Magnetic Resonance T1 Mapping Research Articles

Evaluation of articular cartilage degeneration in patients with osteonecrosis of the femoral head using T2 mapping magnetic resonance imaging.

To evaluate and characterise articular cartilage degeneration in patients with osteonecrosis of the femoral head (ONFH) using T2 mapping magnetic resonance imaging (MRI). We reviewed 35 patients with ONFH (20 males and 15 females, mean age 45.7±12.9 years) without obvious cartilage abnormalities on plain MRI (ONFH group) and 25 healthy volunteers (9 males and 16 females, mean age 42.9±5.8 years) (control group). All patients underwent T2 mapping MRI after ONFH onset. The region of interest was defined as the weight-bearing portion of the articular cartilage in the femoral head and acetabulum in the coronal view. The T2 values of the articular cartilage of the acetabulum and femoral head, including necrotic and normal regions, were significantly higher in the ONFH group than those in the control group. These T2 values of the acetabulum and femoral head in stages 3A and 2 were significantly higher in the ONFH group than those in the control group. The articular cartilage of the acetabulum and femoral head can deteriorate after the onset of ONFH, which may affect the natural history of ONFH and ONFH treatment. Our findings suggest the need for early intervention in joint preservation surgery.

Genetic dilated cardiomyopathy with inflammation in an infant that responded to immunosuppressive therapy evaluated using cardiovascular magnetic resonance.

Cardiovascular magnetic resonance T1 and T2 mapping reflects inflammation, fibrosis, and myocardial oedema. However, its application in infants remains uncertain. Herein, we report a three-month-old boy with dilated cardiomyopathy successfully treated with steroids. Cardiovascular magnetic resonance was useful for diagnosis based on the elevated native T1, T2, and extracellular volume and evaluation of response to immunosuppressive therapy in infantile inflammatory dilated cardiomyopathy.

Myocardial extracellular volume derived from contrast-enhanced chest computed tomography in longitudinal evaluation of cardiac toxicity in patients treated with immune checkpoint inhibitors.

The immune-related adverse effects after immune checkpoint inhibitors (ICIs) treatment have always been a hot topic. Although the incidence of myocarditis is not high among the related adverse effects, the mortality rate is extremely high once it occurs. In the past, the risk of cancer therapy-related cardiac dysfunction (CTRCD) after drug treatment was evaluated based on imaging examinations, but this evaluation still had certain limitations. Currently, the extracellular volume (ECV) score measurement calculated using cardiac magnetic resonance T1 mapping has become a reliable method for evaluating myocardial toxicity and computed tomography (CT) examination may become an alternative. This study aimed to longitudinally evaluate the cardiac toxicity of patients treated with ICIs using myocardial ECV derived from contrast-enhanced chest CT. A total of 500 patients with III-IV lung cancer and esophageal cancer treated with ICIs were evaluated. Participants underwent baseline examination and at least 1 follow-up examination after treatment. Contrast-enhanced chest CT-ECV, left ventricular ejection fraction (LVEF), and measurement of cardiac troponin T (cTnT) were conducted before the first treatment, 3-6 months after the first treatment, and about 12 months after the first treatment, respectively. The ECV value of the middle part of the left ventricular septum was evaluated on CT venography and plain scan, the LVEF value was evaluated by color Doppler ultrasound, and the quantity of cTnT was detected by chemiluminescence. Cancer therapy-related cardiac dysfunction was recorded. The mean baseline LVEF value was 68.51%±4.81% (N0=500), and those of LVEF1, LVEF2, and LVEF3 were 68.77%±4.30%, 68.16%±3.59%, and 66.23%±4.20%, respectively (N1=500, N2=467, and N3=361, respectively). There was no significant difference between LVEF1, LVEF2, and LVEF0 (P1=0.095, P2=0.062), whereas LVEF3 was significantly lower than LVEF0 (P<0.001). The average baseline cTnT0 value was 7.42±3.95 (N0=500). The values of cTnT1, cTnT2, and cTnT3 were 10.05±11.40, 12.24±13.59, and 14.54±14.49, respectively (N1=500, N2=467, N3=361). The values of cTnT1, cTnT2, and cTnT3 were significantly higher than cTnT0 (P1<0.001, P2<0.001, P3<0.001). The average ECV0 was 47.14%±7.48% (N0=500). ECV1, ECV2, and ECV3 were 50.85%±6.79%, 53.44%±6.96% and 52.64%±7.58% respectively (N1=500, N2=467 and N3=361). ECV1, ECV2, and ECV3 were significantly higher than ECV0 (P1<0.001, P2<0.001, P3<0.001). CTRCD occurred in 49 patients. There were significant differences between the CTRCD (+) group and the CTRCD (-) group in cTnT1, cTnT2, and cTnT3 (P1<0.001, P2<0.001, and P3<0.001, respectively) and in ECV1, ECV2, and ECV3 (P1=0.039, P2=0.041, and P3=0.013, respectively). CT-ECV began to increase at the early stage after the treatment of ICIs. CT-ECV is a potential biomarker for dynamically monitoring the cardiac toxicity of tumor patients after receiving ICIs. ECV may be used to speculate the CTRCD caused by the treatment of ICIs.

