Low T2 Research Articles

Hemochromatosis Mutations, Brain Iron Imaging, and Dementia in the UK Biobank Cohort.

Background: Brain iron deposition occurs in dementia. In European ancestry populations, the HFE p.C282Y variant can cause iron overload and hemochromatosis, mostly in homozygous males. Objective:To estimate p.C282Y associations with brain MRI features plus incident dementia diagnoses during follow-up in a large community cohort.Methods: UK Biobank participants with follow-up hospitalization records (mean 10.5 years). MRI in 206 p.C282Y homozygotes versus 23,349 without variants, including T2* measures (lower values indicating more iron). Results: European ancestry participants included 2,890 p.C282Y homozygotes. Male p.C282Y homozygotes had lower T2* measures in areas including the putamen, thalamus, and hippocampus, compared to no HFE mutations. Incident dementia was more common in p.C282Y homozygous men (Hazard Ratio HR = 1.83; 95% CI 1.23 to 2.72, p = 0.003), as was delirium. There were no associations in homozygote women or in heterozygotes. Conclusion: Studies are needed of whether early iron reduction prevents or slows related brain pathologies in male HFE p.C282Y homozygotes.

Magnetic Resonance Myocardial Feature Tracking in Transfusion-Dependent Myelodysplastic Syndrome.

BACKGROUNDMyocardial deformation with echocardiography allows early detection of systolic dysfunction and is related to myocardial iron overload (MIO) determined by T2* in hereditary anemias under transfusion support. Our aim was to analyze the diagnostic and prognostic usefulness of magnetic resonance feature tracking (MR-FT) myocardial strain in low-risk myelodysplastic syndromes (LR-MDS) patients.METHODSProspective study in transfusion-dependent LR-MDS patients and healthy controls who underwent a cardiac MR-FT. We analyzed the relationships between strain MR-FT and iron overload parameters and its prognostic impact in cardiovascular events and/or death.RESULTSThirty-one patients and thirteen controls were included. MIO (T2* < 20 ms) was detected in 9.7% of patients. Left ventricular global longitudinal strain (LV-GLS) by MR-FT was pathological (> −19.3%) in 32.3% of patients. Less negative strain values correlated with lower T2* (R = −0.37, p = 0.033) and native myocardial T1 (R = −0.39, p = 0.031) times. LV-GLS by MR-FT was significantly associated with higher incidence of the combined cardiovascular events and/or all-cause death (p = 0.047), with a cut-off value of −17.7% for predicting them (63% sensitivity and 81% specificity, area under the curve = 0.69). After adjusting analysis including demographic, biomarkers and imaging variables, a higher LV-GLS value by MR-FT remained as predictor of combined event in transfusion-dependent LR-MDS patients (hazard ratio, 0.4; confidence interval, 0.15–0.98; p = 0.045).CONCLUSIONSLongitudinal myocardial strain by MR-FT in LR-MDS patients is associated to MIO and correlates with adverse events in the follow-up, what could serve as a prognostic tool.

Low Amino Acid Score of Breakfast is Associated with the Incidence of Cognitive Impairment in Older Japanese Adults: A Community-Based Longitudinal Study

BackgroundThe protein digestibility-corrected amino acid score (PDCAAS) represents the degree of utilizable dietary protein, namely the protein quality. The PDCAAS of a diet is required to be evaluated on a meal-by-meal basis, as food digestion and absorption occur with each meal intake. Although a positive association between protein intake and cognitive function has been reported, no study has investigated the association between PDCAAS of a diet and cognitive function. Objectives: To investigate the relationship between PDCAAS of a diet and cognitive impairment in older adults.DesignLongitudinal epidemiological study.SettingCommunity-based setting.ParticipantsWe analyzed 541 community-dwellers who participated in both baseline and follow-up survey. They were 60–83 years of age without cognitive impairment at baseline.MeasurementsCognitive impairment was defined as a Mini-Mental State Examination (MMSE) score ≤27. Individual PDCAASs were calculated for each of three regular meals from the 3-day dietary records at baseline. Participants were classified into two groups according to the sex-specific tertiles (T1–T3) of the PDCAAS for each meal (i.e., T1 as the low score group and T2–T3 as the medium and high score group). The dependent variable was cognitive impairment observed after 4 years, and the explanatory variables were the PDCAAS groups for each meal (the medium and high group as the reference) and covariates (sex, age, body mass index, education, depressive symptoms, medical history, protein intake at each meal, and the MMSE score at baseline). Multivariable logistic regression analysis was performed to evaluate the low PDCAAS group for cognitive impairment after 4 years.ResultsA significant association was observed only between a low PDCAAS of breakfast and the incidence of cognitive impairment (the adjusted odds ratios [95% confidence intervals] of low PDCAAS for cognitive impairment for breakfast, lunch, and dinner were 1.58 [1.00–2.50], 0.85 [0.54–1.34], and 1.08 [0.71–1.65], respectively).ConclusionA lower PDCAAS of breakfast, i.e., a diet with poor quality of protein, was associated with the incidence of cognitive impairment in older adults of the community.

