Wearable cardioverter-defibrillator: friend or foe in suspected myocarditis?

AIM: Wearable cardioverter defibrillator (WCD, LifeVest, and Zoll) therapy has become a useful tool to bridge a temporarily increased risk for sudden cardiac death. However, despite extensive use, there is a lack of evidence whether patients with myocarditis and impaired LVEF may benefit from treatment with a WCD. METHODS AND RESULTS: We conducted a single-centre retrospective observational study analysing patients with a WCD prescribed between September 2015 and April 2020 at our institution. In total, 135 patients were provided with a WCD, amongst these 76 patients (mean age 48.9 ± 13.7 years; 84.2% male) for clinically suspected myocarditis. Based on the results of the endomyocardial biopsy and, where available cardiac magnetic resonance imaging, 39 patients (51.3%) were diagnosed with myocarditis and impaired LVEF and 37 patients (48.7%) with dilated cardiomyopathy (DCM) without evidence of cardiac inflammation. The main immunohistopathological myocarditis subtype was lymphocytic myocarditis in 36 (92.3%) patients, and four patients (10.3%) of this group had an acute myocarditis. Three patients had cardiac sarcoidosis (7.7%). Ventricular tachycardia occurred in seven myocarditis (in total 41 VTs; 85.4% non-sustained) and one DCM patients (in total one non-sustained ventricular tachycardia). Calculated necessary WCD wearing time until ventricular tachycardia occurrence is 86.41 days in myocarditis compared with 6.46 years in DCM patients. CONCLUSIONS: Our data suggest that myocarditis patients may benefit from WCD therapy. However, as our study is not powered for outcome, further randomized studies powered for the outcome morbidity and mortality are necessary.

68Ga-PSMA-PET/CT for the evaluation of liver metastases in patients with prostate cancer.

BACKGROUND: The purpose of this study was to evaluate the imaging properties of hepatic metastases in 68Ga-PSMA positron emission tomography (PET) in patients with prostate cancer (PC). METHODS: 68Ga-PSMA-PET/CT scans of PC patients available in our database were evaluated retrospectively for liver metastases. Metastases were identified using 68Ga-PSMA-PET, CT, MRI and follow-up scans. Different parameters including, maximum standardized uptake values (SUVmax) of the healthy liver and liver metastases were assessed by two- and three-dimensional regions of interest (2D/3D ROI). RESULTS: One hundred three liver metastases in 18 of 739 PC patients were identified. In total, 80 PSMA-positive (77.7%) and 23 PSMA-negative (22.3%) metastases were identified. The mean SUVmax of PSMA-positive liver metastases was significantly higher than that of the normal liver tissue in both 2D and 3D ROI (p ≤ 0.05). The mean SUVmax of PSMA-positive metastases was 9.84 ± 4.94 in 2D ROI and 10.27 ± 5.28 in 3D ROI; the mean SUVmax of PSMA-negative metastases was 3.25 ± 1.81 in 2D ROI and 3.40 ± 1.78 in 3D ROI, and significantly lower than that of the normal liver tissue (p ≤ 0.05). A significant (p ≤ 0.05) correlation between SUVmax in PSMA-positive liver metastases and both size (ρSpearman = 0.57) of metastases and PSA serum level (ρSpearman = 0.60) was found. CONCLUSIONS: In 68Ga-PSMA-PET, the majority of liver metastases highly overexpress PSMA and is therefore directly detectable. For the analysis of PET images, it has to be taken into account that also a significant portion of metastases can only be detected indirectly, as these metastases are PSMA-negative.

Assessment of the tibial slope is highly dependent on the type and accuracy of the preceding acquisition.

