had significantly higher LV T2 values than healthy subjects (56.84 ± 4.03 vs. 52.46 ± 2.50 msec, P < 0.0001) and significantly higher LV T1 values than TM patients (1018.32 ± 48.94 vs. 966.66 ± 66.47 msec, P < 0.0001). In β-TI, female gender was associated with significantly increased LV T1 (P = 0.041) and T2 values (P < 0.0001), while splenectomy and presence of regular transfusions were associated with significantly lower LV T1 values (P = 0.014 and P = 0.001, respectively). In β-TI patients, all LV relaxation times were significantly correlated with each other (T2*-T1: P = 0.003; T2*-T2: P = 0.003; T1-T2: P < 0.0001). Two patients with a reduced LV T2* also had a reduced LV T1, while only one had a reduced LV T2. Three patients had a reduced LV T1 but a normal LV T2*; 66.7% of the patients had an increased LV T2. All LV relaxation times were significantly correlated with pancreas T2* values (T2*: P = 0.033; T1: P < 0.0001; T2: P = 0.014). No LV relaxation time was associated (P > 0.05) with hepatic iron concentration, biventricular function parameters, or LGE presence. The combined use of all three myocardial relaxation times has potential to improve sensitivity in the detection of early/subclinical myocardial involvement in β-Tl patients. 2 TECHNICAL EFFICACY: Stage 2.

Accelerated Cartesian cardiac T2 mapping based on a calibrationless locally low-rank tensor constraint.

Cardiac T2 mapping is a valuable tool for diagnosing myocardial edema, inflammation, and infiltration, yet its spatial resolution is limited by the single-shot balanced steady-state free precession acquisition and duration of the cardiac quiescent period, which may reduce sensitivity in detecting focal lesions in the myocardium. To improve spatial resolution without extending the acquisition window, this study examined a novel accelerated Cartesian cardiac T2 mapping technique. We introduce a novel improved-resolution cardiac T2 mapping approach leveraging a calibrationless space-contrast-coil locally low-rank tensor (SCC-LLRT)-constrained reconstruction algorithm in conjunction with Cartesian undersampling trajectory. The method was validated with phantom imaging and in vivo imaging that involved 13 healthy participants and 20 patients. The SCC-LLRT algorithm was compared with a conventional locally low-rank (LLR)-constrained algorithm and a nonlinear inversion (NLINV) reconstruction algorithm. The improved-resolution T2 mapping (1.4 mm × 1.4 mm) was compared globally and regionally with the regular-resolution T2 mapping (2.3 mm × 1.9 mm) according to the 16-segment model of the American Heart Association. The agreement between the improved-resolution and regular-resolution T2 mappings was evaluated by linear regression and Bland-Altman analyses. Image quality was scored by two experienced reviewers on a five-point scale (1, worst; 5, best). In healthy participants, SCC-LLRT significantly reduced artifacts (4.50±0.39) compared with LLR (2.31±0.60; P<0.001) and NLINV (3.65±0.56; P<0.01), suppressed noise (4.12±0.35) compared with NLINV (2.65±0.50; P<0.001), and improved the overall image quality (4.38±0.40) compared with LLR (2.54±0.41; P<0.001) and NLINV (3.04±0.50; P<0.001). Compared with the regular-resolution T2 mapping, the proposed method significantly improved the sharpness of myocardial boundaries (4.46±0.60 vs. 3.04±0.50; P<0.001) and the conspicuity of papillary muscles and fine structures (4.46±0.63 vs. 2.65±0.30; P<0.001). Myocardial T2 values obtained with the proposed method correlated significantly with those from regular-resolution T2 mapping in both healthy participants (r=0.79; P<0.01) and patients (r=0.94; P<0.001). The proposed SCC-LLRT-constrained reconstruction algorithm in conjunction with Cartesian undersampling pattern achieved improved-resolution cardiac T2 mapping of comparable accuracy, precision, and scan-rescan reproducibility compared with the regular-resolution T2 mapping. The higher resolution improved the sharpness of myocardial borders and the conspicuity of image fine details, which may increase diagnostic confidence in cardiac T2 mapping for detecting small lesions.

Evaluation of Right Ventricular Myocardial Properties Using Systolic Myocardial T1 Mapping.

