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Background Scar burden with late gadolinium enhancement (LGE) cardiac MRI (CMR) predicts arrhythmic events in patients with postinfarction in single-center studies. However, LGE analysis requires experienced human observers, is time consuming, and introduces variability. Purpose To test whether postinfarct scar with LGE CMR can be quantified fully automatically by machines and to compare the ability of LGE CMR scar analyzed by humans and machines to predict arrhythmic events. Materials and Methods This study is a retrospective analysis of the multicenter, multivendor CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy (DERIVATE) registry. Patients with chronic heart failure, echocardiographic left ventricular ejection fraction (LVEF) of less than 50%, and LGE CMR were recruited (from January 2015 through December 2020). In the current study, only patients with ischemic cardiomyopathy were included. Quantification of total, dense, and nondense scars was carried out by two experienced readers or a Ternaus network, trained and tested with LGE images of 515 and 246 patients, respectively. Univariable and multivariable Cox analyses were used to assess patient and cardiac characteristics associated with a major adverse cardiac event (MACE). Area under the receiver operating characteristic curve (AUC) was used to compare model performances. Results In 761 patients (mean age, 65 years ± 11, 671 men), 83 MACEs occurred. With use of the testing group, univariable Cox-analysis found New York Heart Association class, left ventricle volume and/or function parameters (by echocardiography or CMR), guideline criterion (LVEF of ≤35% and New York Heart Association class II or III), and LGE scar analyzed by humans or the machine-learning algorithm as predictors of MACE. Machine-based dense or total scar conferred incremental value over the guideline criterion for the association with MACE (AUC: 0.68 vs 0.63, P = .02 and AUC: 0.67 vs 0.63, P = .01, respectively). Modeling with competing risks yielded for dense and total scar (AUC: 0.67 vs 0.61, P = .01 and AUC: 0.66 vs 0.61, P = .005, respectively). Conclusion In this analysis of the multicenter CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy (DERIVATE) registry, fully automatic machine learning-based late gadolinium enhancement analysis reliably quantifies myocardial scar mass and improves the current prediction model that uses guideline-based risk criteria for implantable cardioverter defibrillator implantation. ClinicalTrials.gov registration no.: NCT03352648 Published under a CC BY 4.0 license. Supplemental material is available for this article. ispartof: Radiology vol:307 issue:3 pages:e222239- ispartof: location:United States status: published

While chest radiograph (CXR) is the first-line imaging investigation in patients with respiratory symptoms, differentiating COVID-19 from other respiratory infections on CXR remains challenging. We developed and validated an AI system for COVID-19 detection on presenting CXR.A deep learning model (RadGenX), trained on 168,850 CXRs, was validated on a large international test set of presenting CXRs of symptomatic patients from 9 study sites (US, Italy, and Hong Kong SAR) and 2 public datasets from the US and Europe. Performance was measured by area under the receiver operator characteristic curve (AUC). Bootstrapped simulations were performed to assess performance across a range of potential COVID-19 disease prevalence values (3.33 to 33.3%). Comparison against international radiologists was performed on an independent test set of 852 cases.RadGenX achieved an AUC of 0.89 on 4-fold cross-validation and an AUC of 0.79 (95%CI 0.78-0.80) on an independent test cohort of 5,894 patients. Delong's test showed statistical differences in model performance across patients from different regions (p0.01), disease severity (p0.001), gender (p0.001), and age (p = 0.03). Prevalence simulations showed the negative predictive value increases from 86.1% at 33.3% prevalence, to greater than 98.5% at any prevalence below 4.5%. Compared with radiologists, McNemar's test showed the model has higher sensitivity (p0.001) but lower specificity (p0.001).An AI model that predicts COVID-19 infection on CXR in symptomatic patients was validated on a large international cohort providing valuable context on testing and performance expectations for AI systems that perform COVID-19 prediction on CXR.• An AI model developed using CXRs to detect COVID-19 was validated in a large multi-center cohort of 5,894 patients from 9 prospectively recruited sites and 2 public datasets. • Differences in AI model performance were seen across region, disease severity, gender, and age. • Prevalence simulations on the international test set demonstrate the model's NPV is greater than 98.5% at any prevalence below 4.5%.

Objectives More than a year has passed since the initial outbreak of SARS-CoV-2, which caused many hospitalizations worldwide due to COVID-19 pneumonia and its complications. However, there is still a lack of information detailing short- and long-term outcomes of previously hospitalized patients. The purpose of this study is to analyze the most frequent lung CT findings in recovered COVID-19 patients at mid-term follow-ups. Methods A total of 407 consecutive COVID-19 patients who were admitted to the Fondazione IRCCS Policlinico San Matteo, Pavia and discharged between February 27, 2020, and June 26, 2020 were recruited into this study. Out of these patients, a subset of 108 patients who presented with residual asthenia and dyspnea at discharge, altered spirometric data, positive lung ultrasound and positive chest X-ray was subsequently selected, and was scheduled to undergo a mid-term chest CT study, which was evaluated for specific lung alterations and morphological patterns. Results The most frequently observed lung CT alterations, in order of frequency, were ground-glass opacities (81%), linear opacities (74%), bronchiolectases (64.81%), and reticular opacities (63.88%). The most common morphological pattern was the non-specific interstitial pneumonia pattern (63.88%). Features consistent with pulmonary fibrosis were observed in 32 patients (29.62%). Conclusions Our work showed that recovered COVID-19 patients who were hospitalized and who exhibited residual symptoms after discharge had a slow radiological recovery with persistent residual lung alterations. Advances in knowledge This slow recovery process should be kept in mind when determining the follow-up phases in order to improve the long-term management of patients affected by COVID-19.

