Wall Enhancement Research Articles

CT evaluation of bowel wall enhancement in pneumatosis intestinalis: preventing non-therapeutic laparotomies.

To evaluate the diagnostic performance of bowel wall enhancement for diagnosing concomitant bowel ischemia in patients with parietal pneumatosis (PI) diagnosed at abdominal CT. From January 1, 2012 to December 31, 2021, 226 consecutive patients who presented with PI on abdominal CT from any bowel segment were included. Variables at the time of the CT were retrospectively extracted from medical charts. CT examinations were blindly analyzed by two independent radiologists. The third reader classified all disagreement of bowel enhancement in three categories: (1) normal bowel enhancement; (2) doubtful bowel wall enhancement; (3) absent bowel wall enhancement. Multivariable logistic regression analysis was performed. Concomitant bowel ischemia was defined as requirement of bowel resection specifically due to ischemic lesion in operated patients and death from bowel ischemia in non-operated patients. Overall, 78/226 (35%) patients had PI associated with concomitant bowel ischemia. At multivariate analysis, Only absence or doubtful bowel wall enhancement was associated with concomitant bowel ischemia (OR = 167.73 95%CI [23.39-4349.81], P < 0,001) and acute mesenteric ischemia associated with PP (OR = 67.94; 95%CI [5.18-3262.36], P < 0.009). Among the 82 patients who underwent a laparotomy for suspected bowel ischemia, rate of non-therapeutic laparotomy increased from 15/59 (25%), 2/6 (50%) and 16/17 (94%) when bowel wall enhancement was absent, doubtful and normal respectively. Absence of enhancement of the bowel wall is the primary feature associated with concomitant bowel ischemia. It should be carefully assessed when PI is detected to avoid non-therapeutic laparotomy.

Radiomics-Based Predictive Nomogram for Assessing the Risk of Intracranial Aneurysms.

Aneurysm wall enhancement (AWE) has the potential to be used as an imaging biomarker for the risk stratification of intracranial aneurysms (IAs). Radiomics provides a refined approach to quantify and further characterize AWE's textural features. This study examines the performance of AWE quantification combined with clinical information in detecting symptomatic IAs. Ninety patients harboring 104 IAs (29 symptomatic and 75 asymptomatic) underwent high-resolution magnetic resonance imaging (HR-MRI). The assessment of AWE was performed using two different methods: 3D-AWE mapping and composite radiomics-based score (RadScore). The dataset was split into training and testing subsets. The testing set was used to build two different nomograms using each modality of AWE assessment combined with patients' clinical information and aneurysm morphological data. Finally, each nomogram was evaluated on an independent testing set. A total of 22 radiomic features were significantly different between symptomatic and asymptomatic IAs. The 3D-AWE mapping nomogram achieved an area under the curve (AUC) of 0.77 (63% accuracy, 78% sensitivity, and 58% specificity). The RadScore nomogram exhibited a better performance, achieving an AUC of 0.83 (77% accuracy, 89% sensitivity, and 73% specificity). The comprehensive analysis of IAs with the quantification of AWE data through radiomic analysis, patient clinical information, and morphological aneurysm metrics achieves a high accuracy in detecting symptomatic IA status.

Fire performance enhancement of cold-formed steel walls: experimental and numerical study

Cavity-insulated cold-formed steel (CFS) walls, with gypsum plasterboard (GP) on both sides, are widely used in the construction industry. However, their fire performance is compromised by the faster rise in the temperature of the hot flange (HF), which is influenced by cavity insulation and board joints. This study aims to optimize cavity insulation configuration to enhance fire performance. Two mid-scale, non-load-bearing fire experiments on CFS walls were conducted, employing double-layered GP boards and rock wool insulation, under ISO834 time-temperature fire conditions. Meanwhile, a finite element model of physical space fluid-heat coupling of furnace-CFS walls considering joints with time was developed to reveal joint mechanisms and configurations. The results showed that non-insulated stud CFS walls had slower HF temperature rise and similar insulation compared to insulated counterparts, delaying HF temperature reach to 537 °C by 14 min. The base-layer joints had the least adverse impact on the HF temperature rise than overlapped joints and face-layer joints. The HF temperatures for various joint configurations reached 537 °C at different times: 57 min for the face layer, 62 min for the base layer, and 49 min for overlapped joints. Considering the construction cost-effectiveness, it was found that adding steel strips (the same width as the flange) at the joint position could significantly decelerate the HF temperature rise. In 60 min, the HF temperature in the model with additional steel strips dropped by 94 °C lower than in the 10 mm overlapped joint model. Finally, based on finite element results, design equations were proposed for predicting HF temperature variation over time across joint types.