BACKGROUND: Precise measurement of the tibial slope (TS) is crucial for realignment surgery, ligament reconstruction, and arthroplasty. However, there is little consensus on the ideal assessment. It was hypothesized that the tibial slope changes according to the acquisition technique and both tibial length as well as femoral rotation serve as potential confounders. METHODS: 104 patients (37 women, 67 men; range 12-66 years) were retrospectively selected, of which all patients underwent a 1.5-Tesla MRI and either additional standard lateral radiographs (SLR, n = 52) or posterior stress radiographs (PSR, n = 52) of the index knee. Two blinded observers evaluated the medial tibial slope as the medial TS is primarily used in clinical practice. Additionally, the length of the diaphyseal axis and the extent of radiographic malrotation were measured. RESULTS: Mean TS on MRI was significantly lower compared to radiographs (4.2° ± 2.9° vs. 9.1° ± 3.6°; p < 0.0001). There was a significant correlation between MRI and PSR (p < 0.0001 with r = 0.7), but not with SLR (p = 0.93 with r = 0.24). Tibial length was a significant predictor for the difference between MRI and SLR (regression coefficient ß = - 0.03; p = 0.035), yet not between MRI and PSR (ß = - 0.003; p = 0.9). Femoral rotation proved to be a significant predictor for the agreement between both observers (PSR: ß = 0.14; p = 0.001 and SLR: ß = 0.08; p = 0.04). ICC indicated a high interrater agreement for the radiographic assessment (ICC ≥ 0.72). CONCLUSIONS: There is a substantial variance between MRI and radiographic measurement of the tibial slope. However, as MRI assessment is time-consuming and requires specialized software, instrumented radiographs might be an alternative. Due care has to be taken to ensure that radiographs contain a sufficient tibial length, and femoral rotation is avoided. STUDY DESIGN: Case series (diagnosis); Level of evidence, 4.

68 Ga-PSMA-PET/CT for the evaluation of pulmonary metastases and opacities in patients with prostate cancer.

BACKGROUND: The purpose of this study was to investigate the imaging properties of pulmonary metastases and benign opacities in 68Ga-PSMA positron emission tomography (PET) in patients with prostate cancer (PC). METHODS: 68Ga-PSMA-PET/CT scans of 739 PC patients available in our database were evaluated retrospectively for lung metastases and non-solid focal pulmonary opacities. Maximum standardized uptake values (SUVmax) were assessed by two- and three-dimensional regions of interest (2D/3D ROI). Additionally CT features of the lesions, such as location, morphology and size were identified. RESULTS: Ninety-one pulmonary metastases and fourteen opacities were identified in 34 PC patients. In total, 66 PSMA-positive (72.5%) and 25 PSMA-negative (27.5%) metastases were identified. The mean SUVmax of pulmonary opacities was 2.2±0.7 in 2D ROI and 2.4±0.8 in 3D ROI. The mean SUVmax of PSMA-positive pulmonary metastases was 4.5±2.7 in 2D ROI and in 4.7±2.9 in 3D ROI; this was significantly higher than the SUVmax of pulmonary opacities in both 2D and 3D ROI (p<0.001). The mean SUVmax of PSMA-negative metastases was 1.0±0.5 in 2D ROI and 1.0±0.4 in 3D ROI, and significantly lower than that of the pulmonary opacities (p<0.001). A significant (p<0.05) weak linear correlation between size and 3D SUVmax in lung metastases (ρSpearman=0.207) was found. CONCLUSION: Based on the SUVmax in 68Ga-PSMA-PET alone, it was not possible to differentiate between pulmonary metastases and pulmonary opacities. The majority of lung metastases highly overexpressed PSMA, while a relevant number of metastases were PSMA-negative. Pulmonary opacities demonstrated a moderate tracer uptake, significantly lower than PSMA-positive lung metastases, yet significantly higher than PSMA-negative metastases.

Performance survey on a new standardized formula for oral signal suppression in MRCP.