Tissue Characterization in Cardiology: Moving Beyond Function.

Cardiovascular Magnetic Resonance (CMR) offers accurate and highly reproducible tissue characterization, beyond cardiac function. Late gadolinium enhancement (LGE), although represents a noninvasive biopsy for fibrosis quantification, it is unable to detect diffuse myocardial disease. Native T1 mapping and extracellular volume fraction (ECV) are able to provide important information about processes involving both myocardial cells and interstitium that otherwise cannot be identified. Changes in myocardial native T1 mapping reflect cardiac diseases such as acute coronary syndromes, myocardial infarction, myocarditis, diffuse fibrosis, systemic disease such as cardiac amyloidosis, all presented with high T1 and Anderson-Fabry disease and siderosis, presented with low T1 mapping. The ECV, an index generated by native and postcontrast T1 mapping, introduces a new way to measure the cellular and extracellular interstitial matrix (ECM). ECV has a prognostic value equal to Left ventricular ejection fraction (LVEF); however, LVEF underscores the interstitial matrix. This myocyte-ECM dichotomy has important implications for identifying therapeutic targets that are of great value for heart failure (HF) treatment. Furthermore, T2 mapping is superior compared with myocardial T1 and ECM for assessing the activity of myocarditis in recent-onset HF. These indices will affect significantly the clinical decision making. However, there is still lack of multicenter studies and community-wide approach including MRI vendors, clinicians, fundings, softwares, and contrast agent manufacturers.

Comparison of cortico-medullary phase contrast-enhanced MDCT and T2-weighted MR imaging in the histological subtype differentiation of renal cell carcinoma: radiology-pathology correlation.

PurposeRenal cell carcinoma (RCC) subtype differentiation is of crucial importance in the management and prognosis of these patients. In this study, we investigated the usefulness of unenhanced and cortico-medullary phase contrast-enhanced multidetector-row computed tomography (MDCT) and T2-weighted fast spin-echo (FSE) magnetic resonance imaging (MRI) modalities in the discrimination of the 3 main subtype RCC patients in correlation with their histopathological findings.Material and methodsA total of 80 pathologically proven RCC patients who had undergone either partial or total nephrectomy were retrospectively investigated in this study. Their histological subtypes were 54 clear cell renal cell carcinoma (ccRCC), 15 papillary renal cell carcinoma (pRCC), and 11 chromophobe renal cell carcinoma (cRCC), based on pathological evaluation. There were 62 male (77.5%) and 18 female (22.5%) patients. Among the 54 ccRCC patients, 29 patients had both non-contrast and cortico-medullary phase CT, 1 had only non-contrast CT, 5 only had cortico-medullary phase CT, and 38 had MRI examination. In the pRCC group, 10 patients had both non-contrast and cortico-medullary phase CT, 1 had only non-contrast CT, 1 had only cortico-medullary phase CT, and 12 had MRI. Finally, in the remaining 11 cRCC patients, 9 had both non-contrast and cortico-medullary phase CT, and only 5 had MRI. We calculated both tumour attenuation values as HU (Hounsfield units) on unenhanced and cortico-medullary phase MDCT images and also tumour mean signal intensity values on FSE T2-weighted MRI images by using the region of interest (ROI) including normal renal cortex measurements. Besides quantitative evaluation, we also performed qualitative visual assessment of tumours on contrast-enhanced MDCT and FSE T2-weighted MRI.ResultsThere was no statistically significant difference among the attenuation values of the 3 tumour subtypes on pre-contrast CT images. ccRCC demonstrated a prominent degree of contrast enhancement compared to the chromophobe and papillary ones on cortico-medullary phase MDCT. We found no statistically significant difference between chromophobe and papillary subtypes, although chromophobe tumours showed slightly higher attenuation values compared to papillary ones. ccRCCs usually demonstrated a heterogenous contrast enhancement on cortico-medullary phase CT images, while the papillary subtype usually had a homogenous appearance on visual assessment. On FSE T2-weighted MR images, the signal intensity values of ccRCC patients were found to be significantly higher than both chromophobe and papillary subtypes. Although cRCC patients had a prominently lower T2 signal intensity than clear cell subtype, there was no statistically significant signal intensity difference between chromophobe and papillary subtypes. Regarding visual assessment, papillary subtype tumours showed a mostly homogenous appearance on T2-weighted images and a statistically significant difference was present. On the other hand, there was no significant difference of visual assessment of the clear cell and chromophobe subtypes.ConclusionsThe measurement of the attenuation values on cortico-medullary phase MDCT and the mean signal intensity values on FSE T2-weighted MRI can provide useful information in the differentiation of RCC main subtypes. Also, visual assessment of tumours on both modalities can contribute to this issue by providing additional imaging properties.