A multidimensional pre-operative planning method of unruptured vertebral artery dissecting aneurysms using three-dimensional AWE mapping and hemodynamic simulation

ObjectiveHigh-resolution magnetic resonance imaging (HR-MRI) can provide valuable insights into the evaluation of vascular pathological conditions, and 3D digital subtraction angiography (3D-DSA) offers clear visualization of the vascular morphology and hemodynamics. This study aimed to investigate the potential of a multimodal method to treat unruptured vertebral artery dissection aneurysms (u-VADAs) by fusing image data from HR-MRI and 3D-DSA. MethodsThis observational study enrolled 5 patients diagnosed with u-VADAs, who were scheduled for interventional treatment. The image data of HR-MRI and 3D-DSA were merged by geometry software, resulting in a multimodal model. Quantified values of aneurysm wall enhancement (AWE), wall shear stress (WSS), neck velocity, inflow volume, intra-stent flow velocity (ISvelocity), and intra-aneurysmal velocity (IAvelocity) were calculated from the multimodal method. ResultsWe found the actual lengths of u-VADAs in the multimodal model were longer than the 3D-DSA model. We formulated surgical plannings based on the WSS, IA velocity, and neck velocity. The post-operative value of IAvelocity, neck velocity, and follow-up quantified values of AWE were decreased compared with the pre-operative condition. After that, u-VADAs were complete occlusion in four patients and near-complete occlusion in one patient during the 6th-month follow-up after surgery. ConclusionThe multidimensional method combining HR-MRI with 3D-DSA may provide more valuable information for treating VADAs, with the potential to develop effective surgical planning.

Neutrophil to lymphocyte ratio predicts bowel ischemia in non-strangulated adhesive small bowel occlusions: a retrospective analysis from an acute care surgical service

BackgroundAdhesive small bowel obstruction (ASBO) is a leading cause of hospitalization in emergency surgery. The occurrence of bowel ischemia significantly increases the morbidity and mortality rates associated with this condition. Current clinical, biochemical and radiological parameters have poor predictive value for bowel ischemia. This study is designed to ascertain predictive elements for the progression to bowel ischemia in patients diagnosed with non-strangulated ASBO who are initially managed through conservative therapeutic approaches.MethodsThe study was based on the previously collected medical records of 128 patients admitted to the Department of Acute Care Surgery of Padua General Hospital, from August 2020 to April 2023, with a diagnosis of non-strangulated adhesive small bowel obstruction, who were then operated for failure of conservative treatment. The presence or absence of bowel ischemia was used to distinguish the two populations. Clinical, biochemical and radiological data were used to verify whether there is a correlation with the detection of bowel ischemia.ResultsWe found that a Neutrophil-Lymphocyte ratio (NLR) > 6.8 (OR 2.9; 95% CI 1.41–6.21), the presence of mesenteric haziness (OR 2.56; 95% CI 1.11–5.88), decreased wall enhancement (OR 4.3; 95% CI 3.34–10.9) and free abdominal fluid (OR 2.64; 95% CI 1.08–6.16) were significantly associated with bowel ischemia at univariate analysis. At the multivariate logistic regression analysis, only NLR > 6.8 (OR 5.9; 95% CI 2.2–18.6) remained independent predictive factor for small bowel ischemia in non-strangulated adhesive small bowel obstruction, with 78% sensitivity and 65% specificity.ConclusionsNLR is a straightforward and reproducible parameter to predict bowel ischemia in cases of non-strangulated adhesive small bowel obstruction. Employing NLR during reevaluation of patients with this condition, who were initially treated conservatively, can help the acute care surgeons in the early prediction of bowel ischemia onset.

Diagnostic Value of 3.0 T Magnetic Resonance Imaging in Active Crohn's Disease.

Magnetic resonance enteroclysis (MRE) has been widely applied to diagnose Crohn's disease (CD). Magnetic resonance (MR) at 3.0 T improves signal-to-noise ratio (SNR), shortens image acquisition time, and shows more advantages. This study aimed to retrospectively analyze the diagnostic value of 3.0 T MR imaging for active CD. 48 CD patients hospitalized in our hospital from January 2021 to December 2022 were selected as the study subjects. These 48 CD patients underwent both double-balloon enteroscopy and 3.0 T MRE. All patients' arterial phase signal, venous phase signal, bowel wall, and bowel lumen of MRE were observed to identify whether they suffered from active CD. Based on the results of enteroscopy, the number of true positives, true negatives, false negatives, and false positives diagnosed by MRE were screened; next, the diagnostic accuracy, sensitivity, and specificity of MRE in assessing active CD were calculated. Of the 48 patients, 39 were diagnosed with small bowel CD by MRE, which was not significantly different from the results of enteroscopy (P>0.05). According to MRE diagnostic results, the arterial phase predominantly presented high signal intensity, and the venous phase mainly presented low signal intensity or isointensity. Small bowel CD lesions were primarily characterized by bowel wall thickening, rare pneumatosis enhancement of the bowel wall, bowel lumen pneumatosis or dilatation, and rare strictures. Besides, MRE presented an accuracy of 93.75%, sensitivity of 97.37%, and specificity of 80.00% in diagnosing CD. 3.0 T MR imaging has diagnostic value for active CD and shows certain clinical application value.</P>.

