Comparison of classical Fabry and its p.D313Y and p.A143T variants by cardiac T1 mapping, LGE and feature tracking myocardial strain.

Cardiac manifestation of classical Fabry disease (cFD) varies with sex and presence of left ventricular hypertrophy. p.D313Y/p.A143T variants (vFD) represent milder late-onset phenotypes, however, data on vFD are scarce. Patients with FD (cFD = 37;vFD = 14) and 14 healthy controls underwent 1.5 T CMR including Cine, LGE, native T1 mapping(nT1) and myocardial strain(CMR-FT). CMR-FT was assessed using ventricular longitudinal, circumferential, radial (LV-GLS/RV-GLS, LV-GCS/LV-GRS), and atrial longitudinal strain (LA/RATotal, LA/RAConduit, LA/RABooster). In cFD reduced myocardial strain (LV-GLS: -20 ± 4 vs. -24 ± 3%,p = 0.007; LV-GCS: -20 ± 4 vs. -26 ± 4%,p = 0.002, LA Total -GLS: 29 ± 10 vs. 37 ± 6%,p = 0.007; LA Conduit -GLS: 15 ± 10 vs. 23 ± 5%,p = 0.003) and nT1 values (951 ± 51 ms vs. 1036 ± 20 ms, p < 0.001) were observed compared to controls. In vFD findings were comparable to controls. LV-GCS provided the closest Area under the curve (AUC) to nT1 (0.84 vs. 0.92, p > 0.05) for discrimination of cFD versus controls. Significantly lower LV-GLS/LV-GCS was found in male compared to female cFD (-19 ± 4 vs. -22 ± 4%, p = 0.03). In six non-hypertrophied female cFD with normal nT1 LATotal -GLS was the only discriminating parameter with an accuracy of 86%. LV-GLS, LV-GCS and LATotal -GLS can detect impaired cardiac mechanics of cFD besides nT1. LATotal -GLS might identify non-hypertrophied female cFD. Variants p.D313Y/p.A143T did not reveal cardiac involvement by multiparametric CMR.

Prevalence and pattern of focal and potential diffuse myocardial fibrosis in male and female marathon runners using contrast-enhanced cardiac magnetic resonance.

OBJECTIVES: This study analyzed the prevalence and pattern of focal and potential diffuse myocardial fibrosis detected by late gadolinium enhancement (LGE) and extracellular volume (ECV) imaging in male and female marathon runners using cardiac magnetic resonance (CMR). METHODS: Seventy-four marathon runners were studied including 55 males (44 ± 8 years) and 19 females (36 ± 7 years) and compared to 36 controls with similar age and sex using contrast-enhanced CMR, exercise testing, and blood samples. RESULTS: Contrast-enhanced CMR revealed focal myocardial fibrosis in 8 of 74 runners (11%). The majority of runners were male (7 of 8, 88%). LGE was typically non-ischemic in 7 of 8 runners (88%) and ischemic in one runner. ECV was higher in remote myocardium without LGE in male runners (25.5 ± 2.3%) compared to male controls (24.0 ± 3.0%, p < 0.05), indicating the potential presence of diffuse myocardial fibrosis. LV mass was higher in LGE + males (86 ± 18 g/m2) compared to LGE- males (73 ± 14 g/m2, p < 0.05). Furthermore, LGE + males had lower weight (69 ± 9 vs 77 ± 9 kg, p < 0.05) and shorter best marathon finishing times (3.2 ± 0.3 h) compared to LGE- males (3.6 ± 0.4 h, p < 0.05) suggesting higher training load in these runners to accomplish the marathon in a short time. CONCLUSION: The high frequency of non-ischemic myocardial fibrosis in LGE + male runners can be related to increased LV mass in these runners. Furthermore, a higher training load could explain the higher LV mass and could be one additional cofactor in the genesis of myocardial fibrosis in marathon runners. KEY POINTS: • A high frequency of myocardial fibrosis was found in marathon runners. • Myocardial fibrosis occurred typically in male runners and was typically non-ischemic. • Higher training load could be one cofactor in the genesis of myocardial fibrosis in marathon runners.

Pre-interventional assessment of right renal to right adrenal vein distance: Impact on procedure time and radiation dose in adrenal vein sampling.