Arrhythmogenic cardiomyopathy with left ventricular involvement (ACM-LV), particularly in case of isolated left ventricular involvement (i.e. left dominant arrhythmogenic cardiomyopathy, LDAC) and previous infectious myocarditis (pIM) may have overlapping clinical and cardiac magnetic resonance (CMR) features. To date, there are no validated CMR criteria for the differential diagnosis between these conditions. The present study aimed to identify CMR characteristics to distinguish ACM-LV from pIM.This observational, retrospective, single-centre study included 30 pIM patients and 30 ACM-LV patients. In ACM-LV patients CMR was performed at diagnosis; in patients with pIM, CMR was performed six months after acute infection. CMR analysis included quantitative assessment of left ventricle (LV) volumes, systolic function and wall thicknesses, qualitative and quantitative assessment of late gadolinium enhancement (LGE) sequences. Compared with pIM, ACM-LV patients showed slightly larger LV volumes, more frequent regional wall motion anomalies and reduced wall thicknesses. ACM-LV patients had higher amounts of LV LGE and extension. Notably, the LDAC subgroup had the highest amount of LV LGE. LV LGE amount 15 g and a LV LGE percentage 30% of LV mass discriminated ACM-LV from pIM with a 100% specificity. LGE segmental distribution was superimposable among the groups, except for septal segments that were more frequently involved in ACM-LV and LDAC patients.A great extension of LV LGE (a cut-off of LGE15 g and a percentage above 30% of LV LGE in relation to total myocardial mass) discriminates ACM-LV from pIM with extremely high specificity.

To perform a semiquantitative and quantitative analysis of interstitial lung disease (ILD), through computed tomography (CT), in different serological subgroups of idiopathic inflammatory myopathies (IIM) patients, to find radiologic and clinical differences of disease related to serology.This was a prospective study, which included 98 IIM patients, divided into serological subgroups: anti-aminoacyl-transfer-RNA-synthetases (anti-ARS) positive and myositis-specific autoantibodies (MSA) negative.For each baseline CT the total semiquantitative score of Warrick (WS) and the automated software (Computer-Aided Lung Informatics for Pathology Evaluation and Rating) quantitative scores interstitial lung disease % (ILD%) and vascular-related structure % (VRS%) were calculated. Pulmonary function tests included total lung capacity % (TLC%), forced vital capacity % (FVC%), and diffusing capacity of the lung for carbon monoxide % (DLCO%).Inverse correlations ( P0.001) between the radiologic scores and the functional scores DLCO% and TLC% were found, the most relevant being between ILD% and DLCO% (ρ=-0.590), VRS% and DLCO% (ρ=-0.549), and WS and DLCO% (ρ=-0.471).Positive correlations between ILD% and VRS% (ρ=0.916; P0.001), WS and ILD% (ρ=0.663; ρ0.001), and WS and VRS% (ρ=0.637; P0.001) were obtained.Statistically significant higher values of WS, ILD%, and VRS% were found in the anti-ARS group (WS=15; ILD%=11; VRS%=3.5) compared with the MSA negative one (WS=2.5; ILD%=0.84; VRS%=2.2).The nonspecific interstitial pneumonia pattern was dominant. No statistically significant differences emerged at pulmonary function tests.In this study, ILD in anti-ARS-positive and MSA-negative groups was defined through semiquantitative and quantitative analysis of lung CT. The inverse correlations between the radiologic scores and TLC% and DLCO% ( P0.001) confirm the role of lung CT in the evaluation of ILD in IIM.

Pulmonary involvement in Sjogren syndrome (SS) could manifest as cystic lung disease (CLD). CLD in SS includes lymphocytic interstitial pneumonia (LIP) and pulmonary amyloidosis. Differential diagnosis usually requires surgical lung biopsy, whereas CT-guided percutaneous fine needle aspiration biopsy (CT-FNAB) has not yet explored. We describe the case of a 63-year-old never smoker Caucasian female with a SS diagnosis who displayed a newly detected diffuse CLD at high-resolution computed tomography, though totally asymptomatic. Given the favorable location of one big lesion at the superior left lobe, a CT-FNAB was proposed instead of a more invasive SLB. At histology examination a diagnosis of pulmonary nodular AL kappa amyloidosis in the context of SS was established. In conclusion, CT-FNAB might represent an alternative and less invasive diagnostic procedure than SLB in the differential diagnosis of CLD, even if further research is needed. Moreover, this case presents an unusual association between SS and pulmonary nodular AL kappa amyloidosis, with pulmonary nodules and cysts without systemic manifestations.

