Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia: Correlations between computed tomography findings and cerebral complications.

OBJECTIVES: Computed tomography (CT) is the modality of choice to characterise pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). Our objective was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke. METHODS: This retrospective study included patients with HHT-related PAVMs. CT results, i.e. PAVM presentation (unique, multiple, disseminated or diffuse), the number of PAVMs and the largest feeding artery size, were correlated to prevalence of ischaemic stroke and brain abscess. All CTs were reviewed in consensus by two radiologists. RESULTS: Of 170 patients, 73 patients had unique (42.9 %), 49 multiple (28.8 %), 36 disseminated (21.2 %) and 12 diffuse (7.1 %) PAVMs. Fifteen patients presented with brain abscess; 26 patients presented with ischaemic stroke. The number of PAVMs was significantly correlated with brain abscess (11.5 vs. 6.2, respectively; p=0.025). The mean diameter of the largest feeding artery was significantly correlated with ischaemic stroke frequency (4.9 vs. 3.2 mm, respectively; p=0.0098). CONCLUSIONS: The number of PAVMs correlated significantly with risk of brain abscess, and a larger feeding artery significantly with more ischaemic strokes. These findings can lead to a better recognition and management of the PAVMs at risk of cerebral complications. KEY POINTS: • Chest CT helps clinicians to facilitate appropriate PAVM management strategies. • Pulmonary arteriovenous malformation CT findings are correlated with risk of cerebral complications. • Risk of brain abscess is significantly correlated with number of PAVMs. • Risk of ischaemic stroke is significantly correlated with large feeding artery PAVMs. • Prevalence of observed of brain abscess and ischaemic stroke is 26 %.

A retrospective study of factors associated with treatment decision for nontuberculous mycobacterial lung disease in adults without altered systemic immunity.

BACKGROUND: Nontuberculous mycobacteria (NTM) lung diseases are increasingly recognized as chronic opportunistic infections, occurring in individuals with a wide variety of underlying conditions. In the absence of systemic immunodeficiency, decision of NTM lung disease treatment must relies on a careful risk/benefit assessment, given the requirement of long-term administration of multidrug therapies supported by limited evidence. The primary objective was to identify the factors associated with anti-NTM treatment initiation. Clinical and radiological outcome upon treatment were studied. METHODS: This retrospective, single center study (2013-2016, 45 months) addressed the criteria supporting treatment decision among adults with NTM lung disease without systemic immunodeficiency at our institution, with the assigned goal to harmonize the practice. All patients matched the current international definitions of NTM lung disease according to the American Thoracic Society criteria. Factors associated with anti-NTM treatment were investigated by conditional logistic regression. Clinical and radiological outcomes of treated and untreated NTM-disease cases were examined. Mortality rate was assessed. An expert radiologist conducted a blinded computed tomography (CT)-scan review of the treated and untreated patients. RESULTS: Among 51 cases of NTM lung diseases, 25 (49%) received anti-NTM treatment. In univariate analysis, a body mass index (BMI) < 18 kg/m2 (odds ratio (OR), 4.2 [95% confidence interval (CI) 1.2-15.2]; p = 0.042), hemoptysis (OR, 11.8 [95% CI 1.35-12.9]; p = 0.026), excavation(s) (OR, 4.8 [95% CI 1.4-16.4], p = 0.012), prior anti-NTM treatment (OR, 5.65 [95% CI 1.06-29.9]; p = 0.042), Aspergillus spp. co-infection (OR, 6.3 [95% CI 1.8-22.2]; p = 0.004) were associated with treatment initiation. In multivariate analysis, Aspergillus spp. co-infection was the only independent determinant of treatment initiation (OR, 5.3 [95% CI 1.1-25.4]; p = 0.036). Twenty-one (81%) patients received ≥3 anti-NTM drugs. Median treatment duration and follow-up were 36.3 (interquartile range [IQR], 13.1-64.4) weeks and 17.1 (IQR, 8.7-27.1) months, respectively. Regarding radiological outcome, 85 CT-scans were reviewed, showing similar rates of regression or stabilization in treated and untreated patients. Overall mortality rate was not different in treated and untreated patients. CONCLUSION: The most relevant variable associated with anti-NTM treatment initiation was Aspergillus spp. co-infection. Radiological regression or stabilization of pulmonary lesions was not different between the treated and untreated patients.

Vein Diameter on Unenhanced Multidetector CT Predicts Reperfusion of Pulmonary Arteriovenous Malformation after Embolotherapy.