Contrast-enhanced 3D black-blood magnetic resonance imaging for diagnosis of cerebral venous thrombosis.

Cerebral venous thrombosis (CVT) is often under-recognized on routine magnetic resonance imaging (MRI) examinations without concomitant magnetic resonance venography (MRV). Contrast-enhanced black-blood MRI (BBMRI) based on a three-dimensional T1-weighted variable-flip-angle turbo spin echo sequence, one of the sequences used routinely in our practice, has the potential for detection of thrombi in patients with CVT. The aim of this study is to evaluate the diagnostic performance and enhancement patterns of contrast-enhanced three-dimensional BBMRI for the diagnosis of CVT. Contrast-enhanced BBMRI and contrast-enhanced MRV sequences of 64 patients, acquired from June 2018 to January 2021, were retrospectively reviewed by neuroradiologists for detection of CVT in each venous sinus segment. Diagnostic performance values were calculated for contrast-enhanced BBMRI based on enhancement patterns. Of 749 venous segments from 64 patients analyzed, CVT was demonstrated in 41 venous segments from 12 patients on contrast-enhanced MRV (CE MRV). Thick wall enhancement and total enhancement patterns were dominantly demonstrated in thrombosed segments. Compared with contrast-enhanced MRV, contrast-enhanced BBMRI had a patient-based sensitivity and specificity of 100% and 98.1%, respectively, and a segment-based sensitivity and specificity of 87.8% and 96.2%, respectively. The positive predictive value of contrast-enhanced BBMRI in detecting CVT was 92.3% (patient-based) and 57.1% (segment-based), and the negative predictive value was 100% (patient-based) and 99.3% (segment-based). Contrast-enhanced BBMRI has high diagnostic performance in detection and diagnosis of CVT. This sequence may be useful to recognize CVT when dedicated CE MRV was not performed in patients with nonspecific neurological symptoms.

Optimizing timing for quantification of intracranial aneurysm enhancement: a multi-phase contrast-enhanced vessel wall MRI study.

Aneurysm wall enhancement (AWE) on high-resolution contrast-enhanced vessel wall MRI (VWMRI) is an emerging biomarker for intracranial aneurysms (IAs) stability. Quantification methods of AWE in the literature, however, are variable. We aimed to determine the optimal post-contrast timing to quantify AWE in both saccular and fusiform IAs. Consecutive patients with unruptured IAs were prospectively recruited. VWMRI was acquired on 1 pre-contrast and 4 consecutive post-contrast phases (each phase was 9 min). Signal intensity values of cerebrospinal fluid (CSF) and aneurysm wall on pre- and 4 post-contrast phases were measured to determine the aneurysm wall enhancement index (WEI). AWE was also qualitatively analyzed on post-contrast images using previous grading criteria. The dynamic changes of AWE grade and WEI were analyzed for both saccular and fusiform IAs. Thirty-four patients with 42 IAs (27 saccular IAs and 15 fusiform IAs) were included. The changes in AWE grade occurred in 8 (30%) saccular IAs and 6 (40%) in fusiform IAs during the 4 post-contrast phases. The WEI of fusiform IAs decreased 22.0% over time after contrast enhancement (p = 0.009), while the WEI of saccular IAs kept constant during the 4 post-contrast phases (p > 0.05). When performing quantitative analysis of AWE, acquiring post-contrast VWMRI immediately after contrast injection achieves the strongest AWE for fusiform IAs. While the AWE degree is stable for 36 min after contrast injection for saccular IAs. The standardization of imaging protocols and analysis methods for AWE will be helpful for imaging surveillance and further treatment decisions of patients with unruptured IAs. Imaging protocols and measurements of intracranial aneurysm wall enhancement are reported heterogeneously. Aneurysm wall enhancement for fusiform intracranial aneurysms (IAs) is strongest immediately post-contrast, and stable for 36 min for saccular IAs. Future multi-center studies should investigate aneurysm wall enhancement as an emerging marker of aneurysm growth and rupture.