Introduction Myocardial properties can be quantitatively evaluated using myocardial native T1 values (nT1) obtained using cardiac magnetic resonance imaging (CMR). In terms of myocardial wall thickness, the left ventricular nT1 is easy to measure, but the right ventricular nT1 is difficult. Patients with congenital heart disease often develop right ventricular overload. If right ventricular nT1 can be measured consistently, inflammation and fibrosis of the right ventricular myocardium can be quantitatively evaluated. We aimed to determine whether T1 mapping during systole can be used to evaluate right ventricular myocardial properties. Methods T1 mapping was performed at diastole and systole. Systolic T1 mapping was calculated from diastolic T1 mapping and cine images. The myocardial properties of both ventricles were evaluated in 13 healthy volunteers (21-26 years old) and 12 patients with right ventricular overload (12-41 years old) who underwent CMR examination at our hospital. Results From the analysis of left ventricular nT1, we found that nT1 did not change significantly during the cardiac cycle. However, right ventricular nT1 changed between diastole and systole because the right ventricle is affected by blood. Although there was no difference in right ventricular diastolic nT1 between the patients and volunteers (1,346.8 vs. 1,347.6 ms, p = 0.852), the right ventricular systolic nT1 was significantly higher in patients than in volunteers (1,312.7 vs. 1,233.8 ms, p = 0.002). This indicates that right ventricular myocardial damage occurs in patients with right ventricular overload. Conclusion Systolic right ventricular myocardial T1 mapping allows assessment of right ventricular myocardial properties. The right ventricular myocardial systolic nT1 is useful for evaluating myocardial damage due to right ventricular stress and myocardial injury. Measuring right ventricular nT1 may allow consideration of early therapeutic intervention.

Assessing Gene Therapy Efficacy in Infantile-Onset Pompe Disease: Myocardial Native T1 Values of CMR

We report the novel finding of a widespread reduction in myocardial native T1 values and size of abnormal regions following gene therapy in a pediatric patient with infantile-onset Pompe disease. We emphasize the importance of serial cardiac magnetic resonance in assessing the efficacy of gene therapy and monitoring myocardial alterations in Pompe disease.

Myocardial Fibrosis in Young and Veteran Athletes: Evidence from a Systematic Review of the Current Literature.

Background: Exercise is associated with several cardiac adaptations that can enhance one's cardiac output and allow one to sustain a higher level of oxygen demand for prolonged periods. However, adverse cardiac remodelling, such as myocardial fibrosis, has been identified in athletes engaging in long-term endurance exercise. Cardiac magnetic resonance (CMR) imaging is considered the noninvasive gold standard for its detection and quantification. This review seeks to highlight factors that contribute to the development of myocardial fibrosis in athletes and provide insights into the assessment and interpretation of myocardial fibrosis in athletes. Methods: A literature search was performed using the PubMed/Medline database and Google Scholar for publications that assessed myocardial fibrosis in athletes using CMR. Results: A total of 21 studies involving 1642 endurance athletes were included in the analysis, and myocardial fibrosis was found in 378 of 1595 athletes. A higher prevalence was seen in athletes with cardiac remodelling compared to control subjects (23.7 vs. 3.3%, p < 0.001). Similarly, we found that young endurance athletes had a significantly higher prevalence than veteran athletes (27.7 vs. 19.9%, p < 0.001), while male and female athletes were similar (19.7 vs. 16.4%, p = 0.207). Major myocardial fibrosis (nonischaemic and ischaemic patterns) was predominately observed in veteran athletes, particularly in males and infrequently in young athletes. The right ventricular insertion point was the most common fibrosis location, occurring in the majority of female (96%) and young athletes (84%). Myocardial native T1 values were significantly lower in athletes at 1.5 T (p < 0.001) and 3 T (p = 0.004), although they had similar extracellular volume values to those of control groups. Conclusions: The development of myocardial fibrosis in athletes appears to be a multifactorial process, with genetics, hormones, the exercise dose, and an adverse cardiovascular risk profile playing key roles. Major myocardial fibrosis is not a benign finding and warrants a comprehensive evaluation and follow-up regarding potential cardiac disease.