Inter-scanner comparability of Z-scores for native myocardial T1 and T2 mapping.

Cardiovascular Magnetic Resonance (CMR) native T1 and T2 mapping serve as robust, contrast-agent-free diagnostic tools, but hardware- and software-specific sources of variability limit the generalizability of data across CMR platforms, consequently limiting the interpretability of patient-specific parametric data. Z-scores are used to describe the relationship of observed values to the mean results as obtained in a sufficiently large normal sample. They have been successfully used to describe the severity of quantifiable abnormalities in medicine, specifically in children and adolescents. The objective of this study was to observe whether z-scores can improve the comparability of T1 and T2 mapping values across CMR scanners, field strengths, and sequences from different vendors in the same participant rather than different participants (as seen in previous studies). Fifty-one healthy volunteers (26 men/25 women, mean age = 43±13.51) underwent three CMR exams on three different scanners, using a Modified Look-Locker Inversion Recovery (MOLLI) 5-(3)-3 sequence to quantify myocardial T1. For T2 mapping, a True Fast Imaging with steady-state free precession (TRUFI) sequence was used on a 3T Skyra™ (Siemens), and a T2 Fast Spin Echo (FSE) sequence was used on 1.5T Artist™ (GE) and 3.0T Premier™ (GE) scanners. The averages of basal and mid-ventricular short axis slices were used to derive means and standard deviations of global mapping values. We used intra-class comparisons (ICC), repeated measures ANOVA, and paired Student's t-tests for statistical analyses. There was a significant improvement in intra-subject comparability of T1 (ICC of 0.11 (95% CI= -0.018, -0.332) vs 0.78 (95% CI= 0.650, 0.866)) and T2 (ICC of 0.35 (95% CI= -0.053, 0.652) vs 0.83 (95% CI= 0.726, 0.898)) when using z-scores across all three scanners. While the absolute global T1 and T2 values showed a statistically significant difference between scanners (p<0.001), no such differences were identified using z-scores (T1z: p=0.771; T2z: p=0.985). Furthermore, when images were not corrected for motion, T1 z-scores showed significant inter-scanner variability (p<0.001), resolved by motion correction. Employing z-scores for reporting myocardial T1 and T2 removes the variation of quantitative mapping results across different MRI systems and field strengths, improving the clinical utility of myocardial tissue characterization in patients with suspected myocardial disease.

Kiosk 8R-TB-04 - Cardiac Magnetic Resonance T1 Mapping and Strain in Patients with Crohn’s Disease and Endoscopic Assessment of Inflammation

Kiosk 8R-TB-04 - Cardiac Magnetic Resonance T1 Mapping and Strain in Patients with Crohn’s Disease and Endoscopic Assessment of Inflammation

Potential for intramyocardial fibrosis detection based on estimation of extracellular volume using dual-energy computed tomography

An increase in left ventricular (LV) extracellular volume (ECV) is an important parameter of LV morphology and is considered synonymous with myocardial fibrosis, as well as a reliable marker of myocardial injury and impaired cardiac function. Accurate methods for detecting and assessing myocardial fibrosis are very important for clinical practice. The current standard for myocardial fibrosis imaging is delayed gadolinium enhanced cardiac magnetic resonance imaging (MRI) or T1 mapping, but these techniques have limitations. They can be avoided by using dual-energy computed tomography (DECT), which makes it possible to identify myocardial fibrosis, including small-focal fibrosis, in two different ways (subtraction technique and iodine density measurement technique). The literature analysis carried out by the authors showed good comparability of MRI and DECT results in determining ECV in patients with various heart diseases of both ischemic and non-ischemic nature, including cardiomyopathies, aortic stenosis, pulmonary hypertension, sarcoidosis, and amyloidosis. In addition, the use of DECT to identify myocardial fibrosis is also possible if cardiac inflammation is suspected. In addition to evaluating the effectiveness of DECT compared with MRI, different scanning protocols were analyzed, since there is currently no consensus on the optimal contrast administration regimen. The issue of radiation exposure in modern DECT scanners is also separately considered. The authors showed that DECT is an important tool for determining ECV, which is of interest for clinical practice.