Temporal evolution of primary angiitis of the central nervous system (PACNS) on MRI following immunosuppressant treatment

PurposeTo systematically analyse the time course of vessel wall enhancement and associated stenosis in patients with primary angiitis of the central nervous system (PACNS) following immunosuppressive therapy.Material and methodsTwo neuroradiologists retrospectively analysed MRIs of patients with PACNS seen at the Bern University Hospital and the St. Gallen Cantonal Hospital between 2015 and 2020. MRIs were examined for the presence of vessel wall enhancement, length of vessel wall enhancement (mm), circumferential extent of enhancement (degree) and degree of stenosis (%). Descriptive statistics and measurements of interobserver reliability were obtained. To investigate the temporal profiles of the variables following the commencement of immunosuppressant treatment, four series of Bayesian generalised multi-level models were generated.ResultsA total of 23 patients with 43 affected vessels identified from 209 MRI exams were evaluated (mean follow-up: 715 days, standard deviation ± 487 days), leading to a complete dataset of 402 entries. Vessel wall enhancement and circumferential extent of enhancement decreased for approximately 1 year after the initiation of immunosuppressant therapy. Changes were more pronounced in younger patients. Disappearance of vessel wall enhancement (in at least one vessel) was seen in about half of patients after a median of 172 days interquartile range 113–244, minimum 54 days, maximum 627 days.ConclusionsThis study evaluated the typical time course of vessel wall enhancement in patients with PACNS. Our results could be a useful reference for radiologists and clinicians interpreting follow-up imaging in patients with PACNS.Critical relevance statementRoutine clinical exams can be interpreted with more confidence when radiologists are aware of the typical temporal evolution of vessel wall enhancement in patients with primary angiitis of the central nervous system after initiation of immunosuppressive therapy.Key PointsFew data exist for vessel wall imaging of primary angiitis of the central nervous system.Following immunosuppressant therapy, vessel wall enhancement decreases for approximately one year.These results may serve as a reference for radiologists performing follow-up imaging.Graphical

Diagnostic value of MR and CT enterography in post-operative recurrence of Crohn's disease: a systematic review and meta-analysis.

Post-operative recurrence is a critical issue in the surveillance of Crohn's disease after ileocecal resection. This meta-analysis aims to assess the diagnostic yield of enterography techniques in post-operative recurrence of Crohn's disease. A systematic electronic bibliographic databases search was conducted. The inclusion criteria of original articles were: Utilized MR enterography or CT enterography after ileocolonic resection; Documented recurrence by ileo-colonoscopy (Rutgeerts' score ≥ i2); Provided crude data of diagnostic performance. A random-effect method was used for analysis. Relative risk and diagnostic value of each imaging feature were calculated. Eleven studies (11 populations and 589 patients) were included (4 CTE and 7 MRE with 248 and 341 patients, respectively). The pooled sensitivity and specificity of the enterography were 91% (95% CI: 0.85-0.95) and 75% (95% CI: 0.56-0.87), respectively. The pooled sensitivity and specificity of CTE were 93% (95% CI: 0.87-0.96) and 67% (95% CI: 0.35-0.90), respectively. MRE revealed pooled sensitivity and specificity of 90% (95% CI: 0.78-0.96) and 78% (95% CI: 0.57-0.90), respectively. The inter-study heterogeneity was low for sensitivity (I2 = 29%, p-value = 0.17) and high for specificity (I2 = 85%, p-value < 0.01). Wall enhancement, anastomosis wall thickening, anastomosis stenosis, pre-anastomotic dilatation, penetrating lesion, comb sign, and perivisceral edema were significantly higher in POR patients. Wall thickening and penetrating lesion were the most sensitive (81%) and specific (97%) findings, respectively. MRE and CTE exhibit high sensitivity and acceptable specificity (especially MRE) for detection of recurrence in Crohn's disease which makes them an effective initial screening tool and reserves ileo-colonoscopy for those patients with inconclusive enterography results.

Predictors of Survival Without Intestinal Resection after First-Line Endovascular Revascularization in Patients with Acute Arterial Mesenteric Ischemia.

Background Acute arterial mesenteric ischemia requires emergency treatment and is associated with high mortality rate and poor quality of life. Identifying factors associated with survival without intestinal resection (hereafter, intestinal resection-free [IRF] survival) could help in treatment decision-making after first-line endovascular revascularization. Purpose To identify factors associated with 30-day IRF survival in patients with acute arterial mesenteric ischemia whose first-line treatment was endovascular revascularization. Materials and Methods Patients with acute arterial mesenteric ischemia whose first-line treatment was endovascular revascularization because of a low probability of bowel necrosis were included in this single-center retrospective cohort (May 2014 to August 2022). Patient demographics, laboratory values, clinical characteristics at admission, CT scans, angiograms, and endovascular revascularization-related variables were included. The primary end point was 30-day IRF survival, and secondary end points were 3-month, 1-year, and 3-year overall survival. Factors independently associated with 30-day IRF survival were identified with binary logistic regression. Results A total of 117 patients (median age, 70 years [IQR, 60-77]; 53 female, 64 male) were included. Within 30 days after revascularization, 73 of 117 patients (62%) survived without resection, 28 of 117 (24%) survived after resection, 14 of 117 (12%) died without resection, and two of 117 (2%) underwent resection but died. The 30-day IRF survival was 63% (74 of 117). The 3-month, 1-year, and 3-year mortality rate was 18% (21 of 117), 21% (25 of 117), and 27% (32 of 117), respectively. Independent predictors of 30-day IRF survival were persistent bowel enhancement at initial CT (odds ratio [OR], 0.3; 95% CI: 0.2, 0.8; P = .013) and C-reactive protein (CRP) level less than 100 mg/L (OR, 0.3; 95% CI: 0.1, 0.8; P = .002). The 30-day IRF survival was 86%, 61%, 47%, and 23% in patients with both favorable features, persistent bowel enhancement but CRP level greater than 100 mg/L, no bowel enhancement but CRP level less than 100 mg/L, and both unfavorable features, respectively. Conclusion Independent predictors associated with 30-day IRF survival in patients with acute arterial mesenteric ischemia whose first-line treatment was endovascular revascularization were persistent bowel wall enhancement at initial CT and CRP level less than 100 mg/L. © RSNA, 2024 Supplemental material is available for this article.