Cardiac MRI T2 mapping with heart phase acquisition and improved motion-sensitized driven equilibrium blood suppression

Abstract Background Cardiovascular magnetic resonance (CMR) imaging relies on T2 mapping to assess myocardial tissue properties. However, T2 maps are subject to motion artifacts and signal interference, prompting exploration of techniques such as gradient-spin-echo (GraSE) sequences, heart phase acquisition, and dark blood (DB) imaging to improve image quality and reliability. Purpose This prospective study evaluates the T2 relaxation time (T2) variability and the image quality of T2 maps obtained in end-systole and end-diastole with and without fat saturation (FS), using GraSE sequence in CMR imaging. Furthermore, improved motion-sensitized driven-equilibrium (iMSDE) was compared with double inversion recovery (DIR) as an alternative DB technique. Materials and Methods Five variants of the DB GraSE sequence were developed and performed on a 1.5 Tesla MRI scanner. Forty-four healthy volunteers prospectively underwent the following sequences: GraSE in end-diastole (GraSE-ED), GraSE-ED with FS (GraSE-ED-FS), GraSE in end-systole (GraSE-ES), GraSE-ES with FS (GraSE-ES-FS), and commercial GraSE-ED (GraSE-CO). Four GraSE variants utilized iMSDE technique, while GraSE-CO used DIR for blood suppression. T2, image quality, and visual artifacts were measured. Results Ten volunteers were excluded due to image artifacts or missing datasets. Among the remaining 34 participants, the mean global T2 was measured. No significant differences were seen among all variants (P &amp;gt; .05 for all comparisons). Intra- and inter-reader agreement of global T2 values for all GraSE sequence were very good (r &amp;gt; 0.8 for both). Image quality was rated moderate or good for all variants of GraSE sequences. A lower incidence of artifacts was observed in end-systolic compared to end-diastolic imaging. Conclusion All variants of GraSE sequence are highly reproducible and myocardial T2 values did not significantly differ with heart phase. iMSDE is feasible as an alternative DB technique for T2 mapping enabling acquisition in systole, which shows a lower incidence of artifacts compared to diastole.

The Role of T2 Mapping in Cardiac Amyloidosis.

Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy divided into two types: light-chain (LA) and transthyretin (ATTR) CA. Cardiac magnetic resonance (CMR) has emerged as an important diagnostic tool in CA. While late gadolinium enhancement (LGE), T1 mapping and extracellular volume (ECV) have a consolidate role in the assessment of CA, T2 mapping has been less often evaluated. We aimed to test the value of T2 mapping in the evaluation of CA. This study recruited 70 patients with CA (51 ATTR, 19 AL). All the subjects underwent 1.5 T CMR with T1 and T2 mapping and cine and LGE imaging. Their QALE scores were evaluated. The myocardial T2 values were significantly (p < 0.001) increased in both types of CA compared to the controls. In the AL-CA group, increased T2 values were associated with a higher QALE score. The myocardial native T1 values and ECV were significantly (p < 0.001) higher in the CA patients than in the healthy subjects. Left ventricular (LV) mass, QALE score and ECV were higher in ATTR amyloidosis compared with AL amyloidosis, while the LV ejection fraction was lower (p < 0.001). These results support the concept of the presence of myocardial edema in CA. Therefore, a CMR evaluation including not only myocardial T1 imaging but also myocardial T2 imaging allows for more comprehensive tissue characterization in CA.

Native myocardial T1 mapping using inversion recovery T1-weighted turbo field echo sequence.

This study proposes the use of the inversion recovery T1-weighted turbo field echo (IR-T1TFE) sequence for myocardial T1 mapping and compares the results obtained with those of the modified Look-Locker inversion recovery (MOLLI) method for accuracy, precision, and reproducibility. A phantom containing seven vials with different T1 values was imaged, thereby comparing the T1 measurements between the inversion recovery spin-echo (IR-SE) technique, MOLLI, and the IR-T1TFE. The accuracy, precision, and reproducibility of the T1-mapping sequences were analyzed in a phantom study. Fifteen healthy subjects were recruited for the in vivo comparison of native myocardial T1 mapping using MOLLI and IR-T1TFE sequences. After myocardium segmentation, the T1 value of the entire myocardium was calculated. In the phantom study, excellent accuracy was achieved using IR-T1TFE for all T1 ranges. MOLLI displayed lower accuracy than IR-T1TFE (p =0.016), substantially underestimating T1 at large T1 values (> 1000ms). In the in vivo study, the first mean myocardial T1 values ± SD using MOLLI and IR-T1TFE were 1306 ± 70ms and 1484 ± 28ms, respectively, and the second were 1297 ± 68ms and 1474 ± 43ms, respectively. The native myocardial T1 obtained with MOLLI was lower than that of IR-T1TFE (p < 0.001). The reproducibility of native myocardial T1 mapping within the same sequence was not statistically significant (p = 0.11). This study demonstrates the utility and validity of myocardial T1 mapping using IR-T1TFE, which is a common sequence. This method was found to have high accuracy and reproducibility.