Interpretation of Brain Volume Increase in Multiple Sclerosis.

A high variability of brain MRI volume change measurement renders challenging its interpretation in multiple sclerosis (MS). Occurrence and clinical relevance of observed apparent brain volume increase (BVI) in MS patients have not been investigated yet. The objective was to quantify the prevalence and factors associated with BVI. We examined 366 MS patients (2,317 scans) and 44 controls (132 scans). Volumetric analysis of brain volume changes was performed by SIENA and ScanView. BVI was defined as brain volume change >0%. We compared characteristics of patients with and without BVI. BVI was found in 26.3% (from 1,951) longitudinal scans (SIENA). If BVI occurred, a probability that BVI will be repeated consecutively more than or equal to two times was 15.9%. The repeated BVI was associated with clinical disease activity in 50% of cases. BVI was associated with shorter time and lower T2 lesion volume increase between two MRI scans, and higher normalized brain volume (all P<.0001). A proportion of scans with BVI was higher when analyzed by ScanView (35.3%) and in controls (36.4% by SIENA). BVI occurs in a great proportion of MR scans over short-term follow-up and is not associated with disease stabilization. Although BVI can be caused by several factors, the results indicate that measurement error may contribute to BVI in the majority of cases. Clinicians should be aware of the frequent occurrence of apparent BVI, interpret brain volume changes in MS patients with great caution, and use methods with precise quantification of brain volume changes.

Using excitation-emission matrix fluorescence to evaluate the performance of water treatment plants for dissolved organic matter removal

This study is aimed at assessing the performance of water treatment plants (both wastewater and drinking water treatment plants) for dissolved organic matter (DOM) removal using excitation-emission matrix fluorescence (EEMF) as the monitoring technique.The influent from the wastewater treatment plant (WWTP) of Burgos (Spain) is characterized from the presence of protein-like peaks (T1 and T2) and humic-like peaks (A and C), T2 and A showing the highest fluorescence intensity. The percentages of total removal in the effluent were in the following order: peak T1 (65%) > peak A (45%) > peak C (34%) > peak T2 (26%). The humic-like peaks were the most removed at the primary sedimentation stage, whereas peak T1 was by far the most removed in the biological reactor. Protein-like peaks T1 and T2 experienced a slight increase in the final effluent in comparison to their fluorescence at the previous stage (the exit of the biological reactor), an increase that can be explained by the release of SMP (soluble microbial products) from the biomass in the secondary clarifier. A poor correlation was obtained between peak T2 fluorescence and COD, BOD (r2 = 0.34–0.38).The natural water from the Úzquiza reservoir in Burgos (Spain) is characterized by the only presence of humic substances: a majority peak A (fulvic-like) and a weak peak C (humic-like). The whole fluorescent DOM was removed by coagulation-flocculation but a low fluorescence peak T2 appeared at the final stage, coming from protein-like SMPs released by the biomass attached to the filters.