Risk Factors and Clinical Significance of Ultra-Long-Term Microischemia After Intracranial Aneurysm Embolization.

This study aimed to explore influencing factors and clinical significance of ultra-long-term microischemia following intracranial aneurysm (IA) embolization and establish a theoretical foundation for reducing both the incidence of ultra-long-term microischemia and cognitive dysfunction in patients post embolization. A retrospective analysis was conducted on data from 147 patients who received endovascular treatment for IAs. Patients were categorized into microischemic and control (non-microischemic) groups on the based on the findings of high-resolution magnetic resonance vessel wall imaging (HR-VWI) examinations performed 3days postoperatively and 6months postoperatively. Risk factors for the occurrence of ultra-long-term microischemia were determined by univariate analysis and multivariate logistic regression analysis. Out of 147 patients included in the study, 51 (34.69%) developed microischemia while the remaining 96 (65.31%) did not experience this condition. Analysis revealed that factors such as sex, age, history of underlying diseases (hypertension, diabetes mellitus), aneurysmal site characteristics, the presence or absence of stenosis in the aneurysm-bearing artery, modified Fisher score at admission, Barthel's index at discharge, immunoinflammatory index at 3days postoperatively and at the 6-month follow-up, the presence or absence of aneurysmal wall enhancement, and the presence or absence of aneurysmal lumen showed no statistically significant differences between the two groups (all P > 0.05). By contrast, variables like in operative time, rupture status of the aneurysm before surgery according to World Federation of Neurologic Surgeons (WFNS) grade, aneurysm size, number of stents used, number of guidewires and catheters used, and Evans index between the two groups were found to have statistically significant disparities between those who developed microischemia and those who did not (P < 0.05). A subsequent multivariate analysis revealed that aneurysm size, Evans index, and the number of stents used were independent risk factors for the occurrence of ultra-long-term microischemia after surgical intervention of aneurysms (P < 0.05). The receiver operating characteristic (ROC) curves of the patients were constructed on the basis of risk factors determined through multivariate logistic regression analysis. Results indicated that aneurysm size (area under ROC curve (AUC) 0.619, sensitivity 94.7%, specificity 17.1%, P = 0.049), Evans index (AUC 0.670, sensitivity 96.4%, specificity 26.8%, P = 0.004), and number of stents (AUC 0.639, sensitivity 44.6%, specificity 90.2%, P < 0.001) effectively predicted the occurrence of microischemia. The incidence of cognitive dysfunction was higher in the microischemic group than in the control group (P < 0.05), and a greater number of microischemic foci was associated with a higher incidence of cognitive dysfunction. The proportion of microschemia foci in the thalamus and basal ganglia in patients with cognitive dysfunction (60.87%) was significantly higher than that in patients without cognitive dysfunction (34.55%) (P < 0.05). Aneurysm size, Evans index > 0.3, and the quantity of stents were independent risk factors for the occurrence of ultra-long-term microischemia after aneurysm embolization and provided good predictive performance. Cognitive dysfunction was closely associated with microischemia, with its severity increasing with an increase in the number of ischemic foci.

Pulmonary embolism versus pulmonary vasculitis in Hughes-Stovin syndrome: Characteristic computed tomography pulmonary angiographic findings and diagnostic and therapeutic implications. HSS International Study Group

Background and aimHughes-Stovin syndrome (HSS) is a rare systemic vasculitis with widespread venous/arterial thrombosis and pulmonary vasculitis. Distinguishing between pulmonary embolism (PE) and in-situ thrombosis in the early stages of HSS is challenging. The aim of the study is to compare clinical, laboratory, and computed tomography pulmonary angiography (CTPA) characteristics in patients diagnosed with PE versus those with HSS. MethodsThis retrospective study included 40 HSS patients with complete CTPA studies available, previously published by the HSS study group, and 50 patients diagnosed with PE from a single center. Demographics, clinical and laboratory findings, vascular thrombotic events, were compared between both groups. The CTPA findings were reviewed, with emphasis on the distribution, adherence to the mural wall, pulmonary infarction, ground glass opacification, and intra-alveolar hemorrhage. Pulmonary artery aneurysms (PAAs) in HSS were assessed and classified. ResultsThe mean age of HSS patients was 35 ± 12.3 years, in PE 58.4 ± 17 (p < 0.0001). Among PE 39(78 %) had co-morbidities, among HSS none. In contrast to PE, in HSS both major venous and arterial thrombotic events are seen.. Various patterns of PAAs were observed in the HSS group, which were entirely absent in PE. Parenchymal hemorrhage was also more frequent in HSS compared to PE (P < 0.001). ConclusionMajor vascular thrombosis with arterial aneurysms formation are characteristic of HSS. PE typically appear loosely-adherent and mobile whereas “in-situ thrombosis” seen in HSS is tightly-adherent to the mural wall. Mural wall enhancement and PAAs are distinctive pulmonary findings in HSS. The latter findings have significant therapeutic ramifications.