Objective To analyze the most frequent radiographic features of COVID-19 pneumonia and assess the effectiveness of chest X-ray (CXR) in detecting pulmonary alterations. Materials and methods CXR of 240 symptomatic patients (70% male, mean age 65 ± 16 years), with SARS-CoV-2 infection confirmed by RT-PCR, was retrospectively evaluated. Patients were clustered in four groups based on the number of days between symptom onset and CXR: group A (0–2 days), 49 patients; group B (3–5), 75 patients; group C (6–9), 85 patients; and group D (> 9), 31 patients. Alteration’s type (reticular/ground-glass opacity (GGO)/consolidation) and distribution (bilateral/unilateral, upper/middle/lower fields, peripheral/central) were noted. Statistical significance was tested using chi-square test. Results Among 240 patients who underwent CXR, 180 (75%) showed alterations (group A, 63.3%; group B, 72%; group C, 81.2%; group D, 83.9%). GGO was observed in 124/180 patients (68.8%), reticular alteration in 113/180 (62.7%), and consolidation in 71/180 (39.4%). Consolidation was significantly less frequent (p 9 days from the onset of symptoms.

© Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Although antisynthetase antibodies (ARS) are the established markers of the so-called antisynthetase syndrome (ASSD), in these patients the concomitant positivity of anti-Ro52 antibodies, reported in up to the 50% of cases, is not rare. Several studies focused on the effect of different ARS specificities on the evolution of ASSD, the most recent showing no effects. On the contrary, the role of co-occurring anti-Ro52 antibodies in ASSD is still debated. We investigated the potential of anti-Ro52 antibodies in identifying a clinical phenotype of ASSD or influencing prognosis, irrespectively to the underlying ARS specificity.Retrospective analysis of clinical, imaging and laboratory characteristics, therapeutic approaches and outcome at baseline and at last follow-up, of 60 ASSD patients progressively enrolled at our Hospital.We identified 34 anti-Ro+ and 26 anti-Ro- ASSD patients. Classic triad prevalence at baseline was similar between the two groups, whereas interstitial lung disease (ILD) (p value=0.01) and myositis (p value=0.03) were significantly more prevalent in anti-Ro52+ and in anti-Ro52- patients at last follow up, respectively. No differences in therapeutic approaches, oxygen need and ILD patterns were observed. Overall mortality was 25% (15 subjects). No differences in mortality, overall and disease related, between anti-Ro52+ and anti-Ro52- patients were observed (p value=0.764), despite the more frequent ILD occurrence in anti-Ro52+ patients. Survival curves were not different at any time point (Log-rank test, p value 0.98).Anti-Ro52 antibodies affect time course and clinical characteristics of ASSD. Although ILD is significantly more associated to anti-Ro52 antibodies, no difference in mortality was observed compared to anti-Ro52 negative patients.

Aims Right ventricular systolic dysfunction (RVSD) is an important determinant of outcomes in heart failure (HF) cohorts. While the quantitative assessment of RV function is challenging using 2D-echocardiography, cardiac magnetic resonance (CMR) is the gold standard with its high spatial resolution and precise anatomical definition. We sought to investigate the prognostic value of CMR-derived RV systolic function in a large cohort of HF with reduced ejection fraction (HFrEF). Methods and results Study cohort comprised of patients enrolled in the CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DefibrillAtor ThErapy registry who had HFrEF and had simultaneous baseline CMR and echocardiography (n = 2449). RVSD was defined as RV ejection fraction (RVEF) Conclusion RV systolic dysfunction is an independent predictor of ACM in HFrEF, with a more pronounced prognostic value in select subgroups, likely reflecting the importance of RVSD in the early stages of HF progression.

Interrupted aortic arch diagnosed in adult age is a rare entity, with only a few cases published in the literature. Most of them are classified as type A interrupted aortic arch and differential diagnosis is associated with severe chronic coarctation. We present a case of a 52-year-old woman accessed to the emergency department for chest and right upper limb pain that increased in the last days. She underwent a computed tomography angiogram showing interruption of the aortic arch, distal to left subclavian artery origin, large bilateral collateral vessels connecting subclavian arteries to descending aorta with multiple voluminous aneurysms, a bicuspid aortic valve, dilatated tubular segment of ascending thoracic aorta, and a suspected atrial septal defect. A nonsystematic literature review regarding these conditions has been performed.

The aim of this study is to present the results of the Italian survey on the management of pulmonary nodules incidentally identified at computed tomography (CT). An online electronic survey, consisting of 23 multiple-choice questions, was created using the SurveyMonkey web-based tool. The questionnaire was developed by the Board of the Italian College of Chest Radiology of the Italian Society of Medical and Interventional Radiology (SIRM) and by an experienced group of Italian Academic Chest Radiologists. The link to the online electronic survey was submitted by email to all the SIRM members. A total of 767 radiologists, corresponding to 7.5% of all the SIRM members, participated in the online survey. The majority of participants (92%) routinely describe the attenuation of pulmonary nodules in the report, and 84.1% recommend the further follow-up, with 92.7% of respondents taking CT nodule morphological features into consideration. The 57.7% of participants adhere to the Fleischner Society guidelines for the management of incidental pulmonary nodules. However, 56.6% and 75.6% of respondents have a more cautious approach than that recommended by the guidelines and tend to use a shorter follow-up for both solid and ground-glass nodules, respectively. Finally, 94.5% of participants favor congresses and refresher courses dedicated to insights on lung nodule diagnosis and management. This survey demonstrates that the management of pulmonary nodules incidentally detected on CT is still complex and controversial. The majority of SIRM members express a need for an update on this topic.