PURPOSE: Adrenal vein sampling (AVS) is the reference standard for evaluation of lateralized hormone production in primary aldosteronism. We aimed to investigate the impact of pre-interventional right renal vein (RRV) to right adrenal vein (RAV) distance measurement on fluoroscopy time, contrast agent exposure and radiation dose during AVS. MATERIALS AND METHODS: Forty-five patients with primary aldosteronism undergoing AVS were enrolled in our retrospective study and divided into three groups. In the group "ruler" (n = 14), RRV-RAV-distances were determined pre-interventionally by cross-sectional imaging (CT/MRI) and AVS was performed by one interventional radiologist with limited experience in AVS. CT/MRI-derived and fluoroscopy-derived RRV-RAV-distances were correlated for aimed cannulation of the RAV. Patients in group "no ruler" (n = 24, three interventional radiologists with limited experience in AVS) and in group "expert", (n = 7, one expert interventional radiologist) underwent AVS without pre-interventional estimation of RRV-RAV-distances. Procedure parameters (fluoroscopy time, contrast agent volume, radiation dose) of group "ruler" were compared to both other groups by Kruskal-Wallis rank-sum test. RESULTS: Correlation of CT/MRI-derived and fluoroscopy-derived RRV-RAV-distances was good (r = 0.74;p = 0.003). The median RRV-RAV-distance was 4.5cm at CT/MRI (95%-CI:4.2-5.0cm) and 4.0cm at fluoroscopy (95%-CI:3.8-4.5cm). Fluoroscopy time (p<0.0001), contrast agent exposure (p = 0.0003) and radiation dose (air kerma and dose area product both p = 0.038) were significantly lower in group "ruler" compared to group "no ruler" (all p<0.05), and similar to group "expert" (all p>0.05). CONCLUSIONS: CT/MRI-derived pre-interventional renal-adrenal vein distance measurements correlate well with angiographic distance measurements. Pre-interventional estimation of the RRV-RAV-distance allows for aimed cannulation of the RAV with potential reduction of fluoroscopy time, contrast agent exposure and radiation-dose during AVS.

Impact of Sex and Cardiovascular Risk Factors on Myocardial T1, Extracellular Volume Fraction, and T2 at 3 Tesla: Results From the Population-Based, Hamburg City Health Study.

BACKGROUND: Reliable reference intervals are crucial for clinical application of myocardial T1 and T2 mapping cardiovascular magnetic resonance imaging. This study evaluated the impact of sex and cardiovascular risk factors on myocardial T1, extracellular volume fraction (ECV), and T2 at 3T in the population-based HCHS (Hamburg City Health Study). METHODS: The final study sample consisted of 1576 consecutive HCHS participants between 46 and 78 years without prevalent heart disease, including 1020 (67.3%) participants with hypertension and 110 (7.5%) with diabetes. T1 and T2 mapping were performed on a 3T scanner using 5b(3b)3b modified Look-Locker inversion recovery and T2 prepared, fast-low-angle shot sequence, respectively. Stepwise regression analyses were performed to identify variables with an independent impact on T1, ECV, and T2. Reference intervals were defined as the interval between the 2.5% and 97.5% quantiles. RESULTS: Sex was the major independent influencing factor of myocardial native T1, ECV, and T2. Female patients had significantly higher upper limits of reference intervals for native T1 (1112-1261 versus 1079-1241 ms), ECV (23%-33% versus 22%-32%), and T2 (36-46 versus 35-45 ms) compared with male patients (all P<0.001). Cardiovascular risk factors, such as diabetes and hypertension, did not systematically affect native T1. There was an independent association of T2 by hypertension and, to a lesser degree, by left ventricular mass, heart rate (all P<0.001), and body mass index (P=0.001). CONCLUSIONS: Sex needs to be considered as the major, independent influencing factor for clinical application of myocardial T1, ECV, and T2 measurements. Consequently, sex-specific reference intervals should be used in clinical routine. Our findings suggest that there is no need for specific reference intervals for myocardial T1 and ECV measurements in individuals with cardiovascular risk factors. However, hypertension should be considered as an additional factor for clinical application of T2 measurements. REGISTRATION: URL: https://www. CLINICALTRIALS: gov; Unique identifier: NCT03934957.

Radiation dose reduction during adrenal vein sampling using a new angiographic imaging technology.

To compare the patient radiation doses during angiographic selective adrenal vein sampling (AVS) before and after an imaging technology upgrade. In this retrospective single-center-study, cumulative air kerma (AK), cumulative dose area product (DAP), fluoroscopy time and contrast agent dosage were recorded from 70 patients during AVS. 35 procedures were performed before and 35 after an imaging processing technology upgrade. Mean values were calculated and compared using an unpaired student's t-test. DSA image quality was assessed independently by two blinded readers using a four-point Likert scale (1 = poor; 4 = excellent) and compared using Wilcoxon signed-rank test. After the technology upgrade we observed a significant reduction of 35% in AK (1.7 ± 0.7 vs. 1.1 ± 0.7 Gy, p = 0.01) and a significant reduction of 28% in DAP (235.1 ± 113 vs. 170.1 ± 94 Gy*cm2, p = 0.01) in comparison to procedures before the upgrade. There were no significant differences between the number of exposure frames (143 ± 86 vs. 132 ± 61 frames, p = 0.53), fluoroscopy time (42 ± 23 vs. 36 ± 18 min, p = 0.22), or the amount of contrast medium used (179.5 ± 84 vs. 198.1 ± 109 ml, p = 0.41). There was also no significant difference regarding image quality (3 (2-4) vs. 3 (2-4), p = 0.67). The angiographic imaging technology upgrade significantly decreases the radiation dose during adrenal vein sampling without increasing time of fluoroscopy or contrast volume and without compromising image quality.