Features of cardiovascular magnetic resonance native T1 mapping in maintenance hemodialysis patients and their related factors

Purpose Increased myocardial T1 values on cardiovascular MRI (CMRI) have been shown to be a surrogate marker for myocardial fibrosis. The use of CMRI in patients on hemodialysis (HD) remains limited. This research aimed to explore the characteristics of native T1 values in HD patients and identify factors related to T1 values. Methods A total of thirty-two patients on HD and fourteen healthy controls were included in this study. All participants underwent CMRI. Using modified Look-Locker inversion recovery (MOLLI) sequence, native T1 mapping was achieved. Native CMRI T1 values were compared between the two groups. In order to analyze the relationship between T1 values and clinical parameters, correlation analysis was performed in patients on HD. Results Patients on HD exhibited elevated global native T1 values compared to control subjects. In the HD group, the global native T1 value correlated positively with intact parathyroid hormone (iPTH) (r = 0.418, p = 0.017) and negatively with triglycerides (r= −0.366, p = 0.039). Moreover, the global native T1 value exhibited a positive correlation with the left ventricular end-diastolic volume indexed to body surface area (BSA; r = 0.528, p = 0.014), left ventricular end-systolic volume indexed to BSA (r = 0.506, p = 0.019), and left ventricular mass indexed to BSA (r = 0.600, p = 0.005). A negative correlation was observed between the global native T1 value and ejection fraction (r = 0.-0.551, p = 0.010). Conclusion The global native T1 value was prolonged in HD patients compared with controls. In the HD group, the global T1 value correlated strongly with iPTH, triglycerides, and cardiac structural and functional parameters.

Effect of Pulse Wave Synchronization on T1 Value in Cardiac T1 Mapping: Is Pulse Wave Synchronization a Substitute for Electrocardiogram Gating?

We investigated whether peripheral pulse synchronization (PPUS) can be an alternate method for electrocardiographic synchronization (ECGS) in measuring myocardial T1 values in cardiac magnetic resonance imaging (CMRI). T1 map imaging was performed on 49 patients undergoing CMRI using the 5s (3s) 3s modified Look-Locker inversion recovery (MOLLI) method for both ECGS and PPUS. The short-axis images of basal, mid, and apical segments were obtained. The T1 map images were analyzed using an image processing system, and T1 values were obtained for each cardiac segment. To assess the degree of agreement between T1 values obtained from ECGS and PPUS, the Bland-Altman analysis and the estimating intraclass correlation coefficient (ICC) were performed for the average T1 value of the entire myocardium and T1 values of each cardiac segment. Also, to evaluate whether PPUS imaging is possible in the diastole phase, we measured the length of systole in the electrocardiogram and the length of transmission (R-R') from R in the electrocardiogram to R (R') in the pulse waveform. From the comparison of T1 values, a good agreement of ICC was confirmed between the ECGS and PPUS (whole myocardium: 0.97, apical: 0.93, mid: 0.98, and basal: 0.97). The results of the Bland-Altman analysis also indicated good agreement. Moreover, it was shown that the heart was imaged in the diastole phase even with the default scan parameters of PPUS. Our results indicated that PPUS can be an alternate method for ECGS.