BACKGROUND: Orally administered substances, which reduce image contamination by overlying gastrointestinal fluid signals, can be used to enhance the quality of MRCP images. Recently, a new standardized formula consisting of biological substances has become available. The objective of this study is to provide a first assessment of achievable MRCP image quality, taste and palatability of this new dedicated agent. METHODS: In January 2015 to May 2015 practicing radiologists in Germany, Austria and Switzerland were asked to evaluate image quality as well as taste and palatability when using the new agent (LumiVision®; b.e. imaging; Baden-Baden; Germany) in MRCP questionnaire. Both criteria were rated with a 6-point Likert scale ranging from "1" (best) to "6" (worst). RESULTS: A total 185 of 475 radiologic institutions (39%) submitted feedback on image quality, 187 (39%) on the taste/palatability. Assessments of image quality regarding presence of disturbing gastrointestinal fluid signal resulted in a median of 2. The majority of patients rated the subjective taste as very good (median of 1). No side effects of relevance were recorded. CONCLUSION: This large survey shows that the tested product is considered effective by radiologists regarding MRCP image quality. Patients' feedback on taste and palatability was very positive.

High tibial slope correlates with increased posterior tibial translation in healthy knees.

PURPOSE: Notwithstanding the importance of the tibial slope (TS) for anterior tibial translation, little information is available regarding the implications on posterior laxity, particularly in healthy subjects. It was hypothesized that increased TS is associated with decreased posterior tibial translation (PTT) in healthy knees. METHODS: A total of 124 stress radiographs of healthy knees were enrolled in this study. Tibial slope and the posterior tibial translation were evaluated using a Telos device with a 150-N force at 90° of knee flexion. Two blinded observers reviewed independently on two different occasions. RESULTS: One hundred and twenty-four patients [35 females and 89 males; 41 (range 18-75) years] were enrolled in this study, with a mean PTT of 2.8 mm (±1.9 mm; range 0-8 mm) and a mean TS of 8.6° (±2.6°; range 1°-14°). Pearson correlation showed a significant correlation between the PTT and TS in the overall patient cohort (P < 0.0001) with r = 0.76 and R 2 = 0.58. There was no statistical difference between female and male patients regarding the PTT or the TS. Subgrouping of the patient cohort (four groups with n = 31) according to their TS (groups I < 7°; II = 7°-8.5°; III = 9°-10.5°; IV ≥ 11°) revealed significant differences between each subgroup, respectively. Furthermore, there was a weak but significant correlation between age and PTT (P = 0.004, r = 0.26). CONCLUSION: In addition to the substantial variance in tibial slope and posterior laxity among healthy knees, high tibial slope significantly correlates with increased posterior tibial translation. Increasing age is further associated with a greater magnitude of posterior tibial translation. Consequently, knowledge of the tibial slope facilitates simple estimation of posterior knee laxity, which is mandatory for PCL reconstruction and knee arthroplasty.

Efficacy of oral contrast agents for upper gastrointestinal signal suppression in MRCP: A systematic review of the literature.

BACKGROUND: Orally administered substances which suppress signals from gastrointestinal fluid can be used to enhance image quality in magnetic resonance cholangiopancreatography (MRCP). In daily practice, the available substances range from commercial products to regular viands such as fruit juices. PURPOSE: To provide an overview on the significance of and the substances used as gastrointestinal fluid signal suppressors in MRCP. MATERIAL AND METHODS: A systematic review of the existing literature was performed to evaluate the efficacy and efficiency of oral T2-signal suppressors in MRCP. RESULTS: Twenty-five publications on 16 different oral contrast media were identified. The most commonly used substances were ferumoxsil, ferric ammonium citrate, and pineapple juice. Twenty-three out of 25 publications supported the use of oral signal suppressors in MRCP. Advantages of oral signal suppressors include improved visualization of the pancreatobiliary ductal system, increased help with differential diagnoses, and higher detection rates of relevant diagnoses due to a reduction of overlaying signals. CONCLUSION: The application of oral substances for gastrointestinal signal suppression in MRCP is recommendable. A variety of substances are used in daily routine with good but varying effectivity and patient tolerance.

DNA Cytometry for Differentiation Between Low- and Medium-grade Dysplasia in Intraductal Papillary Mucinous Neoplasms.