Nutritional Status Impacts Quality of Life in Head and Neck Cancer Patients Undergoing (Chemo)Radiotherapy: Results from the Prospective HEADNUT Trial.

Malnutrition negatively impacts quality of life (QoL) in patients with head and neck cancer (HNC). This is the first prospective study to assess the impact of malnutrition (defined by the bioelectrical impedance analysis (BIA)-derived fat-free mass index) on QoL in patients with HNC undergoing (chemo)radiotherapy. Between October 2018 and October 2020, 58 HNC patients prospectively completed the QoL-questionnaires EORTC-QLQ-C30 and EORTC-QLQ-H&N35 at the beginning (tb) and at the end of (chemo)radiotherapy (te) as well as during follow-up (tf). At these time points, nutritional risk assessment (MUST, NRS-2002, Nutriscore), BIA measurement and laboratory testing was performed by a permanent study team. Differences between malnourished (n = 14) and well-nourished patients (n = 44) were observed in UICC classification (P < 0.001) and HPV status (P = 0.03). Well-nourished patients showed higher baseline hemoglobin (P = 0.025) and albumin (P = 0.005), but lower c-reactive protein levels (P < 0.001). At tb, mostly malnourished patients presented with worse QoL. Multivariable analysis showed that MUST, NRS-2002, HPV status, and UICC classification were related to QoL. Nutritional status has a crucial impact on QoL. The nutritional screening protocols MUST and NRS-2002 are suitable for identifying patients at risk and predicting QoL in patients with HNC undergoing (chemo)radiotherapy.

Intraindividual comparison of 1.5 T and 3 T non-contrast MR angiography for monitoring of aortic root diameters in Marfan patients.

BACKGROUND: Reproducible aortic diameter measurements are crucial for assessment of aortic growth and aneurysm formation in patients with Marfan syndrome. The objective of this study was to perform an intraindividual comparison of aortic measurements at 1.5 T and 3 T using non-contrast magnetic resonance angiography (MRA) in pre-surgical and post-surgical Marfan patients. METHODS: Forty consecutive Marfan patients were retrospectively evaluated by ECG-gated 2D balanced steady-state free precession (bSSFP) MRA at 1.5 T and 3 T after 363 ± 58 days. 24 patients were before and 16 patients after aortic root surgery. Two readers independently measured aortic diameters at seven aortic levels and rated the image quality/image artifacts (1 = poor/severe, 4 = excellent/none). Contrast-to-noise ratio (CNR) and signal intensity slopes between aortic lumen and vessel walls were semiautomatically determined. RESULTS: In pre-surgical Marfan patients, interobserver agreement of aortic root diameter measurements was significantly higher at 3 T compared to 1.5 T (p < 0.05). In post-surgical Marfan patients, image quality and artifacts were significantly worse at 3 T compared to 1.5 T (p < 0.05). CNR was higher at 3 T compared to 1.5 T at all aortic levels. Significantly steeper slopes of signal intensity curves were observed at 3 T at all aortic levels (p < 0.001). CONCLUSIONS: In pre-surgical Marfan patients, non-contrast MRA provides higher reproducibility of aortic diameter measurements at 3 T compared to 1.5 T. In post-surgical Marfan patients, metallic implants result in significantly worse imaging artifacts and reduced image quality at 3 T compared to 1.5 T. Therefore, we propose to monitor the thoracic aorta with non-contrast MRA at 3 T in pre-surgical Marfan patients and at 1.5 T in post-surgical Marfan patients.

CMR feature tracking strain patterns and their association with circulating cardiac biomarkers in patients with hypertrophic cardiomyopathy.

AIMS: CMR feature tracking strain (CMR-FT) provides prognostic information. However, there is a paucity of data in hypertrophic cardiomyopathy (HCM). We sought to analyze global CMR-FT parameters in all four cardiac chambers and to assess associations with NT-proBNP and cardiac troponin T (hsTnT) in patients with HCM. METHODS: This retrospective study included 144 HCM patients and 16 healthy controls with CMR at 1.5 T. Analyses were performed on standard steady-state free precession cine (SSFP) CMR data using a commercially available software. Global left ventricular (LV) strain was assessed as longitudinal (LVLAX-GLS), circumferential (LVLAX-GCS) and radial strain (LVLAX-GRS) on long -axis (LAX) and as LVSAX-GCS and LVSAX-GRS on short- axis (SAX). Right ventricular (RV-GLS), left atrial (LA-GLS) and right atrial (RA-GLS) strain were assessed on LAX. RESULTS: We found LVLAX-GLS [- 18.9 (- 22.0, - 16.0), - 23.5 (- 25.5, - 22.0) %, p = 0.0001), LVSAX-GRS [86.8 (65.9-115.5), 119.6 (91.3-143.7) %, p = 0.001] and LALAX-GLS [LA2CH-GLS 29.2 (19.1-37.7), LA2CH-GLS 38.2 (34.3-47.1) %, p = 0.0036; LA4CH-GLS 22.4 (14.6-30.7) vs. LA4CH-GLS 33.4 (28.4-37.3) %, p = 0.0033] to be impaired in HCM compared to healthy controls despite normal LVEF. Furthermore, LV and LA strain parameters were impaired in HCM with elevated NT-proBNP and/or hsTnT, despite preserved LVEF compared to HCM with normal biomarker levels. There was a moderate correlation of LV and LA CMR-FT with levels of NT-proBNP and hsTnT. CONCLUSION: CMR-FT reveals LV and LA dysfunction in HCM despite normal LVEF. The association between impaired LV strain and elevated NT-proBNP and hsTnT indicates a link between unapparent functional abnormalities and disease severity in HCM. Typical CMR-FT findings in patients with hypertrophic cardiomyopathy.