Association of Lipoprotein(a) Levels With Myocardial Fibrosis in the Multi-Ethnic Study of Atherosclerosis.

Lipoprotein(a) (Lp[a]) has been identified as an emerging risk factor for adverse cardiovascular (CV) outcomes, including heart failure. However, the connections among Lp(a), myocardial fibrosis (interstitial and replacement), and cardiac remodeling as pathways to CV diseases remains unclear. This study investigated the relationship between Lp(a) levels and myocardial fibrosis by cardiac magnetic resonance (CMR) T1 mapping and late gadolinium enhancement, as well as cardiac remodeling by cine CMR, in the MESA (Multi-Ethnic Study of Atherosclerosis) cohort. The study included 2,040 participants with baseline Lp(a) measurements and T1 mapping for interstitial myocardial fibrosis (IMF) evaluation in 2010. Lp(a) was analyzed as a continuous variable (per log unit) and using clinical cutoff values of 30 and 50mg/dL. Multivariate linear and logistic regression were used to assess the associations of Lp(a) with CMR measures of extracellular volume (ECV fraction [ECV%]), native T1 time, and myocardial scar, as well as parameters of cardiac remodeling, in 2,826 participants. Higher Lp(a) levels were associated with increased ECV% (per log-unit Lp[a]; β=0.2%; P=0.007) and native T1 time (per log-unit Lp[a]; β=4%; P< 0.001). Similar relationships were observed between elevated Lp(a) levels and a higher risk of clinically significant IMF defined by prognostic thresholds per log-unit Lp(a) of ECV% (OR: 1.20; 95%CI: 1.04-1.43) and native T1 (OR: 1.2; 95%CI: 1.1-1.4) equal to 30% and 955ms, respectively. Clinically used Lp(a) cutoffs (30 and 50mg/dL) were associated with greater prevalence of myocardial scar (OR: 1.85; 95%CI: 1.1-3.2 and OR: 1.9; 95%CI: 1.1-3.4, respectively). Finally, higher Lp(a) levels were associated with left atrial enlargement and dysfunction. Elevated Lp(a) levels are linked to greater subclinical IMF, increased myocardial scar prevalence, and left atrial remodeling.

Role of cardiac magnetic resonance T1 mapping in comparison to T2* for cardiac iron overload assessment in transfusion-dependent thalassemia major patients

BackgroundCardiac complications are the leading cause of death in transfusion-dependent thalassemia major (TDT) patients due to increased cardiac iron overload (CIO), so this study’s purpose is to assess the diagnostic utility of cardiac magnetic resonance T1 mapping in correlation to T2* for the assessment of CIO in TDT patients. This prospective case–control study involved 55 TDT patients and 30 age- and gender-matched healthy controls. All the patients underwent assessment of myocardial T2*, and native T1 values by conventional cardiac magnetic resonance imaging, and native T1 mapping technique to assess the CIO. Comparison and association between T2*, and native T1 values among TDT patients were studied.ResultsA statistically significant difference is noted between control and patient groups in regard to mean T2* and native T1 values (P = 0.03 and 0.01, respectively). The mean native T1 value in TDT patients was lower than in the control group (992 ± 54 vs. 1234 ± 42), respectively, with a statistically significant P value of 0.04. The study evoked a statistically significant strong positive association, and correlation was noted between measured T2* and native T1 values (r = 0.84, P value = 0.001), while a moderate negative association was observed between native T1 values and S. ferritin levels (r = − 0.53, P value = 0.02).ConclusionsNative T1 mapping is a non-invasive promising complementary technique to T2* that helps to detect cardiac iron overload in TDT patients, as it is strongly associated with T2*. Also, native T1 value can differentiate mild, moderate, severe, and no cardiac iron overload.