BACKGROUND/AIM: The indication for resection of cystic pancreatic lesions is usually performed by sectional imaging criteria, such as the Sendai criteria. The aim of this study was to analyze a possible correlation between DNA cytometry and Sendai criteria for the differentiation between low-grade intraductal papillary mucinous neoplasms (IPMN-A) and medium-grade dysplasia (IPMN-B). MATERIALS AND METHODS: Histopathological analysis, DNA index and preoperative Sendai criteria were determined in 16 patients who underwent pancreatic resection for IPMN. RESULTS: All patients with IPMN-B showed aneuploid histograms with DNA indices ≥1.3, whereas three out of four patients with IPMN-A had diploid DNA indices ≤1.3. All 11 patients with one or more high-risk stigmata and aneuploid histograms had IPMN-Bs, whereas both patients who were Sendai-negative and diploid in the DNA analysis had an IPMN-A. CONCLUSION: DNA index may be an important diagnostic tool for the differentiation of different IPMN types beyond the traditional Sendai criteria.

Diagnostic performance of susceptibility-weighted magnetic resonance imaging for the assessment of sub-coracoacromial spurs causing subacromial impingement syndrome.

OBJECTIVES: To evaluate the potential of susceptibility-weighted-magnetic-resonance-imaging (SWMR) for the detection of sub-coracoacromial spurs in patients with clinically suspected subacromial impingement syndrome (SAIS), compared to standard MR-sequences and radiographs. METHODS: Forty-four patients with suspected SAIS were included. All patients underwent radiography, standard MRI of the shoulder and SWMR. Radiograph-based identification of sub-coracoacromial spurs served as goldstandard. Radiographs identified twenty-three spurs in twenty-three patients. Twenty-one patients without spur formation served as reference group. Detection rate, sensitivity/specificity and interobserver-agreements were calculated. Linear regression was applied to determine the relationship between size measurements on radiographs and MRI. RESULTS: Detection rates for spurs on standard MRI and SWMR were 47.8 % and 91.3 % compared to radiography (p<0.001). SWMR demonstrated a sensitivity of 97.7 % (CI=0.92-1) and a specificity of 91.3 % (CI=0.788-1) for the identification of spurs. Standard MR-sequences achieved a sensitivity of 47.8 % (CI=0.185-0.775) and a specificity of 80.8 % (CI=0.642-0.978). Size measurements between SWMR and radiography showed a good correlation (R2=0.75;p<0.0001), while overestimating lesion size (5.7±1.2 mm; 4.3±1.3 mm;p<0.0001). Interobserver-agreement for spurs was high on SWMR (R2=0.74;p<0.0001), but low on standard MRI (R2=0.24;p<0.0001). CONCLUSIONS: SWMR allows a reliable detection of sub-coracoacromial spur formation in patients with SAIS and is superior to standard MR-sequences using radiography as goldstandard. KEY POINTS: • SWMR has the potential to reliably identify sub-coracoacromial spurs without radiation exposure. • SWMR provides comparable detection rates to conventional radiography for sub-coracoacromial spur formation. • SWMR yields higher detection rates compared to standard-MR regarding sub-coracoacromial spur formation. • SWMR can be implemented in routine shoulder MRI protocols.

Molecular imaging of the extracellular matrix in the context of atherosclerosis.

This review summarizes the current status of molecular imaging of the extracellular matrix (ECM) in the context of atherosclerosis. Apart from cellular components, the ECM of the atherosclerotic plaque plays a relevant role during the initiation of atherosclerosis and its' subsequent progression. Important structural and signaling components of the ECM include elastin, collagen and fibrin. However, the ECM not only plays a structural role in the arterial wall but also interacts with different cell types and has important biological signaling functions. Molecular imaging of the ECM has emerged as a new diagnostic tool to characterize biological aspects of atherosclerotic plaques, which cannot be characterized by current clinically established imaging techniques, such as X-ray angiography. Different types of molecular probes can be detected in vivo by imaging modalities such as magnetic resonance imaging (MRI), positron emission tomography (PET) and single photon emission computed tomography (SPECT). The modality specific signaling component of the molecular probe provides information about its spatial location and local concentration. The successful introduction of molecular imaging into clinical practice and guidelines could open new pathways for an earlier detection of disease processes and a better understanding of the disease state on a biological level. Quantitative in vivo molecular parameters could also contribute to the development and evaluation of novel cardiovascular therapeutic interventions and the assessment of response to treatment.