Interstitial lung diseases (ILDs) represent a heterogeneous group of entities, some of them characterized by an excessive deposition of extracellular matrix, in particular collagen, associated with a progressive destruction of the lung parenchyma. Thig group of diseases includes both idiopathic and secondary conditions. Although the underlying causes are different, some fibrotic lung diseases show a similar chronic progressive behavior, independently of the underlying etiology. An accurate diagnosis is mandatory to guide management and treatment of patients with interstitial lung diseases, in particular those with a fibrotic pattern. Imaging, and in particular high-resolution CT, plays a central role, therefore it is fundamental that radiologists become familiar with these diseases and their presentations, in order to be able to provide one or a limited number of possible differential diagnosis.

An accurate non-invasive evaluation of the mechanical properties of the vessel wall is important for a variety of screening protocols and surgical treatments. In this work, we focused on a section of the Pulmonary Artery (PA), and developed a patient-specific approach to estimate its stiffness in terms of the Young's modulus along the circumferential direction (E). First, we developed a patient-specific semi-automatic approach to estimate its expected value and standard deviation. To this end, pressure-length curves were derived from magnetic resonance images acquired during the cardiac cycle and information on vessel pressure obtained by catheterization. Then, the estimates of E were derived through a maximum likelihood estimation approach based on a vessel constitutive law. In particular, we analyzed the entire PA boundary and an arc free from surrounding organs. Second, we applied the approach to the study of pulmonary endarterectomy (PEA) for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). We observed a decrease in the circumferential E after PEA for the whole boundary, while no clear trend was observed for the free arc. The low standard deviations associated with the estimates showed high accuracy when considering the entire boundary, while greater variability was observed for the free arc, which was however limited. Finally, reliable hysteretic behavior was obtained from the reconstructed pressure-length curves.

We report the case of a 69-year-old woman, a never-smoker, with anti-melanoma differentiation-associated gene-5 (MDA5) antibody-positive amyopathic dermatomyositis (ADM) and autoimmune thrombocytop...

Purpose Chest imaging modalities play a key role for the management of patient with coronavirus disease (COVID-19). Unfortunately, there is no consensus on the optimal chest imaging approach in the evaluation of patients with COVID-19 pneumonia, and radiology departments tend to use different approaches. Thus, the main objective of this survey was to assess how chest imaging modalities have been used during the different phases of the first COVID-19 wave in Italy, and which diagnostic technique and reporting system would have been preferred based on the experience gained during the pandemic. Material and Methods The questionnaire of the survey consisted of 26 questions. The link to participate in the survey was sent to all members of the Italian Society of Medical and Interventional Radiology (SIRM). Results The survey gathered responses from 716 SIRM members. The most notable result was that the most used and preferred chest imaging modality to assess/exclude/monitor COVID-19 pneumonia during the different phases of the first COVID-19 wave was computed tomography (51.8% to 77.1% of participants). Additionally, while the narrative report was the most used reporting system (55.6% of respondents), one-third of participants would have preferred to utilize structured reporting systems. Conclusion This survey shows that the participants’ responses did not properly align with the imaging guidelines for managing COVID-19 that have been made by several scientific, including SIRM. Therefore, there is a need for continuing education to keep radiologists up to date and aware of the advantages and limitations of the chest imaging modalities and reporting systems.

Interstitial lung disease (ILD) are a large group of diffuse lung diseases characterized by similar clinical, pathological and radiological features. High resolution computed tomography (HRCT) has a central role in ILD diagnosis and management. In the last few years, computer-aided methods as Quantitative Computer Tomography (QCT) and Artificial Intelligence (AI) software were proposed as a source of reliable quantitative imaging biomarkers. The present review aimed to summarize and describe the current QCT and AI methods and to evaluate their potential diagnostic and prognostic role. The first attempt to a quantitative analysis of HRCT in ILD is represented by the density histogram analysis with the definition of two new parameter, Kurtosis and Skewness. Then texture analysis tools were developed as Adaptive Multiple Features Method (AMFM), Computer-Aided Lung Informatics for Pathology Evaluation and Ratings (CALIPER), Quantitative Lung Fibrosis (QLF) and Automated Quantification System (AQS). The introduction of AI technologies further increased the amount of objective and reproducible biomarkers. The diagnostic and prognostic role of QCT and AI methods was analyzed and confirmed in various studies, as reported in the review. QCT and AI technologies application led to the introduction of new objective biomarkers with relevant diagnostic and prognostic implications. However, there is still the need for more prospective study and the creation of open-source datasets would help to assess QCT and AI methods efficacy and to compare them.