OBJECTIVE: To evaluate the value of the diameter of the draining vein of pulmonary arteriovenous malformation (PAVM) on unenhanced chest MDCT in diagnosing reperfusion after percutaneous vaso-occlusion therapy. METHODS: We retrospectively reviewed our long-term experience of patients with hereditary haemorrhagic telangiectasia and selected cases on the following criteria: an initial pulmonary angiogram with embolotherapy of at least one PAVM, a follow-up MDCT examination in the following year followed by a second pulmonary angiogram with embolotherapy if needed. Follow-up unenhanced chest MDCT examinations were analyzed blindly from results of pulmonary artery angiogram and clinical data, the diameter of the efferent vein close to the PAVM sac was measured, then compared to those of pulmonary artery angiogram as a gold standard. RESULTS: Eighty-eight of 100 patients met inclusion criteria, in whom 62 of 176 PAVMs were reperfused at angiogram. The mean diameter of the efferent vein on MDCT was 4.3 ± 2.1 mm in patent PAVMs and 1.8 ± 0.9 mm in non-patent PAVMs (p < 0.0001). The optimal cutoff diameter based on ROC analysis was 2.5 mm (sensitivity = 98.4 %; specificity = 87.7 %). CONCLUSION: A diameter of the draining vein of PAVM of 2.5 mm or greater on unenhanced MDCT is a strong predictor of reperfusion. KEY POINTS: • Diameter of draining vein of 2.5 mm or greater is associated with reperfusion. • Unenhanced chest MDCT predicts reperfusion of PAVMs with good sensitivity and specificity. • Unenhanced MDCT can guide a decision of repeat pulmonary angiogram and embolotherapy. • The mean vein diameter change of PAVMs occluded at follow-up is 3.8 mm. • Overall success rate after a median of 6 months embolotherapy was 64.7 %.

It Looks Like a Spinal Cord Tumor but It Is Not.

Differentiating neoplastic from non-neoplastic spinal cord pathologies may be challenging due to overlapping clinical and radiological features. Spinal cord tumors, which comprise only 2-4% of central nervous system tumors, are rarer than non-tumoral myelopathies of inflammatory, vascular, or infectious origins. The risk of neurological deterioration and the high rate of false negatives or misdiagnoses associated with spinal cord biopsies require a cautious approach. Facing a spinal cord lesion, prioritizing more common non-surgical myelopathies in differential diagnoses is essential. A comprehensive radiological diagnostic approach is mandatory to identify spinal cord tumor mimics. The diagnostic process involves a multi-step approach: detecting lesions primarily using MRI techniques, precise localization of lesions, assessing lesion signal intensity characteristics, and searching for potentially associated anomalies at spinal cord and cerebral MRI. This review aims to delineate the radiological diagnostic approach for spinal cord lesions that may mimic tumors and briefly highlight the primary pathologies behind these lesions.

Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease.

OBJECTIVE: Connective tissue diseases (CTDs) are associated with several interstitial lung diseases. The aim of this study was to describe the recently individualized syndrome of combined pulmonary fibrosis and emphysema (CPFE) in a population of patients with CTD. METHODS: In this multicenter study, we retrospectively investigated data from patients with CTD who also have CPFE. The demographic characteristics of the patients, the results of pulmonary function testing, high-resolution computed tomography, lung biopsy, and treatment, and the outcomes of the patients were analyzed. RESULTS: Data from 34 patients with CTD who were followed up for a mean±SD duration of 8.3±7.0 years were analyzed. Eighteen of the patients had rheumatoid arthritis (RA), 10 had systemic sclerosis (SSc), 4 had mixed or overlap CTD, and 2 had other CTDs. The mean±SD age of the patients was 57±11 years, 23 were men, and 30 were current or former smokers. High-resolution computed tomography revealed emphysema of the upper lung zones and pulmonary fibrosis of the lower zones in all patients, and all patients exhibited dyspnea during exercise. Moderately impaired pulmonary function test results and markedly reduced carbon monoxide transfer capacity were observed. Five patients with SSc exhibited pulmonary hypertension. Four patients died during followup. Patients with CTD and CPFE were significantly younger than an historical control group of patients with idiopathic CPFE and more frequently were female. In addition, patients with CTD and CPFE had higher lung volumes, lower diffusion capacity, higher pulmonary pressures, and more frequently were male than those with CTD and lung fibrosis without emphysema. CONCLUSION: CPFE warrants inclusion as a novel, distinct pulmonary manifestation within the spectrum of CTD-associated lung diseases in smokers or former smokers, especially in patients with RA or SSc.

Hamartochondroma Pleural Lesion Mimicking Liposarcoma: A Case Report.

Heterogeneous masses developing in the pleural cavity are most often malignant and can pose diagnostic challenges. Fibrous tumors of the pleura, liposarcoma, thymoma or lipoma most frequently affect this anatomic area. Surgical exploration and resection are often mandatory to make the definitive diagnosis. We report the case of a 54-year-old women who presented with an epigastric and right sub costal pain. A complete preoperative workup revealed a large tissular and fatty mass in the right costo-diaphragmatic angle suggestive of liposarcoma. Surgical resection resulted in the surprising diagnosis of hamartochondroma.