Improved discrimination of molecular subtypes in invasive breast cancer: Comparison of multiple quantitative parameters from breast MRI

PurposeTo compare multiple quantitative parameters from breast magnetic resonance imaging (MRI) with the synthetic MRI sequence included for discrimination of molecular subtypes of invasive breast cancer. Materials and methodsBetween March 2019 and September 2020, two hundred breast cancer patients underwent preoperative breast multiparametric MRI examinations including synthetic MRI, diffusion weighted imaging (DWI) and dynamic contrast enhancement (DCE)-MRI sequences. MRI morphological features, T1 and T2 relaxation times (T1, T2) and proton density (PD) values from synthetic MRI, Ktrans, Kep, and Ve from DCE-MRI, mean apparent diffusion coefficient (ADC) from DWI and tumor volume were measured. Quantitative parameters were compared according to molecular markers and subtypes. Logistic regression were performed to find the related MRI parameters and establish combined parameters. The comparison between single and combined quantitative parameters by using DeLong tests. ResultsT1, T2 values were significantly higher in hormone receptor (HR)- negative and Ki67 > 14% tumors (p < 0.05). Human epidermal growth factor receptor 2 (HER2)-positive tumors demonstrated significantly higher Ktrans and Kep (p < 0.01). Mean ADC values were significantly decreased in HR-positive and Ki67 > 14% tumors (p < 0.01). Tumor volumes were significantly higher in HER2-positive and Ki67 > 14% tumors (p < 0.05). Independent influencing factors were lower T2 values (p < 0.001), smaller tumor volume (p = 0.031) and higher mean ADC (p = 0.002) associated with luminal A subtype, while T1 values (p = 0.007) was the only quantitative parameter associated with triple-negative subtype. The diagnostic efficiency of combined parameters (T2 + mean ADC + volume) (AUC = 0.765) was significantly higher than that of mean ADC (AUC = 0.666, p = 0.031 by DeLong test) and volume (AUC = 0.650, p = 0.008 by DeLong test) for separating luminal A subtype. ConclusionsMRI quantitative parameters could help distinguish molecular markers and subtypes. The emerging synthetic MRI parameters - T1 values were associated with the TN subtype, and combined parameters with added T2 values might improve the discrimination of the luminal A subtype. Application of synthetic MRI can enrich quantitative descriptors from breast MRI.

Neural Basis of Semantically Dependent and Independent Cross-Modal Boosts on the Attentional Blink.

The present study recorded event-related potentials (ERPs) in a visual object-recognition task under the attentional blink paradigm to explore the temporal dynamics of the cross-modal boost on attentional blink and whether this auditory benefit would be modulated by semantic congruency between T2 and the simultaneous sound. Behaviorally, the present study showed that not only a semantically congruent but also a semantically incongruent sound improved T2 discrimination during the attentional blink interval, whereas the enhancement was larger for the congruent sound. The ERP results revealed that the behavioral improvements induced by both the semantically congruent and incongruent sounds were closely associated with an early cross-modal interaction on the occipital N195 (192-228ms). In contrast, the lower T2 accuracy for the incongruent than congruent condition was accompanied by a larger late occurring cento-parietal N440 (424-448ms). These findings suggest that the cross-modal boost on attentional blink is hierarchical: the task-irrelevant but simultaneous sound, irrespective of its semantic relevance, firstly enables T2 to escape the attentional blink via cross-modally strengthening the early stage of visual object-recognition processing, whereas the semantic conflict of the sound begins to interfere with visual awareness only at a later stage when the representation of visual object is extracted.

