BACKGROUND:An aberrant subclavian artery (ASA) (or lusoria) is the most common congenital anomaly of the aortic arch (0.5%-2.2%; female-to-male ratio 2:1 to 3:1). ASA can become aneurysmal and result in dissection, involving Kommerell's diverticulum when present and the aorta. Data of its significance in genetic arteriopathies are not available. OBJECTIVES:The purpose of this study was to assess the prevalence and complications of ASA in gene-positive and -negative nonatherosclerotic arteriopathies. MATERIALS:The series includes 1,418 consecutive patients with gene-positive (n = 854) and gene-negative arteriopathies (n = 564) diagnosed as part of institutional work-up for nonatherosclerotic syndromic and nonsyndromic arteriopathies. Comprehensive evaluation includes genetic counseling, next-generation sequencing multigene testing, cardiovascular and multidisciplinary assessment, and whole-body computed tomography angiography. RESULTS:ASA was found in 34 of 1,418 cases (2.4%), with a similar prevalence in gene-positive (n = 21 of 854, 2.5%) and gene-negative (n = 13 of 564, 2.3%) arteriopathies. Of the former 21 patients, 14 had Marfan syndrome, 5 had Loeys-Dietz syndrome, 1 had type-IV Ehlers-Danlos syndrome, and 1 had periventricular heterotopia type 1. ASA did not segregate with genetic defects. Dissection occurred in 5 of 21 patients with genetic arteriopathies (23.8%; 2 Marfan syndrome and 3 Loeys-Dietz syndrome), all with associated Kommerell's diverticulum. No dissections occurred in gene-negative patients. At baseline, none of the 5 patients with ASA dissection fulfilled criteria for elective repair according to guidelines. CONCLUSIONS:The risk of complications of ASA is higher in patients with genetic arteriopathies and is difficult to predict. In these diseases, imaging of the supra-aortic trunks should enter baseline investigations. Determination of precise indications for repair can prevent unexpected acute events such as those described.

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.

In patients with healed myocardial infarction, the left ventricular ejection fraction is characterized by low sensitivity and specificity in the prediction of future malignant arrhythmias. Thus, there is the need for new parameters in daily practice to perform arrhythmic risk stratification. The aim of this study is to identify some features of proarrhythmic geometric configurations of scars and border zones (BZ), by means of numerical simulations based on left ventricular models derived from post myocardial infarction patients. Two patients with similar clinical characteristics were included in this study. Both patients exhibited left ventricular scars characterized by subendo- and subepicardial BZ and a transmural BZ isthmus. The scar of patient #1 was significantly larger than that of patient #2, whereas the transmural BZ isthmus and the subdendo- and subepicardial BZs of patient #2 were thicker than those of patient #1. Patient #1 was positive at electrophysiologic testing, whereas patient #2 was negative. Based on the cardiac magnetic resonance (CMR) data, we developed a geometric model of the left ventricles of the two patients, taking into account the position, extent, and topological features of scars and BZ. The numerical simulations were based on the anisotropic monodomain model of electrocardiology. In the model of patient #1, sustained ventricular tachycardia (VT) was inducible by an S2 stimulus delivered at any of the six stimulation sites considered, while in the model of patient #2 we were not able to induce sustained VT. In the model of patient #1, making the subendo- and subepicardial BZs as thick as those of patient #2 did not affect the inducibility and maintenance of VT. On the other hand, in the model of patient #2, making the subendo- and subepicardial BZs as thin as those of patient #1 yielded sustained VT. In conclusion, the results show that the numerical simulations have an effective predictive capability in discriminating patients at high arrhythmic risk. The extent of the infarct scar and the presence of transmural BZ isthmuses and thin subendo- and subepicardial BZs promote sustained VT.

Objectives The aim of this study was to describe the radiological features of chronic thromboembolic pulmonary disease (CTEPD), not yet systematically described in the literature. Furthermore, we compared vascular scores between CTEPD and chronic thromboembolic pulmonary hypertension (CTEPH) patients, trying to explain why pulmonary hypertension does not develop at rest in CTEPD patients. Methods Eighty-five patients (40 CTEPD, 45 CTEPH) referred to our centre for pulmonary endarterectomy underwent dual-energy computed tomography pulmonary angiography (DE-CTPA) with iodine perfusion maps; other 6 CTEPD patients underwent single-source CTPA. CT scans were reviewed independently by an experienced cardiothoracic radiologist and a radiology resident to evaluate scores of vascular obstruction, hypoperfusion and mosaic attenuation, signs of pulmonary hypertension and other CT features typical of CTEPH. Results Vascular obstruction burden was similar in the two groups ( p = 0.073), but CTEPD patients have a smaller extension of perfusion defects in the iodine map ( p = 0.009) and a smaller number of these patients had mosaic attenuation ( p < 0.001) than CTEPH patients, suggesting the absence of microvascular disease. Furthermore, as expected, the two groups were significantly different considering the indirect signs of pulmonary hypertension ( p < 0.001). Conclusions CTEPD and CTEPH patients have significantly different radiological characteristics, in terms of signs of pulmonary hypertension, mosaic attenuation and iodine map perfusion extension. Importantly, our results suggest that the absence of peripheral microvascular disease, even in presence of an important thrombotic burden, might be the reason for the absence of pulmonary hypertension in CTEPD. Key Points • CTEPD and CTEPH patients have significantly different radiological characteristics . • The absence of peripheral microvascular disease might be the reason for the absence of pulmonary hypertension in CTEPD .