Clinical practices for non-neoplastic thymic lesions: Results from a multidisciplinary cohort.

BACKGROUND: Non-neoplastic thymic lesions are uncommon findings that corresponds to multiple histological and clinical entities that may be difficult to differentiate from thymic malignancies. In this study, our main objective was to describe the clinical, imaging and pathological characteristics of non-neoplastic thymic lesions in a large cohort of patients. We also aimed at understanding the key factors that led to a decision to surgically resect those lesions. METHODS AND MATERIALS: This is an observational, retrospective study. We enrolled both patients with non-neoplastic thymic lesions - normal thymus, thymic lymphoid/non-lymphoid hyperplasia, and thymic cysts - that had been pathologically-confirmed after surgical resection - , and patients with a thymic lesion that was never operated, based on imaging follow-up. RESULTS: A total of 128 patients were included, 88 of whom underwent surgical resection of the lesion (69%), and 40 patients (31%) had follow-up without surgery. Discovery of the lesion was incidental in 69 (54%) cases; thoracic magnetic resonance imaging was performed in 33 (26%) cases, 85% of which showed apparent decrease in the lesion signal intensity in phase opposition at chemical shift sequences. In the 88 operated patients, there were 34 (39%) normal thymuses, 29 (33%) lymphoid hyperplasias, 6 (7%) non-lymphoid thymic hyperplasias, and 19 (22%) thymic cysts. In the 40 non-operated patients, a major driver for the decision of follow-up was the decrease in the lesion signal intensity in phase opposition at chemical shift sequences, observed in 68% of cases; imaging follow-up of these lesions showed sustained regression in the majority of the cases. CONCLUSIONS: The management of benign thymic lesions requires multidisciplinary assessment. A strategy that integrates clinical and imaging features, including chemical-shift sequences at magnetic resonance imaging, as well as follow-up, allows a better selection of the patients for surgery.

Embolization of Recurrent Pulmonary Arteriovenous Malformations by Ethylene Vinyl Alcohol Copolymer (Onyx®) in Hereditary Hemorrhagic Telangiectasia: Safety and Efficacy.

OBJECTIVES: To evaluate short- and long-term safety and efficacy of embolization with Onyx® for recurrent pulmonary arteriovenous malformations (PAVMs) in hereditary hemorrhagic telangiectasia (HHT). METHODS: In total, 45 consecutive patients (51% women, mean (SD) age 53 (18) years) with HHT referred to a reference center for treatment of recurrent PAVM were retrospectively included from April 2014 to July 2021. Inclusion criteria included evidence of PAVM recurrence on CT or angiography, embolization using Onyx® and a minimal 1-year-follow-up CT or angiography. Success was defined based on the standard of reference criteria on unenhanced CT or pulmonary angiography if a recurrence was suspected. PAVMs were analyzed in consensus by two radiologists. The absence of safety distance, as defined by a too-short distance for coil/plug deployment, i.e., between 0.5 and 1 cm, between the proximal extremity of the primary embolic material used and a healthy upstream artery branch, was reported. RESULTS: In total, 70 PAVM were analyzed. Mean (SD) follow-up was 3 (1.3) years. Safety distance criteria were missing in 33 (47%) PAVMs. All procedures were technically successful, with a short-term occlusion rate of 100% using a mean (SD) of 0.6 (0.5) mL of Onyx®. The long-term occlusion rate was 60%. No immediate complication directly related to embolization was reported, nor was any severe long-term complication such as strokes or cerebral abscesses. CONCLUSIONS: In HHT, treatment of recurrent PAVM with Onyx® showed satisfactory safety and efficacy, with an immediate occlusion rate of 100% and a long-term rate of 60%.

First Case of Complete Percutaneous Correction of Isolated Divided Atrium (or Cor Triatriatum) Dexter.

Divided atrium (or Cor triatriatum) dexter (DAD) is a rare congenital cardiopathy, usually associated with other anomalies; isolated forms are even rarer. We report the case of an 84-year-old woman presenting with isolated DAD complicated by right-left atrial shunt through patent foramen ovale (PFO), revealed by right cardiac failure and severe hypoxemia. Late destabilization was caused by total superior vena cava thrombosis, related to her pacemaker. Given the overall context, complete percutaneous treatment was performed, associating PFO occlusion and stent implantation in the right atrial membrane, providing total regression of symptoms. Thus, percutaneous correction of DAD appears to be safe and effective.

Epicardial carvernous hemangioma: The diagnostic challenge of a middle mediastinal mass.