THE GLA D313Y MUTATION IN FABRY DISEASE: DIAGNOSTIC DILEMMAS AND THERAPEUTIC CONSIDERATIONS

Abstract Fabry disease is a genetic disorder caused by mutations in the GLA gene, which encodes alpha–galactosidase A, leading to enzyme dysfunction and subsequent accumulation of glycosphingolipids. In the classical form, it occurs in childhood or early adolescence with renal, cardiac, and neurological involvement, resulting in a poor prognosis. The GLA D313Y is a missense mutation resulting in a significant reduction of enzyme activity in plasma, while leukocyte enzyme activity remains within normal limits, leading to a "pseudo–deficiency" of enzyme activity with uncertain pathogenicity. In literature, it has been observed that individuals with this mutation may present predominantly with central neurologic manifestations, characterized by multifocal white matter lesions. Historically they do not receive indications for initiating enzyme replacement or molecular chaperone therapies. We present the case of a man affected by arterial hypertension and history of chronic lower limb pain who experienced a lacunar stroke at the age of 35. The GLA D313Y mutation was identified and enzyme activity assay in plasma was within normal limits, precluding the initiation of enzyme replacement therapy. Over the following years, he suffered two additional ischemic strokes, a progressive decline in renal function to stage 3, and acute coronary syndrome. Transthoracic echocardiography revealed hypertrophic non–obstructive cardiomyopathy (SIVd 20 mm) with normal biventricular ejection fraction. Cardiac magnetic resonance showed late gadolinium enhancement of inferior junctional and inferior wall in middle segment (Fig.1). Considering the patient‘s clinical conditions involving multi–organ impairment, an enzymatic activity test was performed on leukocytes, revealing a reduced activity. This prompted the decision to initiate enzyme replacement therapy. This case report supports the hypothesis that the D313Y mutation may be associated with a later presentation and not only neurological involvement. In our experience, it’s mandatory to proceed with the determination of leukocyte enzyme activity in presence of normal enzyme activity in plasma. As an alternative, the detection of glycosphingolipids’ accumulation could be useful, but myocardial and renal biopsy poses a high bleeding risk, especially in anticoagulated or antiaggregated patients. The authors propose considering the initiation of therapy in patients with the D313Y mutation who exhibit significant organ involvement.

CLINICAL CHALLENGE: AORTITIS IN THE EMERGENCY DEPARTMENT

Abstract A 67 yo woman presented to the ED for a sustained episode of palpitations and acute chest pain. Past medical history: suspected psoriatic arthritis previously treated with steroids and hydroxychloroquine; previous suspicion of rheumatic polymyalgia and Horton arteritis, regressed with steroids; gastroesophageal reflux disease; mild hypercholesterolemia and a family history of premature coronary artery disease. In the ED the patient was hemodynamically stable. On physical examination: tachyarrhythmic heart sounds with no murmurs. The peripheral arterial pulses were preserved and symmetrical. The remaining physical examination was unremarkable. ECG: atrial fibrillation at 150 bpm. The D–dimer was increased, normal hs–troponin and normal haemoglobin. On echocardiography: normal biventricular structure and function; normal aortic root with dilatation of proximal ascending aorta (47 mm), without visible intimal flaps. The anterior wall of the proximal ascending aorta was thickened. A moderate circumferential pericardial effusion was present, without signs of hemodynamic impact. On suspicion of an acute aortic syndrome with possible intramural haematoma of an ascending aortic aneurism, an urgent CT scan was ordered. It showed a severe dilatation of ascending aorta (52 mm) involving of the proximal aortic arch. The anterior aortic wall was thickened (5 mm), without clear images of IMH, aortic dissection or penetrating ulcer. The pericardial effusion had a supra–fluid density without blood density. A repeated CT at 6 hours showed no changes. The case was suggestive of an inflammatory aetiology. The patient was admitted to the cardiac intensive care unit. A PET–CT scan showed signs of an inflammatory disease of the great vessels. A serological autoimmunity screening was negative. The patient was diagnosed with inflammatory aortitis (giant cell vs Takayasu arteritis) and started on high dose iv glucocorticoid therapy. Given the high risk of severe aortic complications, adjunctive therapy with tocilizumab was prescribed. The therapy was gradually tapered, with subsequent switch to oral steroids plus tocilizumab. At one and a half month of follow–up, a CT scan was repeated, documenting stable aortic diameters and the disappearance of the abnormal thickness and of the enhancement of the aortic wall. A follow–up PET–CT scan has been scheduled at 6 months time. An elective aortic intervention has been indicated after remission of the inflammatory phase.