Differentiation of Osteophytes and Disc Herniations in Spinal Radiculopathy Using Susceptibility-Weighted Magnetic Resonance Imaging.

OBJECTIVE: The aim of this study was to evaluate the diagnostic performance of susceptibility-weighted magnetic resonance imaging (SW-MRI) for the differentiation of osteophytes and disc herniations of the spine compared with that of conventional spine MR sequences and radiography. MATERIALS AND METHODS: This study was approved by the local ethics review board; written consent was obtained from all subjects. Eighty-one patients with suspected radiculopathy of the spine were included prospectively. Radiography, T1/T2, and SW-MRI of the cervical/lumbar spine were performed. As reference standard, 93 osteophytes (n = 48 patients) were identified on radiographs in combination with conventional T1/T2 images. One hundred fourteen posterior disc herniations (n = 60 patients) were identified on T1/T2 in combination with radiography excluding osteophytes. For this study, 2 observers independently assessed the presence of osteophytes and disc herniations on T1/T2 and SW-MRI, with radiographs excluded from the analysis. In a subgroup of patients (n = 19), additional computed tomography images were evaluated. Sensitivity, specificity, and interobserver agreement were calculated. RESULTS: Most osteophytes (n = 92 of 93) and disc herniations (n = 113 of 114) could be identified and differentiated on SW-MRI magnitude/phase images, if radiographs were excluded from analysis. Susceptibility-weighted magnetic resonance imaging achieved a sensitivity of 98.9% and specificity of 99.1% for the identification of osteophytes. Conventional T1/T2 spine MR sequences achieved a sensitivity and specificity of 68.6% and 86.5%, respectively, if radiographs were excluded from analysis. Regarding the size of osteophytes, SW-MRI showed a strong correlation with computed tomography (R = 0.96) and radiography (R = 0.95). In addition, SW-MRI achieved a higher interobserver agreement compared with conventional MR. CONCLUSIONS: Susceptibility-weighted magnetic resonance imaging enables the reliable differentiation of osteophytes and disc herniations in patients with spinal radiculopathy with a higher sensitivity and specificity compared with conventional T1/T2 MR sequences.

Segment-by-segment assessment of left ventricular myocardial affection in Anderson-Fabry disease by non-enhanced T1-mapping.

Background Anderson-Fabry disease (AFD) is an X-linked lysosomal enzyme disorder associated with an intracellular accumulation of sphingolipids, which shorten myocardial T1 relaxation times. Myocardial affection, however, varies between different segments. Purpose To evaluate the specific segmental distribution and degree of segmental affection in AFD patients. Material and Methods Twenty-five patients with AFD, 14 patients with hypertrophic cardiomyopathy (HCM), and 21 controls were included. A Modified Look-Locker Inversion Recovery sequence (MOLLI) was used for non-enhanced T1 mapping at 1.5 T in addition to standard cardiac imaging in 10-12 short axis views. T1 values were evaluated with a mixed model ANOVA and regression analysis to determine the best diagnostic cutoff values for T1 for each myocardial segment. Results Regression analysis showed the best diagnostic cutoff compared to controls in cardiac segments 1-4, 8-9, and 14. Mean differences between T1 for AFD versus HCM were greatest in segment 3, 4, and 9 (99 ms, 103 ms, 86 ms, respectively). Overall T1 times were 888 ± 70 ms and 903 ± 14 ms (AFD with and without LVH); 1014 ± 17 ms and 1001 ± 22 ms (HCM and controls, P < 0.05). Conclusion Myocardial segments are affected by a varying degree of T1 shortening in AFD patients. Segment-specific cutoff values allow the most specific detection and quantification of the extent of myocardial affection.