Abstract 19006: Human Epididymis Protein 4 and Carcinoembryonic Antigen-125: Promising Blood Biomarkers to Identify Diffuse Myocardial Fibrosis in Patients With Severe Aortic Stenosis

Introduction: Cardiovascular magnetic resonance (CMR) T1 mapping, extracellular volume (ECV) and late gadolinium enhancement (LGE) demonstrate strong agreement with endomyocardial biopsy in evaluating myocardial fibrosis. Human epididymis protein 4(HE4) has been shown as a blood biomarker to identify fibrosis, but the relationship with other blood biomarkers is not well established yet. Hypothesis: determine the correlation between different blood biomarkers and myocardial fibrosis by CMR in patients undergoing transcatheter aortic valve implantation(TAVI). Methods: Forty-three patients were included. The cohort was divided into two groups based on the threshold values of HE4(128.5 pmol/l) and/or CA-125 (19.5 U/ml), before TAVI. Native T1 mapping and ECV were measured at 1280 ms and 26%, respectively, corresponding to the median of the population studied. LGE was qualitatively measured. Results: Patients with higher HE4 plasma levels (&gt; 128.25 pmol/l) and/or CA-125 (&gt;19.5 U/ml) prior to TAVI had more comorbidities and had higher median native T1 mapping values, 1278 ms (1239.25-1280.25) vs 1352 ms (1303.50 -1376.00), p &lt; 0.001, and LGE positive, 10 (55.6%) vs 10 (90.9%); p= 0.046, respectively. HE4 (r=0.34, p=0.03) and CA-125 (r=0.371, p=0.034) correlated with prolonged T1 mapping. No significant correlation was found with other biomarkers: troponin T (r = 0.14, p = 0.41), Nt-proBNP (r=0.28, p=0.089), IL-6 (r=0.189, p= 0.250), procalcitonin, (r =0.132, p =0.420). The addition of elevated plasma levels of HE4 and CA-125, provided significant C-statistics for myocardial fibrosis, AUC [95%CI]: 0.74 [0.58- 0.89], p= 0.008, regarding HE4 AUC [95%CI]: 0.69 [0.52- 0.85], p=0.039, and CA-125 AUC [95%CI]: 0.72 [0.57- 0.88], p= 0.014. On univariable analysis, high plasma levels of HE4 and/or Ca-125 were independently associated with diffuse fibrosis by native T1 mapping OR:9.37, [95% CI: 2.07 - 42.33], p = 0.004 and there was a trend toward focal fibrosis by LGE + OR: 8.00, [95% CI: 0.83 - 76.36], p = 0.071. Conclusion: Elevated HE4 and/or Ca-125 plasma levels correlated with prolonged native T1 mapping. The combination of HE4 values ≥ 128 pmol/l and CA-125 ≥ 19.5 U/ml may provide a useful screening biomarker to identify diffuse myocardial fibrosis in AS.

Value of Magnetic Resonance T1 Mapping in Evaluating the Early Response to Treatment for Rheumatoid Arthritis.

Rheumatoid Arthritis Magnetic Resonance Imaging Score (RAMRIS) is usually used for the semi-quantitative evaluation of joint changes in Rheumatoid Arthritis (RA). However, this method cannot evaluate early changes in bone marrow edema (BME). To determine whether T1 mapping of wrist BME predicts early treatment response in RA. This study prospectively enrolled 48 RA patients administered oral anti-rheumatic drugs. MRI of the most severely affected wrist was performed before and after 4 (48 patients) and 8 weeks of treatment (38 patients). Mean T1 values of BME in the lunate, triangular, and capitate bones; RAMRIS for each wrist; Erythrocyte-Sedimentation Rate (ESR); and 28-joint Disease Activity Score (DAS28)-ESR score were analyzed. Patients were divided into responders (4 weeks, 30 patients; 8 weeks, 32 patients) and non-responders (4 weeks, 18 patients; 8 weeks, 6 patients), according to EULAR response criteria. Receiver operating characteristic (ROC) curves were used to evaluate the efficacy of T1 values. ESR and DAS28-ESR were not correlated with T1 value and RAMRIS at each examination (P > 0.05). Changes in T1 value and DAS28-ESR relative to the baseline were moderately positively correlated with each other at 4 and 8 weeks (r = 0.555 and 0.527, respectively; P < 0.05). At 4 weeks, the change and rate of change in T1 value significantly differed between responders and non-responders (-85.63 vs. -19.92 ms; -12.89% vs. -2.81%; P < 0.05). The optimal threshold of the rate of change in T1 value at 4 weeks for predicting treatment response was -5.32% (area under the ROC curve, 0.833; sensitivity, 0.900; specificity, 0.667). T1 mapping provides a new imaging method for monitoring RA lesions; changes in wrist BME T1 values reflect early treatment response.