MRI evaluation of solid soft tissue masses of the fingers with pathology correlation

ObjectiveSpace occupying lesions of the fingers are commonly encountered in clinical and radiology practice. The objective of this study was to determine the characteristics of these lesions on MRI and to correlate with surgical pathology results. Material and methodsThis IRB-approved HIPAA-compliant study retrospectively evaluated the clinical, imaging and pathology findings of 100 consecutive patients referred for evaluation of solid soft tissue masses of the fingers. Only solid lesions with MR imaging prior to surgery were included in this study. MR images and when available corresponding radiographs were evaluated by two radiologist in regard to signal characteristics, relation to surrounding structures and enhancement. All masses were classified into different groups based on pathology results. ResultsTenosynovial giant cell tumor (TSGCT) and fibroma of the tendon sheath were the most common solid tumors (36 %) and malignant tumors represent only 5% of the cohort. 70 % of masses with low T2 signal were TSGCT and fibroma of tendon sheath; 100 % of masses with peripheral high T2 signal and central low T2 signal/thrombus were vascular lesions, representing 41 % of the total vascular lesions in the cohort. Additionally, 100 % of serpiginous/tubular shaped masses were vascular lesions and 67 % of masses with infiltrative borders were malignant masses. Enhancement was important in identifying solid lesions but there was no statistical difference between groups related to enhancement pattern. There was a good agreement for all analyses between both readers. ConclusionTypical MR findings can help to narrow the differential diagnosis in the evaluation of finger masses and provide crucial information to guide further management.

White matter alteration and cerebellar atrophy are hallmarks of brain MRI in alpha-mannosidosis

ObjectiveDespite profound neurological symptomatology there are only few MRI studies focused on the brain abnormalities in alpha-mannosidosis (AM). Our aim was to characterize brain MRI findings in a large cohort of AM patients along with clinical manifestations. MethodsTwenty-two brain MRIs acquired in 13 untreated AM patients (8 M/5F; median age 17 years) were independently assessed by three experienced readers and compared to 16 controls. ResultsFocal and/or diffuse hyperintense signals in the cerebral white matter were present in most (85%) patients. Cerebellar atrophy was common (62%), present from the age of 5 years. Progression was observed in two out of 6 patients with follow-up scans. Cortical atrophy (62%) and corpus callosum thinning (23%) were already present in a 13-month-old child. The presence of low T2 signal intensity in basal ganglia and thalami was excluded by the normalized signal intensity profiling. The enlargement of perivascular spaces in white matter (38%), widening of perioptic CSF spaces (62%), and enlargement of cisterna magna (85%) were also observed. Diploic space thickening (100%), mucosal thickening (69%) and sinus hypoplasia (54%) were the most frequent non-CNS abnormalities. ConclusionWhite matter changes and cerebellar atrophy are proposed to be the characteristic brain MRI features of AM. The previously reported decreased T2 signal intensity in basal ganglia and thalami was not detected in this quantitative study. Rather, this relative MR appearance seems to be related to the diffuse high T2 signal in the adjacent white matter and not the gray matter iron deposition that has been hypothesized.

The diagnostic performance of quantitative mapping in breast cancer patients: a preliminary study using synthetic MRI

BackgroundPrevious studies have indicated that quantitative MRI (qMR) is beneficial for diagnosis of breast cancer. As a novel qMR technology, synthetic MRI (syMRI) may be advantageous by offering simultaneous generation of T1 and T2 mapping in one scan within a few minutes and without concern to the deposition of the gadolinium contrast agent in cell nucleus. In this study, the potential of quantitative mapping derived from Synthetic MRI (SyMRI) to diagnose breast cancer was investigated.MethodsFrom April 2018 to May 2019, a total of 87 patients with suspicious breast lesions underwent both conventional and SyMRI before treatment. The quantitative metrics derived from SyMRI, including T1 and T2 values, were measured in breast lesions. The diagnostic performance of SyMRI was evaluated with unpaired Student’s t-tests, receiver operating characteristic curve analysis and multivariate logistic regression analysis. The AUCs of quantitative values were compared using Delong test.ResultsAmong 77 patients who met the inclusion criteria, 48 were diagnosed with histopathological confirmed breast cancers, and the rest had benign lesions. The breast cancers showed significantly higher T1 (1611.61 ± 215.88 ms) values and lower T2 (80.93 ± 7.51 ms) values than benign lesions. The area under the ROC curve (AUC) values were 0.931 (95% CI: 0.874–0.989) and 0.883 (95% CI: 0.810–0.956) for T1 and T2 maps, respectively, in diagnostic discrimination between breast cancers and benign lesions. A slightly increased AUC of 0.978 (95% CI: 0.915–0.993) was achieved by combining those two relaxation-based quantitative metrics.ConclusionIn conclusion, our preliminary study showed that the quantitative T1 and T2 values obtained by SyMRI could distinguish effectively between benign and malignant breast lesions, and T1 relaxation time showed the highest diagnostic efficiency. Furthermore, combining the two quantitative relaxation metrics further improved their diagnostic performance.