Positive Outcome After a Small-Caliber Gunshot Fracture of the Upper Cervical Spine without Neurovascular Damage.

BACKGROUND: Gunshot wounds to the cervical spine most frequently concur with serious injuries to the spinal cord and cervical vessels and often have a fatal outcome. CASE REPORT: We describe the case of a 35-year-old male with a complex fracture of the C2 vertebra body and a mandibular fracture after a penetration gunshot to the cervical spine. Computed tomography (CT) at admission revealed the exact extent of the fractures and the small caliber bullet lodged next to the C2 vertebra. In this rare and extremely lucky case no collateral vascular or neurological damage was detected. Eighteen months after surgical bullet removal and posterior C1-C3 fusion complete bone healing of the C2 vertebra was achieved and there were no secondary neurovascular deficits. CONCLUSIONS: Immediate surgical C1-C3 fixation resulted in an excellent outcome without secondary neurovascular deficits in this rare case of traumatic complex C2 vertebral fracture caused by a gunshot injury.

Diagnosis of Calcific Tendonitis of the Rotator Cuff by Using Susceptibility-weighted MR Imaging.

PURPOSE: To evaluate the diagnostic performance of susceptibility-weighted imaging (SWI) and standard shoulder joint magnetic resonance (MR) sequences in comparison to that of conventional radiography for the identification of calcifications in the rotator cuff in patients with calcific tendonitis. MATERIALS AND METHODS: The institutional review board approved this prospective study. Written informed consent was obtained from all subjects. Fifty-four patients clinically suspected of having calcific tendonitis of the rotator cuff were included. On radiographs (the standard of reference), 27 patients had positive calcification findings, and 27 did not. Standard MR sequences and SWI, including magnitude and phase imaging, were performed. The diameter of calcifications was measured to assess intermodality correlations. Sensitivity, specificity, and intra- and interobserver agreement were calculated. Phantom measurements were performed to assess the detection limit of SWI. RESULTS: Fifty-six calcifications were detected with radiography in 27 patients. Most (55 calcifications, 98%) could be identified as calcifications by using SWI. Standard T1- and T2-weighted sequences were used to identify 33 calcifications (59%). SWI yielded a sensitivity of 98% (95% confidence interval [CI]: 0.943, 1) and specificity of 96% (95% CI: 0.886, 1) for the identification of calcifications when compared with radiography. Standard rotator cuff MR sequences yielded a sensitivity of 59% (95% CI: 0.422, 0.758) and specificity of 67% (95% CI: 0.493, 0.847). Diameter measurements demonstrated a high correlation between SWI and radiography (R(2) = 0.90), with overestimation of lesion diameter at SWI (mean ± standard deviation for SWI, 7.6 mm ± 5.4; for radiography, 5.3 mm ± 5.1). SWI yielded higher interobserver agreement (R(2) = 0.99, P < .001; 95% CI: 0.989, 0.996) compared with standard MR sequences (R(2) = 0.67, P = .62; 95% CI: 0.703, 0.899). In phantom experiments, SWI and computed tomography were used to identify small calcifications that were missed at radiography. CONCLUSION: SWI enables the reliable detection of calcifications in the rotator cuff in patients with calcific tendonitis by using conventional radiography as a reference and offers better sensitivity and specificity than standard rotator cuff MR sequences.

Cardiovascular Magnetic Resonance Imaging in Patients with an Implantable Loop Recorder.

BACKGROUND: Implantable loop recorders (ILRs) allow continuous cardiac monitoring for 3-6 years and are a valuable tool for the investigation of syncopal episodes, palpitations, and atrial fibrillations as well as risk stratification after myocardial infarction. Although magnetic resonance imaging (MRI) in patients with ILRs has been shown to be safe, the impact of ILRs on cardiac MRI image quality has not been investigated yet. Thus, we tested the diagnostic value of cardiac MRI in patients with various types of ILRs. METHODS: Two patients with an ILR and a clinical indication to assess myocardial burden of scarring and fibrosis or stress-induced myocardial ischemia underwent cardiac MRI. Device interrogation was performed prior to, immediately after, and 3 months after cardiac MRI. RESULTS: The post-MRI follow-ups revealed no change in programmed ILR parameters, sensing fidelity, and battery parameters. However, ILRs caused significant, uninterpretable hyperintensity artifacts in cardiac MRI. CONCLUSIONS: Further clinical studies are warranted to investigate whether modified MRI techniques are helpful to eliminate imaging artifacts.