Pulmonary Hypertension (PH) is defined as an increase in the mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, as assessed by the right heart catheterization (RHC), and it may complicate most cardiovascular and respiratory diseases. PH is clinically classified into five groups according to similar clinical presentation, pathological findings, hemodynamic characteristics and treatment strategy. Interstitial Lung Disease (ILD) are listed in Group 3 (due to lung diseases and/or hypoxia) and Group 5 (with unclear and/or multifactorial mechanisms), based on the physio-pathological mechanisms of PH development, with a prevalent precapillary hemodynamic profile. In patients with ILD, the presence of PH is commonly associated with the advanced phase of the disease and an increase in morbidity and mortality. In the context of PH associated with parenchymal lung diseases, the diagnosis is relevant not only for the patients’ prognosis but also for the treatment choice because the use of vasodilator therapy in patients with chronic lung diseases is generally not recommended. Therefore, the role of the radiologist is to look for computed tomography (CT) signs suggestive of PH and to contribute to the identification of the possible cause of PH, becoming familiar with clinical, hemodynamical and physiological aspects of the different diseases.

BACKGROUND:Few data are known regarding the molecular features and patterns of growth and presentation which characterize those lung neoplastic lesions presenting as non-solid nodules (NSN). METHODS:We retrospectively reviewed two different cohorts of NSNs detected by CT scan which, after transthoracic fine-needle aspiration (FNA) and core needle biopsy (CNB) received a final diagnosis of malignancy. All the enrolled patients were then addressed to surgical removal of lung cancer nodules or to exclusive radiotherapy. Exhaustive clinical and radiological features were available for each case. RESULTS:In all 62 analysed cases the diagnosis of adenocarcinoma (ADC) was reached. In cytologic samples, EGFR activating mutations were identified in 2 of the 28 cases (7%); no case showed ALK/EML4 or ROS1 translocations. In the histologic samples EGFR activating mutation were found in 4 out of 25 cases (16%). PD-L1 immunostains could be evaluated in 30 cytologic samples, while the remaining 7 did not reach the cellularity threshold for evaluation. TPS was < 1% in 26 cases, > 1% < 50% in 3, and > 50% in 1. All surgical samples showed TPS < 1%. Of the 17 cases that could be evaluated on both samples, 15 were concordantly TPS 0, and 2 showed TPS > 1% < 50 on the biopsy samples. TPS was < 1% in 14 cases, > 1%/< 5% in 4 cases, > 5%/< 50% in 2 cases, > 50% in 1 case. CONCLUSIONS:Overall PD-L1 immunostaining documented the predominance of low/negative TPS, with high concordance in FNA and corresponding surgical samples. It can be hypothesized that lung ADC with NSN pattern and predominant in situ (i.e. lepidic) components represent the first steps in tumor progression, which have not yet triggered immune response, and/or have not accumulated a significant rate of mutations and neoantigen production, or that they belong to the infiltrated-excluded category of tumors. The negative prediction of response to immunomodulating therapy underlines the importance of rapid surgical treatment of these lesions. Notably, cell block cytology seems to fail in detecting EGFR mutations, thus suggesting that this kind of sampling technique should be not adequate in case of DNA direct sequencing.