THE DIAGNOSTIC VALUE OF CARDIAC MRI IN DETERMINING HYPERTROPHIC CARDIOMYOPATHY PHENOTYPES Using T1 and T2 mapping and late gadolinium enhancement to accurately diagnose hypertrophic cardiomyopathy

Objective: Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by thickening of the heart muscle and may have serious consequences. Diversity in the distribution and degree of hypertrophy has led to the classification of HCM types. Cardiac MR (CMR) imaging is a non-invasive tool that maintains accurate and detailed data about cardiac functions and structure. The aim of the study was to observe and compare the CMR characteristics of the three HCM morphologic types (asymmetric, concentric, and apical). Materials and Methods: 56 patients (female/male: 29/58, mean age 46.31±15.12, body surface area (BSA) 1.88±0.19 m2) who were referred for HCM diagnosis or follow-up between January 2020- January 2023 were included in this single-center retrospective study. The imaging procedure was conducted using a 1.5 T device (Aera, Siemens Medical Systems, Erlangen, Germany). Cardiac volume and functions were determined quantitatively. The location and amount of late gadolinium enhancement (LGE) was evaluated qualitatively. The statistical analyses were performed using SPSS version 25.0. A P value under 0.05 was considered statistically significant. Results: LGE revealed myocardial fibrosis in apical HCM (ApHCM), a higher prevalence than in asymmetric and concentric HCM. ApHCM and asymmetric HCM patients widely presented midmyocardial fibrosis, where most concentric HCM patients had patchy involvement. The mean native myocardial T1 values for individuals with concentric HCM was found to be higher compared to those with asymmetric and ApHCM and the healthy participants. Conclusion: CMR imaging enables the multiparametric evaluation of HCM through the utilization of native T1 and T2 mapping techniques, as well as LGE. Both T1 and T2 values have the potential to be used in conjunction with LGE to identify regions of myocardial fibrosis.

Diffusing capacity of lungs for carbon monoxide associated with subclinical myocardial impairment in systemic sclerosis: A cardiac MR study

BackgroundSystemic sclerosis (SSc) is characterised by microvascular and fibrotic lesions, which are located not only in skin but also in lungs and heart.ObjectiveThis study aimed to investigate the association between...

Prognostic significance of T2 mapping in evaluating myocardium alterations in patients with ST segment elevation myocardial infarction

To investigate the value of T2 mapping in the assessment of myocardial changes and prognosis in patients with acute ST segment elevation myocardial infarction (STEMI). A retrospective study was conducted. A total of 30 patients with acute STEMI admitted to Tianjin First Central Hospital from January 2021 to March 2022 were enrolled as the experimental group. At the same time, 30 age- and sex-matched healthy volunteers and outpatients with non-specific chest pain with no abnormalities in cardiac magnetic resonance (CMR) examination were selected as the control group. CMR was performed within 2 weeks after the diagnosis of STEMI, as the initial reference. A plain CMR review was performed 6 months later (chronic myocardial infarction, CMI). Plain scanning includes film sequence (CINE), T2 weighted short tau inversion recovery (T2-STIR), native-T1 mapping, and T2 mapping. Enhanced scanning includes first-pass perfusion, late gadolinium enhancement (LGE), and post-contrast T1 mapping. Quantitative myocardial parameters were compared between the two groups, before and after STEMI myocardial infarction. The receiver operator characteristic curve (ROC curve) was used to evaluate the diagnostic efficacy of native-T1 before myocardial contrast enhancement and T2 values in differentiating STEMI and CMI after 6 months. There were no statistically significant differences in age, gender, heart rate and body mass index (BMI) between the two groups, which were comparable. The native-T1 value, T2 value and extracellular volume (ECV) were significantly higher than those in the control group [native-T1 value (ms): 1 434.5±165.3 vs. 1 237.0±102.5, T2 value (ms): 48.3±15.6 vs. 21.8±13.1, ECV: (39.6±13.8)% vs. (22.8±5.0)%, all P < 0.05]. In the experimental group, 12 patients were re-examined by plain CMR scan 6 months later. After 6 months, the high signal intensity on T2-STIR was still visible, but the range was smaller than that in the acute phase, and the native-T1 and T2 values were significantly lower than those in the acute phase [native-T1 value (ms): 1 271.0±26.9 vs. 1 434.5±165.3, T2 value (ms): 34.2±11.2 vs. 48.3±15.6, both P < 0.05]. ROC curve analysis showed that the area under the ROC curve (AUC) of native-T1 and T2 values in differentiating acute STEMI from CMI was 0.71 and 0.80, respectively. When native-T1 cut-off value was 1 316.0 ms, the specificity was 100% and the sensitivity was 53.3%; when T2 cut-off value was 46.7 ms, the specificity was 100% and the sensitivity was 73.8%. The T2 mapping is a non-invasive method for the diagnosis of myocardial changes in patients with acute STEMI myocardial infarction, and can be used to to evaluate the clinical prognosis of patients.