Structural and functional cardiac analyses using modern and sensitive myocardial techniques in adult Pompe disease.

The purpose of this study was to analyze comprehensively the heart using modern and sensitive myocardial techniques in order to determine if structural or functional cardiac alterations are present in adult Pompe disease. Twelve patients with adult Pompe disease and a control group of 187 healthy subjects of similar age and gender were included. Structural and functional cardiac characteristics were analyzed by conventional and 2D speckle-tracking echocardiography. In addition, in a subgroup of adult Pompe patients, we analyzed the myocardial and musculoskeletal features by means of cardiac and whole-body muscle magnetic resonance imaging. Patients with Pompe disease had significant structural and functional musculoskeletal alterations such as atrophy with fatty replacement and weakness in trunk and extremities. In contrast, Pompe patients had similar structural and functional myocardial features to healthy subjects (LV strain -20.7 ± 1.9 vs. -21.3 ± 2.1%; RV strain -24.2 ± 5.3 vs. -24.8 ± 3.8%; LA strain 41.5 ± 10.3 vs. 44.8 ± 11.0%; P > 0.05; and no evidence of LV and RV hypertrophy or LA enlargement). In addition, there was no evidence of valvular cardiac alterations, electrocardiographic abnormalities, or myocardial fibrosis in Pompe patients. In the current study analyzing the heart with modern and sensitive myocardial techniques, we evidenced that functional and structural cardiac alterations are not present when Pompe disease begins in adulthood. Therefore, these findings suggest that adult Pompe disease should not be taken into consideration in the differential diagnostic of structural or functional cardiac disorders.

Gd-EOB enhanced MRI T1-weighted 3D-GRE with and without elevated flip angle modulation for threshold-based liver volume segmentation.

BACKGROUND: Despite novel software solutions, liver volume segmentation is still a time-consuming procedure and often requires further manual optimization. With the high signal intensity of the liver parenchyma in Gd-EOB enhanced magnetic resonance imaging (MRI), liver volume segmentation may be improved. PURPOSE: To evaluate the practicability of threshold-based segmentation of the liver volume using Gd-EOB-enhanced MRI including a customized three-dimensional (3D) sequence. MATERIAL AND METHODS: A total of 20 patients examined with Gd-EOB MRI (hepatobiliary phase T1-weighted (T1W) 3D sequence [VIBE]; flip angle [FA], 10° and 30°) were enrolled in this retrospective study. The datasets were independently processed by two blinded observers (O1 and O2) in two ways: manual (man) and threshold-based (thresh; study method) segmentation of the liver each followed by an optimization step (man+opt and thresh+opt; man+opt [FA10°] served as reference method). Resulting liver volumes and segmentation times were compared. A liver conversion factor was calculated in percent, describing the non-hepatocellular fraction of the total liver volume, i.e. bile ducts and vessels. RESULTS: Thresh+opt (FA10°) was significantly faster compared to the reference method leading to a median volume overestimation of 4%/8% (P < 0.001). Using thresh+opt (FA30°), segmentation was even faster (P < 0.001) and even reduced median volume deviation of 0%/2% (O1/O2; both P > 0.2). The liver conversion factor was found to be 10%. CONCLUSION: Threshold-based liver segmentation employing Gd-EOB-enhanced hepatobiliary phase standard T1W 3D sequence is accurate and time-saving. The performance of this approach can be further improved by increasing the FA.

Global cardiac alterations detected by speckle-tracking echocardiography in Fabry disease: left ventricular, right ventricular, and left atrial dysfunction are common and linked to worse symptomatic status.