Bladder-sparing protocols in the treatment of muscle-invasive bladder cancer.

Bladder-sparing protocols (BSP) have been gaining widespread popularity as an attractive alternative to radical cystectomy (RC) for muscle-invasive bladder cancer. Unimodal therapies are inferior to multimodal regimens. The most promising regimen is trimodal therapy (TMT), which is a combination of maximal transurethral resection of bladder tumor (TURBT), radiotherapy, and chemotherapy. In appropriately selected patients (low volume unifocal T2 disease, complete TURBT, no hydronephrosis and no carcinoma-in-situ), comparable oncological outcomes to RC have been reported in large retrospective studies, with a potential improvement in overall quality of life (QOL). TMT also offers the possibility for definitive therapy for patients who are not surgically fit to undergo RC. Routine biopsy of previous tumor resection is recommended to assess response. Prompt salvage RC is required in non-responders and for recurrent muscle-invasive disease, while non-muscle-invasive recurrence can be managed conservatively with TURBT +/− intravesical BCG. Long-term follow-up consisting of routine cystoscopy, urine cytology, and cross-section imaging is required. Further studies are warranted to better define the role of neoadjuvant or adjuvant chemotherapy in the setting of TMT. Finally, future research on predictive markers of response to TMT and on the integration of immunotherapy in bladder sparing protocols is ongoing and is highly promising.

Myocardial Tissue Reverse Remodeling After Guideline-Directed Medical Therapy in Idiopathic Dilated Cardiomyopathy.

The prognosis of patients with idiopathic dilated cardiomyopathy (DCM) has improved remarkably in recent decades with guideline-directed medical therapy. Left ventricular (LV) reverse remodeling (LVRR) is one of the major therapeutic goals. Whether myocardial fibrosis or inflammation would reverse associated with LVRR remains unknown. A total of 157 prospectively enrolled patients with DCM underwent baseline and follow-up cardiovascular magnetic resonance examinations with a median interval of 13.7 months (interquartile range, 12.2-18.5 months). LVRR was defined as an absolute increase in LV ejection fraction of >10% to the final value of ≥35% and a relative decrease in LV end-diastolic volume of >10%. Statistical analyses were performed using paired t test and student t test, logistic regression analysis, and linear regression analysis. Forty-eight (31%) patients reached LVRR. At baseline, younger age, worse New York Heart Association class, new-onset heart failure, lower LV ejection fraction, absence of late gadolinium enhancement, lower myocardial T2, and extracellular volume were significant predictors of LVRR. During the follow-up, patients with and without LVRR both showed a significant decrease of myocardial native T1 (LVRR: [baseline] 1303.0±43.6 ms; [follow-up] 1244.7±51.8 ms; without LVRR: [baseline] 1308.5±80.5 ms; [follow-up] 1287.6±74.9 ms, both P<0.001), matrix and cellular volumes while no significant difference was observed in T2 or extracellular volume values after treatment. In patients with idiopathic DCM, the absence of late gadolinium enhancement, lower T2, and extracellular volume values at baseline are significant predictors of LVRR. The myocardial T1, matrix, and cell volume decrease significantly in patients with LVRR after guideline-directed medical therapy. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: ChiCTR1800017058.

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

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&amp;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.

A review of clinical and MR imaging features of renal lipid-poor angiomyolipomas.