Investigating the cardiac manifestations of IgG4-related disease using cardiac magnetic resonance imaging

Abstract Introduction IgG4-related disease (IgG4-RD) is a relapsing-remitting immune-mediated condition that affects multiple organ systems. Case reports of suspected cardiovascular involvement in IgG4-RD have emerged though no study has systematically assessed the cardiovascular phenotype of IgG4-RD using cardiac magnetic resonance (CMR) imaging. Purpose We systematically assessed patients with IgG4-RD using CMR to identify the cardiovascular manifestations, and compared them to controls. Methods We recruited 11 patients with histologically-confirmed IgG4-RD (6 female, 61±11 years, 9 with active disease (8 with pancreatic involvement, 3 parotid, 5 bile ducts, 5 kidneys and 3 cardiovascular)). Patients underwent CMR at 1.5T including cine, myocardial tagging, native T1-mapping, late gadolinium enhancement (LGE), extracellular volume (ECV) and quantitative stress perfusion mapping. Results were compared to 10 healthy controls with no cardiac disease (50% female, 35±8 years). Results are presented as mean ± standard deviation (SD) unless otherwise stated. Results Patients with IgG4-RD had similar cardiac geometry to the reference group (Table 1), with similar biventricular and bi-atrial volumes. However, despite similar biventricular ejection fractions compared to controls (patient LVEF: 55-67%, RVEF: 51-65%), IgG4-RD patients showed significantly reduced global longitudinal strain (GLS) (-16.8 ± 2.3% vs -18.7 ± 1.6%, p=0.045). Furthermore, 64% (7/11) of IgG4-RD patients showed LGE, 6 of whom showed non-ischaemic patterns (Figure 1). Male IgG4-RD patients (n=5) as a group showed a significantly higher average myocardial T1 value relative to the reference group, with 3/5 male patients having an abnormally high myocardial T1 (above the 2SD limit of normal). Female IgG4-RD patients (n=6) had similar and normal myocardial T1 values to the reference group. ECV did not significantly differ between the two groups. IgG4-RD patients showed a significantly reduced global myocardial perfusion reserve (MPR), including two patients with a qualitative inducible perfusion defect. Only 3 out of the 11 IgG4-RD patients had a completely normal CMR. Conclusion Patients with IgG4-RD show frequent cardiac abnormalities as revealed by advanced CMR phenotyping. These include subclinical systolic dysfunction, ischaemic and non-ischaemic myocardial fibrosis, reduced myocardial perfusion reserve, and elevated myocardial T1 times. These abnormalities have been described in inflammatory cardiovascular diseases, supporting a plausible pathophysiological link with IgG4-RD. Future work in larger and multicentre cohorts is warranted, to systematically define the novel cardiovascular phenotype of IgG4-RD.Table 1Figure 1

Abstract 18714: Myocardial Infarction With Non-Obstructive Coronary Artery Disease in a Young Woman - The Cardinal Role of Coronary Reactivity Testing in Unveiling the Underlying Etiology