Introduction: Data on disease progression in IPAF and evolution in rheumatic disease (RD) are conflicting [Alevizos, M.C. et al Rheum 2019;10.1093; Tirelli C. et al Front Med 2020;7:11]. Aims: Aim of the study was to prospectively analyze clinical, functional and radiologic data of 46 IPAF patients (pt) in follow-up (FU) in a multidisciplinary setting and determine RD progression rates. Methods: This is a single center prospective study (mean FU 31 months (m), SD ± 22m) focused on rates of RD development, comparing IPAF and RD for fibrosis progression or pattern change at high resolution CT (HRCT), pulmonary function test (PFT: FVC, DLCO), exacerbations and respiratory failure requiring Oxygen therapy (OT). Student’s, χ2, and Fisher’s exact tests were applied. Results: 28.3% IPAF progressed to RD at FU (fig.1). Despite immunosuppression PFT declined (12 m loss of >10% FVC, DLCO) in 37.5% IPAF and 30.8% RD (p=0.95) while HRCT progression (fig.2) was found in 58.7% cases (IPAF 65.3%, RD 83.3%; p=0.26). Mean HRCT progression time was 21.3 ±15.6 m, not different in IPAF and RD (p=0.86). At FU 30% pt needed OT (IPAF 33.3%, RD 30.8%; p=0.87). 5 pt (11%; 3 IPAF, 2 RD) died for exacerbations. Conclusions: During FU a consistent number of IPAF (28.3%) progressed to overt RD, confirming IPAF dynamic nature. HRCT and PFT progression was detectable both among IPAF and RD. Figures:

To analyze the most frequent radiographic features of COVID-19 pneumonia and assess the effectiveness of chest X-ray (CXR) in detecting pulmonary alterations. 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. 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 < 0.01). Distribution among groups was as follows: reticular alteration (group A, 70.9%; group B, 72.2%; group C, 57.9%; group D, 46.1%), GGO (group A, 67.7%; group B, 62.9%; group C, 71%; group D, 76.9%), and consolidation (group A, 35.5%; group B, 31.4%; group C, 47.8%; group D, 38.5%). Alterations were bilateral in 73.3%. Upper, middle, and lower fields were involved in 36.7%, 79.4%, and 87.8%, respectively. Lesions were peripheral in 49.4%, central in 11.1%, or both in 39.4%. Upper fields and central zones were significantly less involved (p < 0.01). The most frequent lesions in COVID-19 patients were GGO (intermediate/late phase) and reticular alteration (early phase) while consolidation gradually increased over time. The most frequent distribution was bilateral, peripheral, and with middle/lower predominance. Overall rate of negative CXR was 25%, which progressively decreased over time. • The predominant lung changes were GGO and reticular alteration, while consolidation was less frequent. • The typical distribution pattern was bilateral, peripheral, or both peripheral and central and involved predominantly the lower and middle fields. • Chest radiography showed lung abnormalities in 75% of patients with confirmed SARS-CoV-2 infection, range varied from 63.3 to 83.9%, respectively, at 0–2 days and > 9 days from the onset of symptoms.

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.

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.

Cystic echinococcosis (CE) is a zoonosis caused by the larval stage of the tapeworm Echinococcus granulosus. In humans, the infection induces the formation of parasitic cysts mostly in the liver and lungs, but virtually any organ can be affected. CE of the bone is one of the rarest forms of the disease, yet it is also extremely debilitating for patients and hard to manage for clinicians. Unlike abdominal CE, there is currently no expert consensus on the management of bone CE. In this study, we conducted a survey of the clinical records of seven European referral centers for the management of patients with CE and retrieved data on the clinical management of 32 patients with a diagnosis of bone CE. Our survey confirmed that the patients endured chronic debilitating disease with a high rate of complications (84%). We also found that diagnostic approaches were highly heterogeneous. Surgery was extensively used to treat these patients, as well as albendazole, occasionally combined with praziquantel or nitaxozanide. Treatment was curative only for two patients, with one requiring amputation of the involved bone. Our survey highlights the need to conduct systematic studies on bone CE, both retrospectively and prospectively.

Few data are known on the molecular features characterizing neoplastic lesions presenting as non-solid nodules (NSN), the specific pattern of tumour presentation and growth. We retrospectively reviewed the CT scans of 36 patients who underwent transthoracic CT-guided fine needle aspiration of suspected NS pulmonary lesions from october 2016 to december 2017 who received a final diagnosis of malignancy. Exhaustive clinical and radiological features were available for each case. In all cases the diagnosis of adenocarcinoma (ADC) was reached. EGFR mutations were identified in 2 of the 28 cases (7%) with adequate material (one exon 19 deletion and L858R); no case showed ALK/EML1 or ROS1 translocations. PD-L1 immunostains could be evaluated in 30 cytological samples, while the remaining 7 did not reach the cellularity threshold for evaluation. TPS was <1% in 26 cases, >1%<50% in 3, and >50% in 1. All surgical samples showed TPS <1%. Of the 17 cases that could be evaluated on both samples, 15 were concordantly TPS 0, and 2 showed TPS >1%<50 on the biopsy samples. Overall PD-L1 immunostains documented the predominance of low/negative TPS, with high concordance in FNA and corresponding surgical samples. It can be hypothesized that lung ADC with NSN pattern and predominant in situ (i.e. lepidic) components represent the first steps in tumor progression, which have not yet triggered immune response, and/or have not accumulated a significant rate of mutations and neoantigen production, or that they belong to the infiltrated-excluded category of tumors. The negative prediction of response to immunomodulating therapy underlines the importance of rapid surgical treatment of these lesions

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.