Multi-parameter cardiac magnetic resonance imaging detects anthracycline-induced cardiotoxicity in rabbits model

BackgroundCardiac magnetic resonance (CMR) quantitative T1 and T2 mapping offers a non-invasive means to evaluate early cardiotoxicity changes. This study aimed to pinpoint the earliest CMR indicators of myocardial injury in Anthracycline-induced cardiotoxicity (AIC) and to elucidate the connections between these CMR indicators and associated pathological indicators. MethodsA total of 34 rabbits were administered doxorubicin at a dosage of 1 mg/kg/weekly. The study incorporated six 3T CMR scan time points: baseline, and at intervals of four, six, eight, twelve, and sixteen weeks. Cine, T1 and T2 mapping sequences assessed the left ventricular ejection fraction (LVEF), native T1, extracellular volume fraction (ECV), and T2 values. Following each time point, three rabbits were sacrificed for histological analysis involving Hematoxylin and eosin (H&E), Masson, TUNEL, and microvascular density (MVD) stains. Spearman correlations and linear mixed model analysis served in the statistical analysis. ResultsDiverse degrees of alternation were recorded in LVEF, native T1, T2, and ECV over time. LVEF declined to 49.0 ± 2.6 % at 12 weeks from the baseline of 53.4 ± 3.2 %, p < 0.001. Native T1 values increase from the baseline (1396.5 ± 79.2 ms) until 8 weeks (1498.8 ± 95.4 ms, p < 0.001). T2 values increased from the baseline (36.6 ± 3.3 ms) within 4 weeks of initiation (37.5 ± 3.4, p = 0.02) and remained elevated through 16 weeks (42.8 ± 0.3, p < 0.01). ECV was elevated at 8 weeks (33.9 ± 3.8 %, p = 0.005) compared to the baseline (30.2 ± 2.5 %). By week 12, myocardial edema and increased CVF were apparent (p = 0.04 and = 0.001, respectively). The area under ROC curve for positive CMR presence and the gold standards were 0.87 (T2-ROC, 4 weeks) and 0.92 (LVEF&BNP-ROC, 12 weeks). ConclusionT1 and T2 mapping are effective tools for cardiotoxicity detection and monitoring. The prolongation of T2 value emerged as the most consistent and early-onset indicator.

Early patello-femoral condropathy assessment through quantitative analyses via T2 mapping magnetic resonance after anterior cruciate ligament reconstruction

BackgroundPatellar femoral chondropathy (FPC) is a common problem in patients undergoing anterior cruciate ligament reconstruction (ACL-R) surgery, which, if left untreated, predisposes to arthrosis. Magnetic resonance imaging (MRI) is the non-invasive gold standard for morphological evaluation of cartilage, while in recent years advanced MRI techniques (such as T2 mapping) have been developed to detect early cartilage biochemical changes. This study evaluates the different onset of early PFC between B-TP-B and HT through T2 mapping. Secondly, it aims to assess the presence of any concordance between self-reported questionnaires and qualitative MRI.Materials and methods19 patients enrolled were divided into two groups based on the type of intervention: B-PT-B and HT. After a median time of 54 months from surgery, patients were subjected to conventional MRI, T2 mapping, and clinical-functional evaluation through three self-reported questionnaires: Knee Injury and Osteoarthritis index (KOOS); Tegner Lysholm Knee Scoring Scale; International Knee Documentation Committee (IKDC).ResultsThere is not statistically significant difference in the comparison between the two MRI techniques and the two reconstructive techniques. KOOS and Tegner Lysholm scales showed significant agreement with MRI results on the grading of chondropathy.ConclusionsThere are no differences between B-TP-B and HT techniques in the early development of PFC detectable through non-invasive methods. Due to the large reduction in the frequency of physical activity following ACL-R and the finding of mild PFC (grade I and II) in a substantial proportion of patients, after a relatively short period from ACL-R, all patients should undergo conservative treatment.

Evaluation of finger cartilage composition in recreational climbers with 7Tesla T2 mapping magnetic resonance imaging.

Sport climbing may lead to tissue adaptation including finger cartilage before apparent surface damage is detectable. The main aim was to assess finger cartilage composition with T2 mapping in young, active climbers and to compare the results to a non-climbers' collective. A secondary aim was to compare whether differences in cartilage T2 times are observed between older vs. younger volunteers. 7 Tesla MRI of the fingers Dig.2-4 was performed using a multi-echo spin echo sequence. Manual segmentation of 3 ROIs at the metacarpal heads, 1 ROI at the base phalanx and 1 ROI at the proximal interphalangeal joint was performed. Included were 13 volunteers without history of trauma who are regularly performing climbing activities as a recreational sport (>20 h/month). These were age-matched with 10 control subjects not performing climbing activities. Mean age was 32.4 years for the climbing group and 25.8 years for the controls. Mean T2 values for the 5 different ROIs were 42.2 ± 7.8 msec for climbers and 41.4 ± 6.8 msec for non-climbers. No significant differences were observed for T2 values between both groups. However, higher age had a significant impact on T2 values for all assessed ROIs (higher age 44.2 ± 9.5, younger age 32.9 ± 5.7, p = 0.001). This study evaluated the cartilage composition of young, engaged climbers with a T2 mapping MRI technique with the purpose to depict early onset joint changes. No negative impact on cartilage composition due to the sport activity was found, whereas age-related effects on the cartilage seemed to be more prominent.