AN ACUTE MYOCARDIAL INFARCTION IN A VERY YOUNG MAN DUE TO HYPERHOMOCYSTEINEMIA

Abstract Introduction A young 17–year–old boy smoker and obese was admitted to our cardiology unit for continuous oppressive chest pain radiating to the left arm that had arisen three days earlier. In the emergency room, he had performed blood tests with evidence of an increased troponin and an electrocardiogram that showed mild ST–elevation in inferior leads with ST depression in aVL. We performed an urgent transthoracic echocardiography examination that was unremarkable Investigation Once diagnosed with acute ST–segment elevation myocardial infarction, the patient underwent emergency coronary angiography. Coronary angiography highlighted an intermediate stenosis of the first branch diagonal to the proximal tract and a possible occlusion of the periapical branch of the anterior descending artery. It was decided not to perform PTCA as we could not exclude a distal coronary dissection and considering the vessel was of small caliber. We decided to complete the diagnostic process by performing a cardiac MRI showing edema in the apex and distal inferior wall and transmural gadolinium enhancement at the level of the septum and inferior wall of the distal section. In the context of the damaged wall a focal area of hypointensity to be referred to the "no–reflow" phenomenon Management The patient was treated with DAPT, low molecular weight heparin and high dose statin. A few hours after the coronary angiography the patient stopped having chest pain and remained asymptomatic for the rest of the hospital stay. We performed complete blood tests including with evidence of hyperhomocysteinemia. The lipid panel was normal. Hematological consultation recommended folate and Vitamine B12 treatment. After 12 months the patient is asymptomatic, has stopped smoking and continues to take DAPT. He repeated the echocardiogram which showed no alterations. Genetic testing was performed after hospital discharge and was positive for Methylenetetrahydrofolate reductase (MTHFR) homozygous mutation Conclusion Our clinical case shows a rare case of acute myocardial infarction in a very young person. Diagnosis and treatment were complicated by the extreme distality of the occlusion and the few modifiable risk factors identified (smoker, obesity and hyperhomocysteinemia). Hyperhomocysteinemia is an often underestimated cardiovascular risk factor associated with a high risk of cardiovascular events and multivessel coronary disease that should be sought in all young patients with AMI.

ANOTHER BRICK IN THE WALL: A STRANGE CASE OF A BIG HUG

Abstract Background Left ventricular pseudoaneurysm (LVP) is a rare complication of myocardial infarction MI (less than 0,1% of all patients with MI) and develops when cardiac rupture is contained by adherent pericardium or scar tissue, thus contains no endocardium or myocardium, unlike a true left ventricular aneurysm (LVA). Clinical Case A 41–year–old man without remote cardiac history was admitted to the Emergency Department with chest pain that had started 7 days before after a referred ‘big hug’ from a friend. Results of the physical examination were unremarkable. Electrocardiogram: T waves negative in the inferior and lateral leads. Blood exams: normal troponins, C–reactive protein and D–dimer values. Chest computed tomography: no sign of pulmonary embolism. Transthoracic echocardiography (TE): ejection fraction 45%, voluminous aneurismal chamber communicating with the infero–posterior wall of the LV, stratified thrombus and mild mitral valve regurgitation. Coronary angiography: three vessel coronary artery disease. The last diagnosis was established by cardiac magnetic resonance imaging (MRI) which revealed a voluminous pseudoaneurysm of the inferior wall (56 mm x 50 mm with a 51 mm neck) and transmural late gadolinium enhancement (LGE) of the wall adjacent to the pseudoaneurysm with fibroadipose replacement and calcific metaplasia. The patient was transferred to the Cardiac Surgery Department. Intraoperatively the LVP was resected and the defect of the LV was repaired with a patch in bovine pericardium. In addition, coronary artery bypass grafting and mitral valve repair were performed. Discussion LVP develops when pericardium, in combination with thrombus and inflammation, surrounds a cardiac rupture. Inferior and posterolateral myocardial infarction (MI), due to occlusion of the Circumflex artery, are responsible for 82% of the LVP. LVP can be also found in the apical region due to anterior MI. In conclusion symptoms, signs, electrocardiographic abnormalities and radiographic findings seen in patients with LVP can be indistinguishable from those in patients with coronary disease alone. TE is the first non–invasive method for early diagnosis of LVA, but MRI is the gold standard for differential diagnosis between true aneurysm vs pseudoaneurysm. LGE of the pericardium is a new specific marker of pseudoaneurysm as opposed to true aneurysm.