Lipid-poor angiomyolipomas (lpAMLs) constitute up to 5% of renal angiomyolipomas and are challenging to differentiate from malignant renal lesions on imaging alone. This review aims to identify clinical and MRI features which can be utilized to improve specificity and diagnostic accuracy for detecting lpAMLs in patients being considered for active surveillance rather than intervention. Young age, female sex, and small lesion size are associated with lpAMLs in studies evaluating indeterminate renal lesions. The accuracy of criteria using T2-weighted imaging, diffusion-weighted imaging, chemical shift imaging, dynamic contrast enhancement, multiparametric imaging, and radiomics are reviewed. Low T2 signal intensity is a particularly important MRI feature for lpAML. In studies with low T2 signal intensity, homogeneous early enhancement is a typical feature with an arterial-to-delay enhancement ratio > 1.5. Intratumoral hemorrhage with decrease in signal intensity on in-phase chemical shift imaging may be particularly useful for differentiating papillary renal cell carcinomas from lpAMLs in low T2 signal intensity lesions. Combining clinical and multiparametric MRI features can result in near-perfect specificity for lpAML. In select patients, clinical and MRI features can result in a high specificity and diagnostic accuracy for lpAMLs. These lesions can be considered for active surveillance rather than invasive diagnostic and therapeutic procedures such as biopsy or surgery.

Subluxing fractured plantar fat pad: a case series and description of novel sonographic findings.

Plantar fat pad syndrome has received little attention in the literature. A variety of structural changes of the plantar fat pad have been described in the literature, including atrophy, contusion, and fractured fat pad. This case series presents 4 patients (5 heels) with subluxation of a fractured plantar fat pad on dynamic ultrasound. Patients with subluxing fractured fat pad typically present with heel pain and a "snapping" or "popping" sensation when weight-bearing. Other causes of heel pain were excluded, and all patients in this series had an MRI that initially did not report any findings in the fat pad. Retrospective review of the MRI showed evidence of diffuse low T1 and T2 infiltration. To the authors' knowledge, subluxation of the plantar fat pad and the respective correlation to MRI findings have not been described in the literature. Here we describe the sonographic findings of this novel condition.

The myocardial phenotype of Fabry disease pre-hypertrophy and pre-detectable storage

Abstract Background Cardiac involvement in Fabry Disease (FD) occurs prior to left ventricular hypertrophy (LVH) and is characterized by low myocardial native T1 with storage reflected by cardiovascular magnetic resonance (CMR) and ECG changes. Objectives We hypothesize that a pre-storage myocardial phenotype might occur even earlier, prior to T1 lowering. Methods FD patients and age, sex and heart rate matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR (cines, global longitudinal strain [GLS], T1 and T2 mapping, stress perfusion [myocardial blood flow, MBF] and late gadolinium enhancement [LGE]). Results 114 Fabry patients (46±13 years, 61% female) and 76 controls (49±15 years, 50% female) were included. In pre-LVH FD (n=72, 63%), a low T1 (n=32/72, 44%) was associated with a constellation of ECG and functional abnormalities compared to normal T1 FD patients and controls. However, pre-LVH FD with normal T1 (n=40/72, 56%) also had abnormalities compared to controls: reduced GLS (−18±2 vs −20±2%, P&amp;lt;0.001), microvascular changes (lower MBF 2.5±0.7 vs 3.0±0.8mL/g/min, P=0.028), subtle T2 elevation (50±4 vs 48±2ms, p=0.027) and limited LGE (%LGE 0.3±1.1 vs 0%, P=0.004). ECG abnormalities included shorter P wave duration (88±12 vs 94±15ms, P=0.010) and T wave peak time (Tonset–Tpeak; 104±28 vs 115±20ms, P=0.015), resulting in a more symmetric T wave with lower T wave time ratio (Tonset–Tpeak)/(Tpeak–Tend) (1.5±0.4 vs 1.8±0.4, P&amp;lt;0.001) compared to controls. Conclusions FD has a measurable myocardial phenotype pre-LVH and pre-detectable myocyte storage with microvascular dysfunction, subtly impaired GLS and altered atrial depolarization and ventricular repolarization intervals. Proposed stages of cardiac involvement Funding Acknowledgement Type of funding source: None

Progressive electrocardiographic changes in parallel with cardiac magnetic resonance findings in fabry disease