Structural alteration of lung parenchyma in patients with NF1: a phenotyping study using multidetector computed tomography (MDCT).

BACKGROUND: Diffuse interstitial lung disease have been described in Neurofibromatosis type 1 (NF1), but its diversity and prevalence remain unknown. The aim of this study was to assess the prevalence and characteristics of (NF1)-associated lung manifestations in a large single-center study using multidetector computed tomography (MDCT) and to evaluate the smoking history, patients' age, genetics, and the presence of malignant peripheral nerve sheath tumors (MPNST) as potential influencing factors for lung pathologies. METHODS: In this retrospective study, 71 patients with NF1 were evaluated for the presence of distinctive lung manifestations like reticulations, consolidations, type of emphysema, pulmonary nodules and cysts. All patients underwent F-18-FDG PET/CT scans, which were reviewed by two experienced radiologists in consensus. Patients' subgroups were formed based on their smoking history (current smokers/previous smokers/never smokers), age (< 12 years, 12-18 years, > 18 years), and presence of MPNST (MPNST/no MPNST). In 57 patients (80%), genetic analysis of sequences coding for the neurofibromin on chromosome 17 was performed, which was correlated with different lung pathologies. RESULTS: Among all NF1 patients (33 ± 14 years, 56% females), 17 patients (24%) were current smokers and 62 patients (87%) were > 18 years old. Pulmonary cysts, nodules, and paraseptal emphysema were the most common pulmonary findings (35%, 32%, 30%). The presence of pulmonary metastases, MPNST and centrilobular emphysema was associated with smoking. Cysts were observed only in adults, whereas no significant correlation between age and all other pulmonary findings was found (p > 0.05). Presence of MPNST was accompanied by higher rates of intrapulmonary nodules and pulmonary metastasis. Neither the presence nor absence of any of the specific gene mutations was associated with any particular lung pathology (p > 0.05). CONCLUSIONS: All pulmonary findings in NF1 patients occurred independently from specific mutation subtypes, suggesting that many NF1 mutations can cause various pulmonary pathologies. The presence of pulmonary metastases, MPNST and centrilobular emphysema was associated with smoking, indicating the value of smoking secession or the advice not to start smoking in NF1 patients as preventive strategy for clinicians. For screening of pulmonary manifestations in NF1 patients, an MDCT besides medical history and physical examination is mandatory in clinical routine.

Prognosis of early pre-discharge and late left ventricular dilatation by cardiac magnetic resonance imaging after acute myocardial infarction.

To study the long-term prognosis of early pre-discharge and late left ventricular (LV) dilatation in patients with first ST-elevation myocardial infarction (STEMI) treated by percutaneous coronary intervention (PCI) and contemporary medical therapy. Long-term follow-up > 15 years was available in 53 consecutive patients (55 ± 13 years) with first STEMI. Late gadolinium enhanced (LGE) cardiac magnetic resonance imaging (CMR) was obtained at baseline 5 ± 3 days and follow-up 8 ± 3 months after STEMI to measure LV function, volumes and infarct size. Early pre-discharge dilatation was defined as increased left ventricular end-diastolic volume index (LVEDVi) at baseline CMR with > 97 ml/m2 for males and > 90 ml/m2 for females. Late dilatation was defined as initially normal LVEDVi, which increased ≥ 20% at follow-up. Early dilatation was present in 7 patients (13%), whereas late dilatation occurred in 11 patients (21%). Patients with early LV dilatation had highest mortality (57%), whereas patients with late dilatation had similar mortality (27%) compared to patients without dilatation (26%). Multivariate Cox analysis showed that age (P < 0.001), ejection fraction at baseline (P < 0.01) and early dilatation (P < 0.01) were independent predictors of death. Early dilatation qualified as an exclusive independent predictor of long-term mortality after adjustment for age and ejection fraction (P < 0.05, hazard ratio: 2.2, 95% confidence interval: 1.2 to 7.9). Early pre-discharge LV dilatation by CMR enabled strong long-term risk stratification after STEMI. The high mortality of early LV dilatation underscores the clinical importance of this post-infarction complication, which occurred despite PCI and contemporary medical therapy.

Efficiency of Percutaneous Stent Angioplasty in Renal Artery Stenosis - 15 Years of Experience at a Single Center.