FULMINANT EOSINOPHILIC MYOCARDITIS TREATED WITH STEROIDS AND INTRA–AORTIC BALLOON PUMP

Abstract Background Hypereosinophilic syndrome (HES) is a hematological disorders characterized by a chronic, unexplained hypereosinophilia (HE) with tissue damage. Cardiac involvement occurs in ∼20% of patients with HES and it carries a poor prognosis.Early detection and treatment of myocardial involvement improves prognosis. Immunosuppressive therapy represents the core treatment in the majority of patients with Eosinophilic Myocarditis. Case Summary 50–year–old man with history of intrinsic asthma and HE was admitted for cardiogenic shock B stage according SCAI classification (heart rate was 130 bpm, systolic blood pressure was 90 mmHg and lactate &amp;lt;2). EKG showed sinus tachycardia and diffuse ST segment depression. Echocardiography revealed severe left ventricular systolic dysfunction and mild pericardial effusion. Blood test showed leukocytosis (33x10^9/L) with HE (47%), mild hepato–renal damage, high TroponinT 3874 pg/mL and NT–proBNP 11060 ng/L. Coronary angiography was unremarkable. Pulmonary artery catheter was placed: IC 1,78 l/min/mq, CPO 0,63, WP 21, SVO2 60%, SVR 17 WU, RAP/WP 0.74. We reclassified patients as SCAI C with “mixed shock” due to inflammatory component and latent right ventricular dysfunction. Intra–aortic balloon pump (IABP) was placed and we performed endomyocardial biopsy (EMB). High dose of intra venous corticosteroids was started obtaining progressive improvement of hemodynamic parameters and reduction of inflammation parameters (Fig.1). EMB confirmed the diagnosis of myocarditis with initial transition into ‘’Loffler’s cardiomyopathy’’. Polymerase chain reaction (PCR) analysis did not detect cardiotropic viruses. After improvement of clinical status we performed cardiac MRI that revealed diffuse biventricular myocardial edema and the presence of subepicardial and mid–wall enhancement with non ischemic pattern,endocardial fibrosis and multiple left ventricular thrombi (Fig. 2). β–blockers and angiotensin–converting enzyme inhibitors were started and the patient was discharged at home with a complete myocardial recovery and immunosuppressive therapy based on corticosteroids and azathioprine. Discussion This case demonstrates the usefulness of myocardial biopsy in fulminant myocarditis. Treatment with corticosteroids resulted in rapid improvement. Early invasive monitoring allowed more precise hemodynamic characterization and mechanical circulatory support with IABP proved feasible and might have helped by unloading the left ventricle.

Association of Morphology of Lenticulostriate Arteries and Proximal Plaque Characteristics With Single Subcortical Infarction: A Whole-Brain High-Resolution Vessel Wall Imaging Study.

We aimed to investigate the association of characteristics of lenticulostriate artery (LSA) morphology and parental atheromatous disease (PAD) with single subcortical infarction (SSI) and to explore whether the LSA morphology is correlated with proximal plaque features in asymptomatic PAD. Patients with acute SSI were prospectively enrolled and classified as large- and small-SSI groups. The clinical data and imaging features of LSA morphology (branches, length, dilation, and tortuosity) and middle cerebral artery plaques (normalized wall index, remodeling index, enhancement degree, and hyperintense plaques) were evaluated. Logistic regression was performed to determine the association of large SSIs with morphologic features of LSAs and plaques. The Spearman correlation between the morphologic characteristics of LSAs and plaque features in asymptomatic PAD was analyzed. Of the 121 patients recruited with symptomatic PAD, 102 had coexisting asymptomatic contralateral PAD. The mean length of LSAs (odds ratio, 0.84 [95% CI, 0.73-0.95]; P=0.007), mean tortuosity of LSAs (odds ratio, 1.13 [95% CI, 1.05-1.22]; P=0.002), dilated LSAs (odds ratio, 22.59 [95% CI, 2.46-207.74]; P=0.006), and normalized wall index (odds ratio, 1.08 [95% CI, 1.01-1.15]; P=0.022) were significantly associated with large SSIs. Moreover, the normalized wall index was negatively correlated with the mean length of LSAs (r=-0.348, P<0.001), and the remodeling index was negatively correlated with the mean tortuosity of LSAs (r=-0.348, P<0.001) in asymptomatic PAD. Our findings suggest that mean length of LSAs, mean tortuosity of LSAs, dilated LSAs, and normalized wall index are associated with large SSIs. Moreover, plaque features in asymptomatic PAD are correlated with morphologic features of LSAs.

Performance Enhancement of a Retaining Wall with Geofoam Laid in Different Orientations

Performance Enhancement of a Retaining Wall with Geofoam Laid in Different Orientations