T2 mapping magnetic resonance imaging of cartilage in hemophilia

BackgroundIn hemophilia, recurrent hemarthrosis may lead to irreversible arthropathy. T2 mapping MRI may reflect cartilage changes at an earlier reversible stage of arthropathy as opposed to structural MRI. ObjectivesTo evaluate interval changes of T2 mapping compared with the International Prophylaxis Study Group (IPSG) structural MRI scores of ankle cartilage in boys with hemophilia receiving prophylaxis. MethodsEight boys with hemophilia A (median age, 13; range, 9-17 years), 7 age- and sex-matched healthy boys (controls, median age, 15; range, 7-16 years). A multiecho spin-echo T2-weighted MRI sequence at 3.0T was used to obtain T2 maps of cartilage of boys with hemophilia and controls. Structural joint status was evaluated using the IPSG MRI score. ResultsT2 relaxation times of ankle cartilage increased significantly over time in both persons with hemophilia and controls (P = .002 and P = .00009, respectively). Changes in T2 relaxation time strongly correlated with changes in IPSG cartilage scores (rs = 0.93 to rs = 0.78 [P = .0007 to P = .023]), but not with changes in age (P = .304 to P = .840). Responsiveness of T2 relaxation times were higher than that of IPSG cartilage scores, with standardized response means >1.4 for T2 mapping in all regions-of-interest compared with 0.84 for IPSG cartilage scores. Baseline T2 relaxation time strongly correlated with timepoint 2 IPSG cartilage score (rs = 0.93 to rs = 0.82 [P = .001 to P = .012]) and T2 relaxation time (rs = 0.98 to rs = 0.88 [P = .00003 to P = .004]) changes in most regions-of-interest. ConclusionT2 mapping shows sensitivity to biochemical changes in cartilage prior to detectable damage using conventional MRI, offering potential for early detection of bleed-related cartilage damage in boys with hemophilia.

Anderson-Fabry disease cardiomyopathy: an update on epidemiology, diagnostic approach, management and monitoring strategies.

Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by deficient activity of the enzyme alpha-galactosidase. While AFD is recognized as a progressive multi-system disorder, infiltrative cardiomyopathy causing a number of cardiovascular manifestations is recognized as an important complication of this disease. AFD affects both men and women, although the clinical presentation typically varies by sex, with men presenting at a younger age with more neurologic and renal phenotype and women developing a later onset variant with more cardiovascular manifestations. AFD is an important cause of increased myocardial wall thickness, and advances in imaging, in particular cardiac magnetic resonance imaging and T1 mapping techniques, have improved the ability to identify this disease non-invasively. Diagnosis is confirmed by the presence of low alpha-galactosidase activity and identification of a mutation in the GLA gene. Enzyme replacement therapy remains the mainstay of disease modifying therapy, with two formulations currently approved. In addition, newer treatments such as oral chaperone therapy are now available for select patients, with a number of other investigational therapies in development. The availability of these therapies has significantly improved outcomes for AFD patients. Improved survival and the availability of multiple agents has presented new clinical dilemmas regarding disease monitoring and surveillance using clinical, imaging and laboratory biomarkers, in addition to improved approaches to managing cardiovascular risk factors and AFD complications. This review will provide an update on clinical recognition and diagnostic approaches including differentiation from other causes of increased ventricular wall thickness, in addition to modern strategies for management and follow-up.