PURPOSE: To determine the therapeutic efficiency of percutaneous revascularization in renal artery stenosis (RAS), as well as the role of comprehensive factors such as patient selection and degree of artery stenosis, on clinical outcome. METHODS AND MATERIALS: 101 patients with hemodynamically relevant RAS underwent percutaneous angioplasty (PTA). 65.7 % were male (mean age: 64 years; range: 18-84). The clinical data was retrospectively analyzed. The serum creatinine (Cr), glomerular filtration rate (GFR), and blood pressure (BP) levels pre- and postprocedural, between 6 months and 1 year, were retrospectively collected and statistically analyzed. RESULTS: Follow-up data was available in 34 (33.7 %) and 28 patients (27.7 %) for Cr and MAP, respectively. A significant drop in mean arterial pressure (MAP) was observed on follow-up (mean -5.27 mmHg). Higher baseline Cr and MAP values showed a more pronounced drop in the follow-up (Cr: p 0.002; difference to baseline -0.25 mg/dL, 95 %CI:-0.36, -0.07 and BP p < 0.001; diff. to baseline -0.72 mmHg; 95 %CI: -1.4, -0.40). There was no association between comorbidities, gender, and degree of stenosis with renal and BP outcome. No significant improvement in renal function was observed on follow-up (mean Cr drop: -0.015 mg/dL). The age group 51-60 years showed a significant improvement in BP (p 0.030; diff. to baseline -19.2 mmHg; 95 %CI: -34, -4.3). There was a slight reduction in antihypertensive medication following angioplasty (0.2 fewer). Minor complications were recorded in five procedures (4.9 %). CONCLUSION: Percutaneous renal artery revascularization in the presence of atherosclerotic RAS is a safe procedure associated with a significant drop in post-procedural BP. No significant improvement in renal function was observed. Further prospective studies focused on patient selection are necessary. KEY POINTS: · Percutaneous stent angioplasty in renal artery stenosis is associated with a significant improvement in post-procedural blood pressure control.. · There is no improvement in renal function after percutaneous stent angioplasty for renal artery stenosis (RAS).. · Percutaneous stent angioplasty is a safe procedure.. CITATION FORMAT: · Guerreiro H, Avanesov M, Dinnies S et al. Efficiency of Percutaneous Stent Angioplasty in Renal Artery Stenosis - 15 Years of Experience at a Single Center. Fortschr Röntgenstr 2021; 193: 298 - 304.

Reliability of non-contrast magnetic resonance angiography-derived aortic diameters in Marfan patients: comparison of inner vs. outer vessel wall measurements.

Aortic diameter measurements play a crucial role for the indication of aortic root surgery in Marfan patients. However, for magnetic resonance angiography (MRA)-derived measurements, there is no consensus on whether the aortic wall should be included or excluded in the aortic diameter. The purpose of this retrospective study was to compare the reliability of non-contrast bright blood MRA aortic inner-to-inner and outer-to-outer edge measurements in patients with Marfan syndrome. Forty Marfan patients underwent ECG-gated balanced steady-state free-precession MRA of the aorta at 1.5 T. Two readers independently performed inner and outer measurements at different aortic levels. They rated the image quality of the delineation of both inner and outer vessel wall edges on a four-point scale. MRA-derived diameters of the sinuses of Valsalva were compared with echocardiography-derived diameters. Aortic vessel wall delineation score was rated higher at all levels for inner than for outer vessel walls (p < 0.001). Inter- and intraobserver variances of aortic measurements were smaller for inner-to-inner measurements at the sinuses of Valsalva, sinotubular junction and ascending aorta (p < 0.03). There was a difference of 1.1 ± 2.3 mm for inner MRA measurements (p = 0.014) and 6.9 ± 3.1 mm for outer MRA measurements (p < 0.001) when compared to echocardiographic leading-edge measurements. Inner-to-inner vessel wall diameter measurements in non-contrast bright blood MRA provide more reliable diameters when compared to outer-to-outer vessel wall measurements of the aortic root. Therefore, we propose to rely on inner rather than outer aortic wall measurements in non-contrast-MRA when monitoring aortic diameters in patients with Marfan syndrome.

MDCT in the Setting of Suspected Colonic Diverticulitis: Prevalence and Diagnostic Yield for Diverticulitis and Alternative Diagnoses.

OBJECTIVE. The purpose of this study was to determine the prevalence and demographic distribution of colonic diverticulitis (CD) and alternative diagnoses (AD), as well as the diagnostic accuracy of MDCT in patients with suspected CD. MATERIALS AND METHODS. This study retrospectively included 1069 patients (560 women) undergoing MDCT for the evaluation of suspected CD. The prevalence of CD and AD was determined and the diagnostic accuracy of MDCT calculated. The final clinical diagnosis derived from the discharge report served as the standard of reference. Prevalence of diagnoses by age, sex, and admission status were compared using Cochran-Armitage, chi-square, and Fisher exact tests. RESULTS. Prevalence of CD was 52.5% (561/1069) and of AD was 39.9% (427/1069). In the remaining 7.6% (81/1069) no final clinical diagnosis was established. The most frequent AD were appendicitis (12.6%, 54/427), infectious colitis (10.5%, 45/427), infectious gastroenteritis (8.2%, 35/427), urolithiasis (6.1%, 26/427), and pyelonephritis (4.9%, 21/427). The prevalence of diverticulitis and AD varied statistically significantly according to both age (p < 0.001) and admission status (p < 0.001). Also, the prevalence of the 10 most frequent specific AD varied statistically significantly according to sex (p = 0.022). CT had a sensitivity and specificity of 99.1% and 99.8% for diagnosing CD and 92.7% and 98.8% for AD, respectively. CONCLUSION. In about 40% of patients with suspected diverticulitis a broad spectrum of AD is causative for symptoms. MDCT provides high diagnostic accuracy in the diagnosis of diverticulitis and AD. The prevalence of diagnoses is related to admission status and demographic data; in particular age-related AD have to be considered in patients with clinically suspected diverticulitis.