43-year old female presented to the emergency department with substernal chest pain. She had similar symptoms years ago without obstructive CAD on coronary angiogram and was diagnosed with myocarditis. Presenting ECG revealed new anterolateral T wave inversions (Fig 1), and high-sensitivity troponins peaked at 322 ng/L consistent with NSTEMI. TTE was significant for global hypokinesis of the left ventricle with a reduced ejection fraction of 35 to 40%. Invasive coronary angiogram showed no obstructive CAD or spontaneous coronary artery dissection however there was reduced flow in the mid to distal LAD raising suspicion for endothelial and microvascular dysfunction. Contrast-enhanced cardiac MRI showed myocardial edema in the anterior and anteroseptal walls with elevated myocardial T1 and T2 values, and early enhancement post gadolinium administration showed an acute LAD territorial infarct (Fig 2). Findings were consistent with myocardial infarction with non-obstructive coronary artery disease (MINOCA). She was started on carvedilol for empiric treatment of coronary artery spasm and microvascular dysfunction, along with aspirin and atorvastatin. Losartan was added for heart failure management. Outpatient invasive coronary reactivity testing showed severe epicardial (70% vasoconstriction) and microvascular (80% decrease in coronary blood flow) endothelial dysfunction in response to acetylcholine (Fig 3 and Fig 4) as underlying etiology of recent MINOCA. CFR was normal. L-arginine (NO precursor) was added to the medical regimen. Patient has not had recurrence of chest pain or ACS at 1 year follow-up and LVEF normalized on repeat echocardiogram. This case shows the importance of cardiac MRI in confirming the diagnosis of MINOCA in patients with no obstructive CAD, and the crucial role of coronary reactivity testing in diagnosing and treating severe coronary endothelial dysfunction, which is aligned with the AHA/ACC MINOCA consensus document.

Abstract 15484: Heart Transplantation in a Light-Chain Amyloidosis Patient at Mayo Stage IIIa After Anti-Plasma Cell Chemotherapy With Complete Remission

A 42-year-old female patient was admitted for lower limb edema. Laboratory tests showed nephrotic proteinuria (2.76g/24h). Serum-free λ light chains were 84.9 mg/L, with a κ/λ ratio of 0.12. Bone marrow examination detected 6% immature clonal plasma cells. Amyloid deposition was identified in a renal biopsy. Cardiac magnetic resonance (CMR) revealed normal left ventricular ejection fraction (LVEF), left ventricular circumferential late gadolinium enhancement (LGE), and elevated myocardial T1 value (1459ms) and extracellular volume (47.4%). She was diagnosed with AL amyloidosis and classed as IIIa by the Mayo Stage 2004. A hematologic complete response (CR) was achieved and sustained after treatment with sequencing cyclophosphamide, bortezomib, dexamethasone (CyBorD) and daratumumab regimen. The follow-up CMRs were underwent at 6, 12, and 18 months after starting chemotherapy, which monitored cardiac changes in reponse to CR. Unfortunately, there was no alleviation of cardiac involvement. CMR re-examination (at 42 months) revealed severe cardiac function impairment (LVEF 28%), and dramatically increased extracellular volume (66.2%). Then, she underwent heart transplantation (HTx) evaluated by the heart transplantation multidisciplinary team. Three months later, she was doing well, CMR demonstrated stable cardiac allograft function. Repeated endomyocardial biopsies showed no amyloid deposits. This case illustrates that cardiac amyloid deposition might be a dynamic process in AL amyloidosis and highlights the value of CMR in long-term monitoring and identifying the patients reached CR who need HTx.

The Role of Improved Motion-Sensitized Driven Equilibrium Blood Suppression and Fat Saturation on T2 Relaxation Time, Using GraSE Sequence in Cardiac Magnetic Resonance Imaging.

T2 mapping is a valuable technique in cardiac MR imaging that offers insights into the microstructural characteristics of myocardial tissue. However, it was shown that myocardial T2 relaxation times (T2) measured vary significantly depending on sequence, sequence parameters, and field strength. To assess T2 variability and image quality in cardiac T2 maps using four variants of the gradient-spin echo (GraSE) sequence, having different methods of blood signal suppression (double inversion recovery (DIR) and improved motion-sensitized driven equilibrium (iMSDE) and with and without the addition of fat saturation (FS). Prospective. 48 healthy volunteers (46.7 +/- 21.5 years, 24 male) with no cardiac history. GraSE sequence with DIR (GraSEDIR), with iMSDE (GraSEiMSDE) and FS (GraSEDIR-FS) and with both iMSDE and FS (GraSEiMSDE-FS) at 1.5T. Global T2 from three short axis myocardial slices. and image quality assessments using a 5-point Lickert scale (1, (non-diagnostic) to 5, (excellent)) were conducted to evaluate the impact of DB and FS techniques on myocardial T2 measurements and image quality. Paired t-tests or non-parametric equivalents for comparisons between sequences. The Bland-Altmann plots and Pearson rank correlation analyses, as appropriate. A P value <0.05 was considered statistically significant. The mean global T2 values for GraSEDIR, GraSEDIR-FS, GraSEiMSDE, and GraSEiMSDE-FS, were 52.84 ± 5.72 msec, 54.98 ± 3.59 msec, 53.9 ± 4.05 msec, and 55.14 ± 4.28 msec, respectively, with no significant differences (P = 0.092). High image quality scores (>4 out of 5) were obtained for all sequence variants with no significant differences between them (P = 0.11). All GraSE sequence variants exhibited approximately the same results and variations in the DB technique and addition of FS did not have significant impact on myocardial T2 values. 2 TECHNICAL EFFICACY: Stage 1.