Endogenous Sex Hormone Levels and Myocardial Fibrosis in Men and Postmenopausal Women

BackgroundSex hormone (SH) imbalances have been linked to a higher risk of heart failure in both sexes. However, mechanisms that underlie this relationship remain unclear. We examined the association of baseline SH with interstitial and replacement myocardial fibrosis in the MESA (Multi-Ethnic Study of Atherosclerosis) using cardiac magnetic resonance (CMR) T1 mapping and late gadolinium enhancement (LGE). ObjectivesThe purpose of this study was to assess the link between baseline sex hormone levels and myocardial fibrosis in the MESA cohort using CMR. MethodsA total of 2,324 participants (men and postmenopausal women [PMW]) were included in the MESA with SH measured at baseline and had underwent CMR 10 years later. All analyses were stratified by sex and age. Regression models were constructed to assess the associations of baseline SH with extracellular volume (ECV)% and native T1 time and with LGE. Higher native T1 time and ECV% are interpreted as evidence of increasing interstitial myocardial fibrosis (IMF). Given the limited number of myocardial scars present in PMW, analysis of LGE was limited to men. ResultsAmong older men (age ≥65 years), a 1-SD increment higher free testosterone was significantly associated with 2.45% lower ECV% and 21.5% lower native T1 time, while a 1-SD increment higher bioavailable testosterone was associated with 12.5% lower native T1 time. A 1-SD increment greater sex hormone-binding globulin level was associated with 1% higher ECV%. Among PMW of 55 to 64 years, a 1-SD increment higher total testosterone was associated with 9.5% lower native T1 time. Higher levels of estradiol in older men were independently associated with higher odds of having a myocardial scar (OR: 4.10; 95% CI: 1.35-12.40; P = 0.01). ConclusionsAmong older men, SH imbalances at initial evaluation were independently associated with CMR defined IMF and replacement fibrosis, respectively; while increasing total testosterone in middle-aged PMW was associated with lesser marker of IMF.

Association between myocardial oxygenation and fibrosis in Duchenne Muscular Dystrophy: analysis by oxygenation-sensitive and T1 mapping cardiovascular magnetic resonance (P7-9.002)

<h3>Objective:</h3> To explore whether myocardial hypoxia exists in DMD patients and whether an association exists between regional myocardial oxygenation and fibrosis, using oxygenation-sensitive cardiovascular magnetic resonance and native T1 mapping. <h3>Background:</h3> Myocardial hypoxia is common in many cardiomyopathies and has been proven to be closely related to the occurrence and development of myocardial fibrosis. However, limited studies are available on the relationship between myocardial oxygenation and fibrosis in DMD patients using oxygenation-sensitive magnetic resonance, and whether myocardium hypoxia exists in DMD patients and its underlying relationship with fibrosis in DMD remains unclear. <h3>Design/Methods:</h3> In this prospective study, 91 DMD patients and 30 volunteers underwent CMR. The correlation between oxygenation SI and native T1 value was assessed by Pearson or Spearman correlation coefficients and the association between oxygenation SI and LGE was analyzed by logistic regression. The survival curves were generated by the Kaplan–Meier analysis and compared by the log-rank test. <h3>Results:</h3> In LGE-patients, the oxygenation SI of all segments except the septal segments was significantly decreased than those of the controls. Inferolateral SI and anterolateral SI were weakly correlated with native T1 values. As for LGE+ patients, a further and significant reduction in oxygenation SI was observed relative to the controls. SI was moderately correlated with native T1 values in both negative and positive segments. Multivariable regression modeling indicated inferolateral SI independently associated with LGE. At an average follow-up of 18.63 months, 31 patients experienced progress of LGE. Patients with reduced inferolateral SI were more likely to have an exacerbation of fibrosis. <h3>Conclusions:</h3> Analysis of OS-CMR and native T1 mapping revealed that myocardial hypoxia is common and related to the onset of myocardial fibrosis in DMD patients. And myocardial oxygenation by OS-CMR might be used as the quantitative imaging biomarker for the assessment of myocardial damage and prediction of fibrosis progression in DMD patients. <b>Disclosure:</b> Ms. Zhou has nothing to disclose.

3-Tesla T2 Mapping Magnetic Resonance Imaging for Evaluation of SLAP Lesions in Patients with Shoulder Pain: An Arthroscopy-Controlled Study.