The effect of pregnancy on growth-dynamics of neurofibromas in Neurofibromatosis type 1.

INTRODUCTION: Patients with Neurofibromatosis type 1 (NF1) develop plexiform neurofibromas (PNF) and cutaneous neurofibromas. These tumors are a major cause of the patient's morbidity and mortality. An influence of estrogen and progesterone on tumor growth has been suggested but reports on growth or malignant transformation of tumors during pregnancy remain anecdotal. The purpose of this study was to quantify growth of cutaneous and plexiform neurofibromas in NF1 patients during pregnancy, and to assess the onset of NF1 related symptoms. MATERIAL AND METHODS: Retrospectively, 13 mothers with NF1 were included and compared to nullipara, nulligravida, age-matched women with NF1. All women received whole-body magnetic resonance imaging (MRI) before and after pregnancy or after a matched time period. Presence of plexiform and cutaneous neurofibromas was evaluated. PNF were subjected to semi-automated volumetry (MedX). The sum of the longest diameters (SLD) of representative cutaneous neurofibromas was determined for both groups. Clinical symptoms and subjective tumor growth were assessed. RESULTS: PNF were identified in 12/26 women (46.2%). Follow up showed neither new PNF nor a significant difference in growth rate (median tumor-growth/year: pregnant group-0.38% (IQR -1.1-5.4%) vs control group 3.59% (IQR -2.1-5.5%; P = 0.69). Malignant transformation of PNF was not observed. There was a significant growth of cutaneous neurofibromas in both groups (median SLD increase: pregnant group 17mm; P = 0.0026 / control group 12mm; P = 0.0004) The difference in increase of SLD was not significant (P = 0.48). Singular cutaneous neurofibromas in the pregnant group displayed high levels of tumor growth (>20%/year). NF1-associated symptoms and subjective tumor growth were not significantly increased in pregnant patients. CONCLUSIONS: Growth of plexiform and cutaneous neurofibromas in pregnant patients is not significantly different compared to non-pregnant patients. Cutaneous neurofibromas show a significant increase in growth over time in both, pregnant and non-pregnant patients and NF1 related clinical symptoms do not significantly aggravate during the course of pregnancy.

Association of Asymmetric Dimethylarginine and Diastolic Dysfunction in Patients with Hypertrophic Cardiomyopathy.

Despite genetic heterogeneity, early manifestation of diastolic dysfunction (DD) is common in hypertrophic cardiomyopathy (HCM). Nitric oxide (NO) may contribute to myocardial relaxation. NO synthases (NOS) use l-arginine (Arg) as a substrate, as asymmetric dimethylarginine (ADMA) is a direct endogenous inhibitor of NOS. This study aimed to analyze the association of Arg and its derivates, i.e., l-homoarginine (hArg), ADMA and symmetric dimethylarginine (SDMA), with DD in HCM patients. In 215 HCM patients (mean age 54 ± 15 years, 58% male) transmitral and mitral annulus velocities were echocardiographically analyzed. Plasma concentrations of Arg derivatives were measured by liquid chromatography tandem-mass spectrometry. In 143 (70%) patients suffering from DD, ADMA showed the strongest association with DD (0.66 ± 0.16, 0.72 ± 0.24, and 0.76 ± 0.26 µmol/L, p < 0.01 for trend). In linear regression analyses, positive association per standard deviation increase of ADMA was found with E-wave (beta coefficient (95% confidence interval): 4.72 (0.43-9.01); p < 0.05) and mean E/E' (1.76 (0.73-2.79) p < 0.001). Associations were adjusted for age, sex, body mass index (BMI), diabetes mellitus, coronary artery disease, and arterial hypertension. Elevated ADMA is associated with the severity of DD in HCM. Higher ADMA level might lead to decreased NO production and thus an impaired myocardial relaxation pattern.

Effect of renal denervation procedure on left ventricular mass, myocardial strain and diastolic function by CMR on a 12-month follow-up.