Objective: to analyze the most frequent radiographic features of COVID-19 pneumonia and assess the effectiveness of CXR in detecting pulmonary alterations.Materials and Methods: CXR of 240 symptomatic patients (70% male, mean age 65±16 years), with SARS-CoV-2 infection confirmed by RT-PCR were retrospectively evaluated. Patients were clustered in four groups based on the number of days between symptom onset and CXR: A (0-2 days) 49 patients, B (3-5) 75 patients, C (6- 9) 85 patients and D (>9) 31 patients. Alteration’s type (reticular/opacification/consolidation) and distribution (bilateral/unilateral, upper/middle/lower fields, peripheral/central) were noted. Statistical significance was tested using chi-squared test.Results: among 240 CXR, 60 (25%) were negative (A 36.7%, B 28%, C 18.8%, D 16.1%). Opacification was observed in 124/180 (68.8%), reticular alteration in 113/180 (62.7%), consolidation in 71/180 (39.4%). Consolidation was significantly less frequent (pConclusions: the most frequent lesions in COVID-19 patients were opacification (intermediate/late phase) and reticular alteration (early phase) while consolidation gradually increased over time. The most frequent distribution was bilateral, peripheral, with middle/lower predominance. Overall rate of negative CXR is 25%, progressively decreased over time.

Interstitial lung disease (ILD) encompasses a wide range of parenchymal lung pathologies with different clinical, histological, radiological, and serological features. Follow-up, treatment, and prognosis are strongly influenced by the underlying pathogenesis. Considering that an ILD may complicate the course of any connective tissue disease (CTD) and that CTD's signs are not always easily identifiable, it could be useful to screen every ILD patient for a possible CTD. The recent definition of interstitial pneumonia with autoimmune features is a further confirmation of the close relationship between CTD and ILD. In this context, the multidisciplinary approach is assuming a growing and accepted role in the correct diagnosis and follow-up, to as early as possible define the best therapeutic strategy. However, despite clinical advantages, until now, the pathways of the multidisciplinary approach in ILD patients are largely heterogeneous across different centers and the best strategy to apply is still to be established and validated. Aims of this article are to describe the organization of our multidisciplinary group for ILD, which is mainly focused on the early identification and management of CTD in patients with ILD and to show our results in a 1 year period of observation. We found that 15% of patients referred for ILD had an underlying CTD, 33% had interstitial pneumonia with autoimmune feature, and 52% had ILD without detectable CTD. Furthermore, we demonstrated that the adoption of a standardized strategy consisting of a screening questionnaire, specific laboratory tests, and nailfold videocapillaroscopy in all incident ILD proved useful in making the right diagnosis.

Introduction SCAD is a rare coronary event that may cause acute coronary syndromes (ACS). SCAD predominantly occurs in apparently healthy, young to middle aged women (up to 95% of cases). The known causes include: heritable connective tissue diseases, fibromuscular dysplasia, arteritis, contraceptives, cocaine abuse and chest trauma. A variable proportion of patients manifests progression to longer segments of the same vessel or recurrence of dissection in other coronary arteries. SCAD can be the first manifestation of a previously unrecognised systemic disease. Methods In 2010 we started collecting consecutive SCAD, as first clinical manifestation, in patients addressed to our attention for investigation of genetic or non genetic causes, after successful management of the acute phase and exclusion of systemic inflammatory and autoimmune diseases. All patients underwent genetic visit and counselling, collection of clinical reports and imaging records, clinical cardiologic evaluation with pan-angio CT scan, biochemical testing including coagulation-related tests, and and genetic testing of genes causing connective tissue diseases. Parallel clinical family screening and genetic testing were systematically performed. Results The series is constituted of 35 patients (28F and 7M) (age at onset, mean ± SD, 44±7.6 years) with ACS-SCAD (20 STEMI and 15 NSTEMI) and 9 second dissections in a different coronary artery. Two sisters had ACS-SCAD caused by dissection of the same coronary artery. We identified pathologic mutations (n=19/35, 54%) in COL3A1 (n=3), FBN1 (n=1), FBN1+TGFBR1 (n=1), TGFBR1 (n=2), TGFBR2 (n=1), MYLK (n=1), SMAD3 (n=1), COL5A1 (n=1 homozygous), COL5A2 (n=1), MYH11 (n=1), TGFB2 (n=1), ABCC6 (1 homozygous), ELN (2 homozygous sisters and 1 heterozygous unrelated patient), NOTCH1 (n=1). In 8 (23%) patients we identified VUS classified as C3 because previously unreported and predicted as uncertain on the basis of in silico analyses. In the remaining 8 patients we only identified C2 variants. A second SCAD (14 days to 78 months after the first event) occurred in 9 patients (9/35, 25%) (COL3A1 (n=2), FBN1 (n=1), FBN1+TGFBR1 (n=1), MYLK (n=1), COL5A2 (n=1), NOTCH1 (n=1) COL5A2 (n=1) and 1 with a C2 variant in COL3A1. Two patients with thrombocytosis were carriers of the somatic JAK2 V617F mutation. Extra-coronary arterial dilations/aneurysms occurred in 13 families; in the follow-up 2 patients demonstrate dissection in non-coronary arteries. Conclusions Our series, with the potential bias of a referral centre for inherited cardiovascular disease, demonstrated that SCAD is the possible first manifestation of a genetic disorder and that neither disease gene or mutation predicts the risk of a second coronary event. SCAD is a potentially fatal coronary event associated with ACS, warning for familial disease and unpredictable risk of recurrence.