This study investigated the ability of T2 mapping to assess the glenoid labrum and to differentiate between healthy labral substances and superior labral anterior posterior (SLAP) lesions using arthroscopy as the gold standard. Eighteen patients (mean age: 52.4 ± 14.72 years, 12 men) with shoulder pain were examined using 3-Tesla T2 mapping. All the patients underwent shoulder arthroscopy. Using morphological sequences for correlation, regions of interest covering the entire labral substance were placed in the corresponding T2 maps. The diagnostic cutoff values, sensitivities, and specificities, as well as the inter-reader correlation coefficients (ICCs) determined by two independent radiologists, were calculated. The mean T2 value was 20.8 ± 2.4 ms for the healthy labral substances and 37.7 ± 10.63 ms in the patients with SLAP lesions. The maximum T2 value in normal labrum (21.2 ms) was lower than the minimum T2 value in the patients with SLAP lesions (27.8 ms), leading to sensitivities, specificities, and positive and negative predictive values of 100% (95% CI 54.1-100.0) for all the cutoff values between 21.2 and 27.8 ms. The ICCs ranged from 0.91 to 0.99. In summary, the data suggest that evaluation and quantification of the labral (ultra)structural integrity using T2 mapping may allow discrimination between arthroscopically confirmed SLAP lesions and a healthy glenoid labrum. T2 mapping may therefore be helpful in diagnosing patients with suspected labral damage.

Chronic troponin elevation assessed by myocardial T1 mapping in patients with stable coronary artery disease.

Cardiac troponin detected with sensitive assays can be chronically elevated, in the absence of unstable coronary syndromes. In patients with chronic coronary artery disease, clinically silent ischemic episodes may cause chronic troponin release. T1 mapping is a cardiovascular magnetic resonance technique useful in quantitative cardiac tissue characterization. We selected patients with anatomically and functionally normal hearts to investigate associations between chronic troponin release and myocardial tissue characteristics assessed by T1 mapping. We investigated the relationship between cardiac troponin I concentrations and cardiovascular magnetic resonance T1 mapping parameters in patients with stable coronary artery disease enrolled in MASS V study before elective revascularization. Participants had no previous myocardial infarction, negative late gadolinium enhancement, normal left ventricular function, chamber dimensions and wall thickness. A total of 56 patients were analyzed in troponin tertiles: nativeT1 and extracellular volume (ECV) values (expressed as means ± standard deviations) increased across tertiles: nativeT1 (1006 ± 27 ms vs 1016 ± 27 ms vs 1034 ± 37 ms, ptrend = 0.006) and ECV (22 ± 3% vs 23 ± 1.9% vs 25 ± 3%, ptrend = 0.007). Cardiac troponin I concentrations correlated with native T1(R = 0.33, P = .012) and ECV (R = 0.3, P = .025), and were independently associated with nativeT1 (P = .049) and ventricular mass index (P = .041) in multivariable analysis. In patients with chronic coronary artery disease and structurally normal hearts, troponin I concentrations correlated with T1 mapping parameters, suggesting that diffuse edema or fibrosis scattered in normal myocardium might be associated with chronic troponin release.

Comprehensive cardiac magnetic resonance T1, T2, and extracellular volume mapping to define Duchenne cardiomyopathy

BackgroundCardiomyopathy is the leading cause of death in Duchenne muscular dystrophy (DMD). Cardiac magnetic resonance (CMR) parametric mapping sequences offer insights into disease pathophysiology. We propose a novel approach by leveraging T2 mapping in conjunction with T1 and extracellular volume (ECV) mapping to perform a virtual myocardial biopsy. While previous work has attempted to describe myocardial changes in DMD, our inclusion of T2 mapping enables comprehensive categorization of myocardial tissue characteristics of fibrosis, edema, and fat to better understand the pathological composition of the myocardium with disease progression. MethodsDMD patients (n = 49; median: 12 years-old) underwent CMR, including T1, T2, and ECV. Categories were defined as normal, isolated high T1 (normal ECV, high T1, normal T2), fibrosis (high ECV, normal or high T1, normal T2), edema (normal or high ECV, normal or high T1, high T2), fat (normal ECV, low T1, high T2) or fibrofatty (high ECV, low T1, high T2). ResultsMedian left ventricular ejection fraction (LVEF) was 59% with 27% having LVEF < 55%. Those with normal LVEF and no late gadolinium enhancement (37%) were younger in age (10.5 ± 2.6 vs. 15.0 ± 4.3 years-old, p < 0.001). Native T1 was elevated in at least one slice in 82% of patients. Those with high T2 at any slice (27%) were older (p = 0.005) and had lower LVEF (p = 0.005) compared with subjects with normal T2 (73%). The most common myocardial characterization was fibrosis (43%) followed by isolated high T1 (24%). Of the 13 with high T2, ten were categorized as edema, two as fibrofatty, and one as fat. ConclusionCMR parametric mapping sequences offer insights into Duchenne cardiomyopathy pathophysiology, which should drive development of therapeutic interventions aimed at these targets. Myocardial fibrosis is common in DMD. Patients with elevated T2 were older and had lower LVEF. Though fat infiltration was present, the majority of subjects with elevated T2 met criteria for myocardial edema.