Abstract Background Cardiac Magnetic Resonance (CMR) allows to detect progressive stages of cardiac involvement in Fabry Disease (FD). A systematic description of electrocardiographic (ECG) alterations occurring in FD is currently missing. Purpose To explore ECG changes in progressive stages of FD cardiomyopathy. Methods 71 FD patients and 17 healthy controls underwent CMR with T1 mapping and 12-lead ECG. ECG analysis included the duration of the P-wave and the interval between the end of P-wave and the beginning of QRS (PendQ). FD patients in the test cohort were divided into 3 groups with increasing severity of cardiac involvement: A) normal T1, no LVH; B) low T1, no LVH; C) low T1, LVH. Results An increase of Pwave/PendQ ratio was observed in Group A compared to Controls (1.08 vs. 0.75, p&amp;lt;0.0001). Higher Pwave/PendQ ratio (1.50 vs. 1.08, p&amp;lt;0.0001), shorter PQc interval (127.9 vs. 159.5, p=0.0007), increased Sokolow-Lyon Index (SLI) (3.2 vs. 2.4, p&amp;lt;0,001) and T wave amplitude (0.6 vs. 0.4 mV, p=0.002) characterized Group B in comparison with Group A. A higher prevalence of left bundle branch blocks (13.6% vs. 0%, p=0.03) and repolarization abnormalities (77.3% vs. 5.7%), wider QRS (120 vs 95 msec, p&amp;lt;0.0001) and QT (460 vs 400 msec, p=0.003) intervals were found in Group C compared to Group B. SLI (AUC 0.769), Pwave/PendQ (AUC 0.778), QRS (AUC 0.703) and QT (AUC 0.769) durations resulted to be independent predictors of low T1 values on CMR at stepwise multivariate analysis. Conclusion FD is characterized by progressive ECG changes. The identification of ECG parameters able to predict a lowering of myocardial T1 values on CMR may promote early detection of cardiac involvement, helping to target the therapeutic approach. Progressive ECG and CMR changes in FD Funding Acknowledgement Type of funding source: Other. Main funding source(s): This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.

Cardiac tissue characterization with t1 and t2 mapping in anderson fabry patients: new pathophysiological concepts and early cardiac involvement

Abstract Background Anderson Fabry (AF) disease is a X-linked lysosomal storage disorder with multiorgan involvement. Cardiac disease, mainly represented by left ventricular hypertrophy (LVH) and arrhythmias, is the most frequent cause of premature death. It is well know that specific therapy is less effective after the development of LVH and myocardial fibrosis, therefore early cardiac detection (before LVH) is important. New cardiac magnetic resonance (CMR) parametric imaging techniques (T1 and T2 maps) enable myocardial tissue changes associated with AF disease. Purpose To evaluate the relationship between CMR tissue characterization and clinical and instrumental manifestations of AF disease to find early markers of cardiac involvement. Methods 31 AF patients (9 males, mean age 49±16 years) underwent ECG, echocardiogram and contrast CMR. TnI, BNP, pro-BNP and serum lyso-Gb3 were dosed. T1 mapping was performed in a pre-contrast acquisition with the modified Look-Locker inversion recovery (MOLLI) sequences. CMR results were compared with those of 43 healthy age and gender-matched controls. Results In AF patients native septal T1 values were significantly lower compared to healthy controls (median 949 vs 991 msec, p=0.0137) and were inversely related to Lyso-Gb3 serum levels (p=0.003). Patients with LVH had lower T1 septal values in comparison with patients without LVH (892 vs 981 msec; p=0.0012). Patients with classic form had abnormal low T1 values more frequently than pts with late onset variant (78 vs 23%; p=0.038). In AF patients native septal T2 values were significantly higher compared to the control group (53 vs 49 msec; p=0.0004) and correlated with troponin I (p=0.008) and NT-pro BNP (p=0.006) serum levels. No difference was found between pts with and without LVH (53.5 vs 52.5 ms; p=0.797) and the prevalence of abnormal high T2 values was similar between patients with late onset AF and pts with classical form (53% vs 50%; p=1.000). All patients with late onset AF and high T2 values were females. Conclusions CMR T1 (low values) and T2 (high values) mapping are useful tools to detect early cardiac involvement before LVH and to better understand the pathophysiology of cardiac disease in AF patients. Subclinical tissue inflammation, detectable through T2 maps, seems to be an additional pathogenetic mechanism related to the Gb3 storage that contributes to organ damage and precedes LVH, particularly in females patients with late onset phenotype. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Sant'Orsola-Malpighi Hospital