Background: Clinical evolution and optimal management of IPAF are still undefined and need prospective assessment. Objectives: to define the characteristics and evolution of IPAF patients in a multidisciplinary setting. Methods: IPAF pts referring to multidisciplinary Rheumatology/Pneumology outpatient clinic. We excluded patients with diagnosis of Anti-synthetase Sd. Data were retrospectively collected. Results: we analyzed the first 25 pts (19 F, 76%, 6 M, 24%), with a median onset age of 67 years (IQR, 59-74) and follow-up of 32 months (IQR 22-69). ANA test was positive in 23 (92%) cases (cytoplasmic+ in 17:68%). Anti-ENA screen was + in 9: 36% pts. One pt was + for anti-PM-Scl, one for for anti-Mi2 and one for anti-Ku Ab. Pts had mainly a NSIP pattern (19, 76%, cases; with concomitant OP, in 3). Three patients had UIP-like and 3 OP pattern. The majority of patients (15, 60%) satysfied only the morphological and serological domains. Clinical domains satisfied were: arthritis (4) Raynaud’s ph. (5) palmar teleangectasias (2) mechanic’s hands (1) and Hikers feet (1). Clinical spectrum time course was variable in 5 pts: 3 pts developed arthritis after ILD, and 2 developed ILD respectively after arthritis and inflammatory miopathy. Three pts were admitted in ICU for Rapidly Progressive (RP) ILD and 2 died. All pts received low dose steroids in combination mainly with Azathioprine, Mycophenolate, Cyclosporin, Hydroxychloroquine. 92% of patients were alive at FU. Conclusions: A multidisciplinary approach is useful in the diagnosis and the management of IPAF. RP-ILD was common, prognosis of IPAF was highly variable.

Interstitial Lung Disease (ILD) is a common finding of Systemic Sclerosis (SSc) mainly presenting in the form of Nonspecific Interstitial Pneumonia (NSIP) and deeply affecting patients' prognosis. Beside NSIP, other types of ILD have been reported. The most recently described pattern is the so-called Combined-pulmonary emphysema and lung fibrosis, characterized by the coexistence of both upper lobes centrilobular and paraseptal emphysema and lower lobes ILD. We presented three cases of patients with SSc, in which High Resolution Computed Tomography examinations showed emphysema with atypical distribution and radiological presentation, without or with mild signs of fibrosing lung disease, that stabilized after immunosuppressive treatment.

Background Interstitial Pneumonia with Autoimmune feature (IPAF) is a recently defined disease that includes Interstitial Lung Disease (ILD) patients with features of autoimmunity, not satisfying any of the established classification criteria for connective tissue diseases (CTD). Even if IPAF pateints share clinical and serological findings with CTD, we are unaware about the clinical evolution of IPAF pateints. Objectives To define the characteristics and evolution of IPAF patients in a multidisciplinary setting. Methods We selected patients with a diagnosis of IPAF referring to a multidisciplinary Rheumatology/Pneumology/Radiology team at our hospital. We excluded from the analysis patients positive for antisynthetase antibodies, as diagnosed with antisynthetase syndrome. Data were retrospectively collected from our hospital medical records. Results We analyzed 25 patients (19 females, 76%, 6 males, 24%), with a median onset age of 67 years (interquartile range, IQR, 59-74) and a follow-up of 32 months (IQR 22-69). ANA test was positive in 23 (92%) cases (fig 1), whereas cytoplasmic positivity was observed in 17 (68%). Anti-ENA screen was positive in 9 patients (36%) (7 [28%] anti-Ro52 and 2 [8%] anti-RNP). One patient (4%) was positive for anti-PM-Scl, 1 (4%) for anti-Mi2 and 1 (4%) for anti-Ku antibodies. These 2 latter antibodies are not included in the serological domain of IPAF. Patients had mainly a Non Specific Interstitial Pneumonia (NSIP) pattern (19 [76%]; fibrosing in 7 [28%], and with concomitant organizing pneumonia (OP) in 3 [12%]). Three (12%) patients had Usual Interstitial Pneumonia-like and 3 (12%) an OP pattern. The majority of patients (15 [60%]) satisfied only the morphological and serological domains. Clinical domains satisfied were: arthritis (4 [16%]), Raynaud’s phenomenon (5 [20%]), palmar telangiectasias (2 [8%]), mechanic’s hands (1 [4%]) and hiker’s feet (1 [4%]). We observed other findings not included in IPAF criteria but suggestive for CTDs, both clinical (inflammatory myopathy 3 [12%]; dry eye1 [4%]; sclerodactyly 1 [4%]), and instrumental (scleroderma pattern at naifold capillaroscopy 1 [4%]; dilated esophagus at barium X-rays 2 [8%]). Clinical spectrum time course was variable in 5 (20%) cases: 3 (12%) patients developed arthritis after ILD, and 2 (8%) developed ILD respectively after arthritis and inflammatory myopathy onset. Three (12%) patients were admitted to the Intensive Care Unit for Rapidly Progressive (RP) ILD and 2 died (respectively 2 months and 54 months after ILD onset), whereas the alive patient had 2 ICU admission for RP-ILD. Five (20%) patients, including the only 1 dismissed from ICU, needed home O2 therapy. Ongoing and previous therapies are reported in figure 2. Conclusion We showed that the prognosis of IPAF is highly variable, with patients experiencing RP ILD, other slow progressive worsening of respiratory functions and other a substantially stable disease. Furthermore, we showed other findings, laboratory, clinical and instrumental, that could help clinicians in a better identification and stratification of IPAF patients. As a matter of fact, at present, IPAF appears as a generic term including very different conditions that can be further differentiated according to clinical and serological data. References [1] Fischer A, et al. Eur Respir J 2015 [2] Scire CA, et al. Resp Med 2017 [3] Sambataro G, et al. Eur Resp Rev2018 [4] Cavagna L, et al. Eur Resp Rev2018 Disclosure of Interests Emiliano Marasco: None declared, Federica Meloni: None declared, Giovanni Zanframundo: None declared, Adele Valentini: None declared, Valentina Morandi: None declared, Veronica Codullo: None declared, Lorenzo Volpiano: None declared, Claudia La Carruba: None declared, Francesca Motta: None declared, Carlomaurizio Montecucco Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Sanofi, Genzyme, Lilly, MSD, Pfizer, UCB, Lorenzo Cavagna: None declared