PURPOSE: To investigate the effects of renal denervation (RDN) on left ventricular (LV) mass, myocardial strain and diastolic function in patients with treatment-resistant arterial hypertension by cardiac magnet resonance imaging on a 12-month follow-up. MATERIALS AND METHODS: Sixteen patients (38% female) were examined before and 12 months after RDN. LV morphology and strain were analyzed. Diastolic function was determined by early (EPFR) and atrial peak filling rates (APFR) derived from differential volume-time-curve analysis. Clinical visits included 24-h ambulant blood pressure monitoring (ABPM). RESULTS: Twelve months after RDN LV mass decreased from 80 ± 21 g/m2 to 74 ± 20 g/m2 (P < 0.05). Global radial (35 ± 12% vs. 41 ± 10%, P < 0.05) and longitudinal strain improved (- 15 ± 4% vs. - 17 ± 3%, P < 0.05). Global circumferential strain (- 16 ± 5% vs. - 18 ± 4%, P = 0.12) remained unchanged. The parameter of diastolic LV function PFRR (EPFR/APFR) improved following RDN (0.9 ± 0.4 vs. 1.1 ± 0.5, P < 0.05). Individual changes of LV mass were associated with an increase of EPFR (r = - 0.54, P < 0.05) and a reduction of APFR by trend (r = 0.45, P = 0.08). Systolic ABPM showed a decrease by trend (152 mmHg vs. 148 mmHg, P = 0.08). CONCLUSIONS: After RDN we observed a reduction of LV mass, improvement of global strain and diastolic function.

Increased urinary bladder volume improves the detectability of urinary stones at the ureterovesical junction in non-enhanced computed tomography (NECT).

OBJECTIVES: To evaluate the influence of the urinary bladder volume on the detectability of urolithiasis at the ureterovesical junction (UVJ) using a low-dose CT (LD-CT) with iterative reconstruction (IR) and a standard-dose CT (SD-CT) without IR in a large cohort. METHODS: Four hundred patients (278 males (69.5%), mean 44.6 ± 14.7 years) with urolithiasis at the UVJ were investigated either by an LD-CT with IR (n = 289, 72%) or an SD-CT without IR (n = 111, 28%) protocol. The detectability of distal urolithiasis was assessed by a dichotomous assessment (definite or questionable) by two radiologists in consensus and by a quantitative analysis of the signal density distribution across a line drawn parallel to the distal ureter. Based on the resulting graph, minimum/maximum density values and mean/maximum upslopes and downslopes were derived and calculated automatically. In all patients, the total bladder volume was calculated by a slice-by-slice approach on axial CT images. RESULTS: Patients with definite stones showed significantly higher urinary bladder volumes compared to patients with questionable stones in both LD-CT and SD-CT (p < 0.01). These results were independent of stones' length and patients' BMI values. Using cutoffs of 92 ml for LD-CT and 69 ml for SD-CT, high positive predictive values/accuracy rates of 96%/85% (LD-CT) and 98%/86% (SD-CT) were observed to identify definite urinary stones. CONCLUSIONS: Urinary bladder volume has a significant impact on the detectability of distal urolithiasis. Moderate bladder filling by pre-CT hydration with subsequent CT scan at the time of high urge to void increases the detectability of urinary stones at the UVJ in clinical routine. KEY POINTS: • Urinary bladder volume significantly affects the detectability of distal urolithiasis • Higher bladder volumes are associated with improved detectability of distal urinary stones • Oral pre-CT hydration for urolithiasis is easily applicable and cost-effective.

Impact of Myocardial Fibrosis on Left Ventricular Function Evaluated by Feature-Tracking Myocardial Strain Cardiac Magnetic Resonance in Competitive Male Triathletes With Normal Ejection Fraction.

BACKGROUND: To analyze the effect of myocardial fibrosis on left ventricular (LV) function evaluated by feature-tracking strain analysis by cine cardiac magnetic resonance (CMR) in competitive male triathletes with normal ejection fraction (EF).Methods and Results:78 asymptomatic male triathletes with >10 weekly training hours (43±11 years) and 28 male age-matched controls were studied by late gadolinium enhancement (LGE) and cine CMR. Global and segmental radial, longitudinal and circumferential strains were analyzed using feature-tracking cine CMR. Focal non-ischemic LGE was observed in 15 of 78 triathletes (19%, LGE+) with predominance in the basal inferolateral segments. LVEF was normal in LGE+ (62±6%) and LGE- triathletes (62±5%, P=0.958). In contrast, global radial strain was lower in LGE+ triathletes at 40±7% compared with LGE- triathletes (45±7%, P<0.05). Reduced segmental radial strain occurred either in LGE+ segments or in directly adjacent segments. Strain analysis revealed regional differences in controls, with the highest radial and longitudinal strain in the inferolateral segments, which were typically affected by fibrosis in LGE+ triathletes. CONCLUSIONS: Reduced global and regional radial strain suggests a negative effect of myocardial fibrosis on LV function in LGE+ triathletes with normal EF. The observed regional differences in controls with the highest radial and longitudinal strain in the inferolateral segments may explain the typical occurrence of fibrosis in this myocardial region in triathletes.

Stress T1-mapping cardiovascular magnetic resonance imaging and inducible myocardial ischemia.