Ferlini, Marcoa; Dore, Robertob; Iannopollo, Gianmarcoa; De Ferrari, Gaetano M.c; Oltrona Visconti, Luigia Author Information

Malignant pleural mesothelioma is an aggressive cancer with an ominous prognosis due to the absence of effective treatments. MPM diagnosis is often delayed consequently to insidious and non-specific symptoms. Past asbestos exposure represents the major risk factor for MPM onset, although the carcinogenic mechanisms of asbestos is not fully understood. In those cases, the parietal pleura is the target of disease arousal. As opposite, here we present and discuss two clinical cases of MPM who firstly presented as primary spontaneous pneumothorax. In both cases lung nodules where detected, allowing in one of them a misdiagnosis of lung cancer. None of the patients referred exposure to asbestos fibers. We strongly suggest considering MPM among the differential diagnosis of spontaneous pneumothorax in elderly population. Moreover, in this setting, a main pathogenic involvement of the visceral pleura can be hypothesized.

Marino, Marcelloa; Cacucci, Michelea; Catanoso, Antonioa; Demarchi, Andreab; Dore, Robertoc; Landolina, Maurizioa Author Information

Not-solid and part-solid lung nodules often reveal as neoplastic when biopsed. This study aimed at analyzing the radiological and molecular aspects of a series of not-solid neoplastic nodules detected a to ur Institution to generate their matched pathologic and radiologic profiles. We studied a consecutive series of 28 cases from oct 2016 to dec 2017 which underwent transthoracic CT-guided biopsy. For each case exhaustive clinical, molecular and tomographic data were available. Eighteen out of the 28 patients were males, 10 females; mean age at diagnosis was 71 years. In all cases the pathologic diagnosis corresponded to ADC. One nodule carried deleted ex 19 of EGFR (female, past smoker patient), all featured wt ALK and ROS1 profiles. In 24 out of 28, PDL1 expression <1%. A small increase in dose (150 mGy vs standard 100 mGy) was required. Acinar components seemed to have a poorer performance on cytology and nodules with mainly acinar components were only suspect to be neoplastic on cytology and operatory confirmation was needed. Lepidic and papillary components generally got a precise diagnosis of ADC directly on cytology. No increase of postoperative complication was reported. Tumor cytology correctly predicts its histology on radical surgery. Parenchymal destruction correlates with histology: there was a significant difference between destructive nodules (80% moderately differentiated) and non-destructive nodules (50% moderately differentiated – 50% well differentiated). No acinar components in non-solid nodules as acinar components could account for solid portion of the mixed sub solid-nodules

BACKGROUND:Mesothelioma is a rare neoplasm which commonly develops in the pleura of people exposed to asbestos. Pericardial mesothelioma accounts for only 0.7 % of all malignant mesotheliomas and it usually presents with pericardial effusion, mimicking serositis. To date, there are approximately 200 cases of pericardial mesothelioma described in the medical literature, and little knowledge exists about the systemic manifestations of this pathology. The first and only described case of pericardial mesothelioma with autoimmune features dates back to 1984 and, in our case report, we describe the second.CASE PRESENTATION:We report a case of a 45-year-old white woman whose pericardial mesothelioma was initially misdiagnosed as pericardial involvement of an autoimmune disease (systemic lupus erythematosus). After several relapses of pericardial effusion, a computed tomography scan and a biopsy with histological analysis were performed revealing neoplastic growth.CONCLUSIONS:We describe a rare case of pericardial mesothelioma in a patient with a clinical presentation compatible with lupus serositis. Clinicians should consider malignant mesothelioma in the differential diagnosis of pericardial effusion, especially when it is recurrent and not clearly explained by other causes. Cytological samples should always be obtained and, if imaging tools are suggestive for solid processes, histological confirmation is mandatory.

Introduction The cryoballoon (CB) technology nowadays represents a valid alternative to the radiofrequency for pulmonary vein (PV) isolation. Transient ST elevation has been reported as a rare adverse event occurring during atrial fibrillation (AF) ablation and is mostly related to air embolism during contrast injection. However, the present case describes the occurrence of ST elevation during CB ablation secondary to the proximity of a venous bypass graft for the distal right coronary artery to the ostium of left inferior pulmonary vein (LIPV).