“Aortic acute syndrome” comprises several life-threatening aortic pathologies that give rise to aortic symptoms. An early diagnosis and an adequate immediate treatment are mandatory for the survival of these patients. We discuss the diagnostic approach and the most recent surgical treatment for this frightening syndrome that still carries high morbidity and mortality despite the continuous development of new surgical procedures and technologies.

Background:Interstitial lung disease (ILD), is common in patients with idiopathic inflammatory myopathies (IIM) and strongly impact on patients’ morbidity and mortality. Patients with anti-aminoacyl-transfer RNA-synthetases (anti-ARS) antibodies are associated with an increased risk of ILD.Objectives:Defining the radiological characteristics of IIM patients, with special focus on serological groups, through qualitative, semiquantitative and quantitative analysis of lung CT.Methods:This was a prospective study conducted from 2016 to 2019. Ninety-eight IIM patients (35 men, 63 women) were included. Myositis specific autoantibodies (MSA) were assessed with Myositis Prophyle III (Euroimmune, Lubeck).Each patient had a baseline CT; the total score of Warrick (WS) was obtained at semiquantitative analysis. The radiological scores ILD% (interstitial lung disease %) and PVRS% (pulmonary vascular related structure) were the result of quantitative analysis in 61 patients (CALIPER). Pulmonary function tests (PFTs) included TLC%, FVC% and DLCO% (65 patients). The analysis was conducted in the whole group and divided in subgroups based on their MSA pattern: in particular anti-ARS (Group 1) and patients negative to MSA (Group 2) were analysed.Results:Positive correlations between ILD% and PVRS% (Rho=0.916; ρ=0.000), WS and ILD% (Rho=0.663; ρ=0.000) and WS and PVRS% (Rho=0.637; ρThe most relevant inverse correlations were found between ILD% and DLCO% (Rho=-0.590; ρ=0.001), PVRS% and DLCO% (Rho=-0.549; ρStatistically significant higher values of WS, ILD% and PVRS% were found in Group 1 (WS=15, ILD%=11 and PVRS%=3.5), compared to Group 2 (WS=2.5, ILD%=0.84 and PVRS%=2.2). NSIP pattern resulted dominant represented in the two groups (80% Group 1, 75% Group 2). No statistically significant differences of DLCO%, FVC% and TLCO% were found.Conclusion:The inverse correlations between the radiological scores and the functional data TLC% and DLCO% (ρDisclosure of Interests:None declared

In CTEPH patients who have undergone pulmonary endarterectomy, exercise capacity is better associated with changes in invasively measured pulmonary arterial compliance than with changes in magnetic resonance imaging of pulmonary artery stiffnesshttp://bit.ly/30DAWUj

Lemierre syndrome usually affects otherwise healthy adolescents or young adults and occurs at an overall rate of 1 to 10 cases per million person-years with an estimated fatality rate of 4 to 9%. Diagnostic criteria remain debated and include acute neck/head bacterial infection (often tonsillitis caused by anaerobes at high potential for sepsis and vascular invasion, notably Fusobacterium necrophorum) complicated by local vein thrombosis, usually involving the internal jugular vein, and systemic septic embolism. Medical treatment is based on antibiotic therapy with anaerobic coverage, anticoagulant drugs and supportive care in case of sepsis. Surgical procedures can be required, including drainage of the abscesses, tissue debridement and jugular vein ligation. Evidence for clinical management is extremely poor in the absence of any adequately sized study with clinical outcomes. In this article, we illustrate two cases of Lemierre syndrome not caused by Fusobacterium necrophorum and provide a clinically oriented discussion on the main issues on epidemiology, pathophysiology and management strategies of this disorder. Finally, we summarize the study protocol of a proposed systematic review and individual patient data meta-analysis of the literature. Our ongoing work aims to investigate the risk of new thromboembolic events, major bleeding or death in patients diagnosed with Lemierre syndrome, and to better elucidate the role of anticoagulant therapy in this setting. This effort represents the starting point for an evidence-based treatment of Lemierre syndrome built on multinational interdisciplinary collaborative studies. ispartof: HAMOSTASEOLOGIE vol:39 issue:1 pages:76-86 ispartof: location:Germany status: published