The aim of this study was to test the hypothesis that in patients with Fabry disease, 2D speckle-tracking echocardiography (2DSTE) could detect functional myocardial alterations such as left ventricular (LV), right ventricular (RV), and left atrial (LA) dysfunction, even when conventional cardiac measurements are normal. In addition, we hypothesized that these global cardiac alterations could be linked to worse symptomatic status in these patients. Fifty patients with Fabry disease and a control group of 118 healthy subjects of similar age and gender were included. The myocardial function and structural changes of the LV, RV, and LA were analyzed by 2DSTE and cardiac magnetic resonance imaging. Patients with Fabry disease had significantly lower functional myocardial values of the LV, RV, and LA than healthy subjects (LV, RV, and LA strain -18.1 ± 4.0, -21.4 ± 4.9, and 29.7 ± 9.9 % vs. -21.6 ± 2.2, -25.2 ± 4.0, and 44.8 ± 11.1 %, respectively, P < 0.001) and elevated rates of LV, RV, and LA myocardial dysfunction (24, 20, and 26 %, respectively), even when conventional cardiac measurements such as LVEF, TAPSE, and LAVI were normal. LV septal wall thickness ≥15 mm, RV free wall thickness ≥7 mm, and LV longitudinal dysfunction were the principal factors linked to reduced LV, RV, and LA strain, respectively. In addition, but to a lesser extent, LV and RV fibrosis were linked to reduced LV and RV strain. Patients with reduced LV, RV, and LA strain had worse functional class (dyspnea-NYHA classification) than those with normal cardiac function. In conclusion, in patients with Fabry disease, 2DSTE analyses detect LV, RV, and LA functional myocardial alterations, even when conventional cardiac measurements are normal. These functional myocardial alterations are common and significantly associated with worse symptomatic status in Fabry patients. Therefore, these findings provide important evidence to introduce global myocardial analyses using 2DSTE in the early detection of functional cardiac alterations in Fabry disease.

Implications of imaging criteria for the management and treatment of intraductal papillary mucinous neoplasms - benign versus malignant findings.

OBJECTIVES: Evaluation of computed tomography (CT) and magnetic resonance imaging (MRI) for differentiation of pancreatic intraductal papillary mucinous neoplasm (IPMN) subtypes based on objective imaging criteria. METHODS: Fifty-eight patients with 60 histologically confirmed IPMNs were included in this retrospective study. Eighty-three imaging studies (CT,n = 42; MRI,n = 41) were analysed by three independent blinded observers (O1-O3), using established imaging criteria to assess likelihood of malignancy (-5, very likely benign; 5, very likely malignant) and histological subtype (i.e., low-grade (LGD), moderate-grade (MGD), high-grade dysplasia (HGD), early invasive carcinoma (IPMC), solid carcinoma (CA) arising from IPMN). RESULTS: Forty-one benign (LGD IPMN,n = 20; MGD IPMN,n = 21) and 19 malignant (HGD IPMN,n = 3; IPMC,n = 6; solid CA,n = 10) IPMNs located in the main duct (n = 6), branch duct (n = 37), or both (n = 17) were evaluated. Overall accuracy of differentiation between benign and malignant IPMNs was 86/92 % (CT/MRI). Exclusion of overtly malignant cases (solid CA) resulted in overall accuracy of 83/90 % (CT/MRI). The presence of mural nodules and ductal lesion size ≥30 mm were significant indicators of malignancy (p = 0.02 and p < 0.001, respectively). CONCLUSIONS: Invasive IPMN can be identified with high confidence and sensitivity using CT and MRI. The diagnostic problem that remains is the accurate radiological differentiation of premalignant and non-invasive subtypes. KEY POINTS: • CT and MRI can differentiate benign from malignant forms of IPMN. • Identifying (pre)malignant histological IPMN subtypes by CT and MRI is difficult. • Overall, diagnostic performance with MRI was slightly (not significantly) superior to CT.