BACKGROUND: Alterations in native myocardial T1 under vasodilation stress ("T1 reactivity") were recently proposed as a non-contrast cardiovascular magnetic resonance (CMR) method to detect myocardial ischemia. This study evaluated the performance of a segmental, truly non-contrast stress T1 mapping CMR approach to detect inducible ischemia. METHODS AND RESULTS: One-hundred patients with suspected/known coronary artery disease underwent CMR at 3.0 or 1.5 T. T1 mapping was performed using the 5s(3s)3s-modified look-locker inversion-recovery (MOLLI) sequence at rest and under regadenoson stress. We defined T1 reactivity as the change in native T1 from rest to stress (1) in the 16-segment AHA model independent from perfusion images and (2) in focal regions of interest that were copied from perfusion images to T1 maps. We compared T1 reactivity between segments/regions with inducible ischemia, scar, and remote myocardium for both approaches. Segmental T1 reactivity was significantly lower in segments including inducible ischemia [- 1.15 (95% CI, - 2.16 to - 0.14)%] compared to remote segments [2.49 (95% CI, 1.87 to 3.11)%; p < 0.001]. Focal T1 reactivity was also significantly lower [- 2.65 (95% CI, - 3.84 to - 1.46)%] in regions with stress-perfusion defects compared to remote regions [4.72 (95% CI, 3.90 to 5.54)%; p < 0.001]. However, the performance of segmental T1 reactivity to depict inducible ischemia was significantly inferior compared to the focal approach (AUCs 0.68 versus 0.85; p < 0.0001). CONCLUSIONS: Myocardium with inducible ischemia is characterized by the absence of significant T1 reactivity, but a clinically applicable approach for truly non-contrast stress T1 mapping remains to be determined.

Clinico-radiological comparison and short-term prognosis of single acute pancreatitis and recurrent acute pancreatitis including pancreatic volumetry.

PURPOSE: The necrosis-fibrosis hypothesis describes a continuum between single attacks of acute pancreatitis (SAP), recurrent acute pancreatitis (RAP) and chronic pancreatitis (CP) with endocrine and exocrine pancreatic insufficiency. For prevention purposes we evaluated clinico-radiological parameters and pancreatic volumetry to compare SAP and RAP and provide prognostic relevance on short-term mortality, need for intervention and the hospitalization duration. MATERIALS AND METHODS: We retrospectively investigated 225 consecutive patients (150 males, range 19-97years) with acute pancreatitis (74%SAP, 26%RAP) according to the revised Atlanta classification. All patients received an intravenous contrast-enhanced CT after a median time of 5 (IQR 5-7) days after onset of symptoms. Two experienced observers rated the severity of AP by 3 CT scores (CTSI, mCTSI, EPIC). Moreover, total pancreatic volumes and additional parenchymal necrosis volumes were assessed, when appropriate. Clinical parameters were etiology of AP, lipase on admission, CRP 48 hours after admission (CRP48), and the presence of organ dysfunction, assessed by the modified Marshall score. The modified Marshall score included systolic blood pressure, serum creatinine, and the ratio of arterial oxygen partial pressure to fractional inspired oxygen (PaO2/FiO2 ratio) and was assessed on admission and 48 hours after admission to find patients with persistent organ failure. Outcome parameters were total hospitalization duration, short-term mortality and need for intervention. RESULTS: Lipase, CRP48, etiology of AP, EPIC, PaO2/FiO2 ratio, and the presence of a pleural effusion differed significantly in both groups (p<0.05). In 109 patients with interstitial edematous AP, the total pancreatic volume was significantly smaller in patients with RAP compared to those with SAP (69±35cm3; (RAP) vs 106±45cm3; (SAP), p<0.001). All outcome parameters including the mortality rates (SAP vs. RAP: 15% vs. 7%) were comparable in both groups (p>0.05). In the necrotizing RAP group, only the necrotic volume correlated significantly with total hospitalization time (r = 0.72, p<0.001), whereas the systolic blood pressure was the only, but weak predictor for short-term mortality (ß-coefficient: -0.05, p = 0.03) and the need for intervention (ß-coefficient: -0.02, p = 0.048) in the total RAP group. In patients with SAP, the modified Marshall score was the strongest predictor of short-term mortality, followed by the mCTSI on multivariate logistic regression (Marshall score: ß-coefficient: 1.79, p<0.001; mCTSI: ß-coefficient: 0.40, p<0.001). CTSI was the best predictor for required intervention in necrotizing SAP (ß-coefficient: 0.46, p<0.001), followed by the volume of intrapancreatic necrosis (ß-coefficient: 0.17, p = 0.03). CONCLUSION: Total pancreatic volume differed significantly between interstitial RAP and SAP and intrapancreatic necrosis volume revealed prognostic value for the total hospitalization duration in necrotizing RAP. Although all outcome parameters were comparable between SAP and RAP, only systolic blood pressure and pancreatic volumetry were prognostic in RAP. In SAP, only the modified Marshall score and mCTSI revealed prognostic value for short-term mortality, whereas CTSI was predictive for the need for intervention.