Background Accuracy in left atrial (LA) anatomical reconstruction is crucial to the safe and effective performance of catheter ablation of atrial fibrillation (AF). The aim of this study was to evaluate the accuracy of LA reconstruction performed with intracardiac echocardiography (ICE) as compared to fast anatomical mapping (FAM) both integrated in the CARTO mapping system (Biosense Webster, Diamond Bar, CA, USA). Methods A multislice computed tomography (MSCT) was preacquired from 29 patients with AF who underwent catheter ablation and 3D-LA geometry was reconstructed using both ICE and FAM separately. The accuracy of the LA anatomical definition was evaluated by comparing LA volumes, LA and pulmonary vein (PV) diameters obtained using ICE and FAM versus MSCT (gold standard). Results Anterior-posterior and superior-inferior LA diameters were shorter in ICE versus MSCT (32 ± 10 vs 46 ± 9 mm and 48 ± 7 vs 53 ± 7 mm, P < 0.01) but similar in FAM versus MSCT (45 ± 9 vs 46 ± 9 mm and 52 ± 10 vs 53 ± 7 mm). Latero-septal LA diameter was similar in ICE versus MSCT (63 ± 11 vs 63 ± 9 mm) but larger in FAM versus MSCT (69 ± 9 vs 63 ± 9 mm, P < 0.001). LA volume was lower in ICE versus MSCT (73 ± 30 mL vs 116 ± 45 mL, P < 0.0001) and slightly larger in FAM versus MSCT (132 ± 45 vs 116 ± 45 mL, P = 0.06). PV diameters were similar in FAM versus MSCT but significantly underestimated with ICE. Conclusions Overall accuracy in the LA and PV anatomical reconstruction was found to be superior with FAM compared to ICE-guided approach. ICE resulted in a significant underestimate of both LA and PV dimensions, while FAM slightly overestimated LA geometry.

The presence of activating mutations of the epidermal growth factor receptor (EGFR)-gene identifies a distinct and clinically relevant molecular subset of non-small-cell lung cancer. It is now well demonstrated that EGFR tyrosine kinase inhibitors (TKIs) gefitinib and erlotinib are superior to standard chemotherapy in this subset of tumors. Nevertheless, in many cases, responses are not durable and last for 6–12 months due to the occurrence of secondary or acquired resistance. Here we present three cases of EGFR-mutant lung adenocarcinomas (ADC), that showed an unexpected response to anti-EGFR small molecules. The first patient presented a continued 89 month-long response to erlotinib in a tumor recurred after surgery and conventional chemotherapy. In the other cases, subclinically persistent tumor in the lung tissue was documented histologically in lung resections performed after partial response to TKI treatment. The persistence of interstitial and endolymphatic tumor cells after TKI treatment might explain the common observation of tumor relapse after TKI discontinuation, and sustain the decision to continue treatment in responsive patients as in our first case.

The unroofed coronary sinus is a spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and the left atrium is absent. Most cases are associated with anomalous systemic venous return, such as persistent left superior vena cava.The diagnosis of this lesion is important to the prognosis of the patient, but it is often difficult because of nonspecific clinical features.Here we report a case of a complete unroofed coronary sinus and persistent left superior vena cava discovered during a computed tomographic investigation for pulmonary hypertension.Sommario“Unroofed coronary sinus” comprende un insieme di anomalie cardiache in cui la parete comune fra seno coronarico e atrio sinistro è parzialmente o completamente assente. La maggior parte dei casi è associata a un ritorno venoso sistemico anomalo, come la persistenza della vena cava superiore sinistra.La diagnosi di questa anomalia è importante per la prognosi del paziente, ma è spesso difficoltosa a causa dei segni clinici aspecifici.Qui illustriamo un caso di assenza completa del tetto del seno coronarico associato a persistenza della vena cava superiore sinistra, riscontrato durante un esame di tomografia computerizzata effettuato per ipertensione polmonare.

Aims To assess the relationship between cardiovascular magnetic resonance (CMR) parameters and both spontaneous ventricular tachycardia (VT) and risk of sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) patients. Methods and results One hundred and eight consecutive HCM patients (mean age 42 ± 15 years, 76% males) underwent CMR evaluation and risk assessment. Delayed contrast enhancement (DCE) was quantified with a specifically designed score. Endpoints were either the presence of clinical VT/ventricular fibrillation (VF) or of acknowledged risk factors for SCD. Compared to patients without arrhythmia, those with VT/VF ( n = 33) had a higher DCE score [median 8 (2–13) vs. 11 (6–20); P = 0.01]; DCE score was also the only independent predictor of VT/VF in the multivariable model. DCE score [median 6 (1–10.5) vs. 12 (6–18); P = 0.001], mean and maximal left ventricular (LV) wall thickness (MaxLVWT), as well as LV mass index were significantly greater among patients at risk for SCD ( n = 51) compared with the remaining 57 patients at low risk. DCE score and MaxLVWT were independent predictors of SCD risk. Conclusion In HCM patients several CMR parameters are associated with risk for SCD. A semi-quantitative index of DCE is a significant multivariable predictor of both clinical VT/VF and of risk for SCD and may contribute to risk assessment in borderline or controversial cases.