Normal Ventricular and Regional Blood Flow Volumes and Native T1 Values in Healthy Japanese Children Obtained from Comprehensive Cardiovascular Magnetic Resonance Imaging.

Age-related mean and reference ranges for ventricular volumes and mass, regional blood flow measurements, and T1 values using cardiovascular magnetic resonance (CMR) imaging are yet to be established for the pediatric population. Especially in infants and toddlers, no consistent flow volume sets or T1 values have been reported. The purpose of this study was to determine the relevant normal values.Twenty-three children (aged 0.1-15.3 years) without cardiovascular diseases were included. Comprehensive CMR imaging including cine, 2-dimensional phase-contrast, and native T1 mapping, were performed. Ventricular volumes and masses, 11 sets of regional blood flow volumes, and myocardial and liver T1 values were measured. All intraclass correlation coefficient values were > 0.94, except for the right ventricular mass (0.744), myocardial (0.868) and liver T1 values (0.895), reflecting good to excellent agreement between rates.Regression analysis showed an exponential relationship between body surface area (BSA) and ventricular volumes, mass, and regional blood flow volumes (normal value = a*BSAb). Left ventricular myocardial T1 values were regressed on linear regression with age (normal value = -7.39*age + 1091), and hepatic T1 values were regressed on a quadratic function of age (normal value = 0.923*age2 -18.012*age + 613).Comparison of the 2 different methods for the same physical quantities by Bland-Altman plot showed no difference except that the right ventricular stroke volume was 1.5 mL larger than the main pulmonary trunk flow volume.This study provides the normal values for comprehensive CMR imaging in Japanese children.

MRI detects increased aortic stiffening and myocardial dysfunction after TEVAR of blunt injury in young patients.

Background: Thoracic endovascular aortic repair (TEVAR) is a well-established technique for the management of blunt thoracic aortic injury (BTAI). Despite improvements in vascular imaging, graft material properties, and implant techniques, stent-graft deployment artificially induces aortic stiffening. This study aimed to evaluate the midterm effect of thoracic endovascular aortic repair after blunt thoracic aortic injury on aortic stiffness and cardiac function in young patients using cardiovascular magnetic resonance (CMR) imaging. Patients and methods: From all patients who underwent TEVAR for BTAI between 2009 and 2019 in a single institution, 10 patients with no other comorbidities affecting arterial stiffness were sex-, age-, height-, and body surface area-matched to 10 healthy controls. Comprehensive CMR examination was performed in all controls and patients. The mean follow-up period was 5.4±1.8 years; the mean age at the time of TEVAR was 30.3±8.7 years. Results: Four patients who underwent TEVAR developed arterial hypertension. 4D flow CMR-based analysis demonstrated higher global pulse wave velocity (PWV) in TEVAR patients than in controls (p=0.012). Segmental analysis showed a higher PWV in the descending and abdominal aorta. The indexed diameter of the ascending aorta was larger in TEVAR patients than in controls (p=0.007). The CINE acquisitions demonstrated increased left ventricular myocardial thickness (p<0.001). The 3D global diastolic strain rate and diastolic longitudinal velocity (e') decreased, and the A-wave velocity increased. Native myocardial T1 values were significantly higher in TEVAR patients (p=0.037). Conclusions: Young patients with TEVAR after BTAI are at an increased risk of developing vascular and myocardial dysfunction due to increased aortic stiffness. CMR follow-up allows for a comprehensive and radiation-free evaluation of vascular stiffness and associated myocardial changes, especially at the early and subclinical stages.