Primary tumors in the middle mediastinum are rare and pose diagnostic challenges. Lymphomas, mediastinal cysts and thymomas most frequently affect this anatomic area. Primary cardiac tumors are rare and constitute a differential diagnosis for the inferior middle mediastinum. Surgical exploration and resection is often mandatory in order to make a definitive diagnosis. Here, we report the case of a 69 year-old women who presented with persistent dyspnea. A complete preoperative workup revealed a large tissular mass adjacent to the right atrium. A diagnosis of a typical epicardial cavernous hemangioma was made following surgical resection.

Feasibility of lung imaging with a large field-of-view spectral photon-counting CT system.

PURPOSE: The purpose of this study was to characterize the technical capabilities and feasibility of a large field-of-view clinical spectral photon-counting computed tomography (SPCCT) prototype for high-resolution (HR) lung imaging. MATERIALS AND METHODS: Measurement of modulation transfer function (MTF) and acquisition of a line pairs phantom were performed. An anthropomorphic lung nodule phantom was scanned with standard (120kVp, 62mAs), low (120kVp, 11mAs), and ultra-low (80kVp, 3mAs) radiation doses. A human volunteer underwent standard (120kVp, 63mAs) and low (120kVp, 11mAs) dose scans after approval by the ethics committee. HR images were reconstructed with 1024 matrix, 300mm field of view and 0.25mm slice thickness using a filtered-back projection (FBP) and two levels of iterative reconstruction (iDose 5 and 9). The conspicuity and sharpness of various lung structures (distal airways, vessels, fissures and proximal bronchial wall), image noise, and overall image quality were independently analyzed by three radiologists and compared to a previous HR lung CT examination of the same volunteer performed with a conventional CT equipped with energy integrating detectors (120kVp, 10mAs, FBP). RESULTS: Ten percent MTF was measured at 22.3lp/cm with a cut-off at 31lp/cm. Up to 28lp/cm were depicted. While mixed and solid nodules were easily depicted on standard and low-dose phantom images, higher iDose levels and slice thicknesses (1mm) were needed to visualize ground-glass components on ultra-low-dose images. Standard dose SPCCT images of in vivo lung structures were of greater conspicuity and sharpness, with greater overall image quality, and similar image noise (despite a flux reduction of 23%) to conventional CT images. Low-dose SPCCT images were of greater or similar conspicuity and sharpness, similar overall image quality, and lower but acceptable image noise (despite a flux reduction of 89%). CONCLUSIONS: A large field-of-view SPCCT prototype demonstrates HR technical capabilities and high image quality for high resolution lung CT in human.

Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease.

OBJECTIVE: Patients with systemic sclerosis and both pulmonary hypertension and interstitial lung disease (SSc-PH-ILD) generally carry a worse prognosis than patients with SSc and pulmonary arterial hypertension (SSc-PAH) without ILD. There is no evidence of the efficacy of PAH therapies in SSc-PH-ILD. We undertook this study to compare survival of and response to treatment in patients with SSc-PH-ILD and those with SSc-PAH. METHODS: We analyzed 128 patients (66 with SSc-PH-ILD and 62 with SSc-PAH) from 15 centers, in whom PH was diagnosed by right-sided heart catheterization; they were prospectively included in the PH registry. All patients received PAH-specific therapy. Computed tomography of the chest was used to confirm or exclude ILD. RESULTS: At baseline, patients with SSc-PH-ILD had less severe hemodynamic impairment than those with SSc-PAH (pulmonary vascular resistance 5.7 Wood units versus 8.7 Wood units; P = 0.0005) and lower diffusing capacity for carbon monoxide (median 25% [interquartile range (IQR) 18%, 35%] versus 40% [IQR 31%, 51%]; P = 0.0005). Additionally, patients with SSc-PH-ILD had increased mortality (8.1% at 1 year, 21.2% at 2 years, and 41.5% at 3 years) compared to those with SSc-PAH (4.1%, 8.7%, and 21.4%, respectively; P = 0.04). Upon treatment with PAH-targeted therapy, no improvement in the 6-minute walk distance was observed in either group. Improvement in the World Health Organization functional class was observed less frequently in patients with SSc-ILD-PH compared to those with SSc-PAH (13.6% versus 33.3%; P = 0.02). Hemodynamics improved similarly in both groups. CONCLUSION: ILD confers a worse prognosis to SSc-PH. Response to PAH-specific therapy is clinically poor in SSc-PH-ILD but was not found to be hemodynamically different from the response observed in SSc-PAH.

4D time-resolved magnetic resonance angiography for noninvasive assessment of pulmonary arteriovenous malformations patency.

PURPOSE: To assess the capability of four-dimensional (4D) time-resolved magnetic resonance angiography (MRA) to assess pulmonary arteriovenous malformations (PAVMs) patency by analyzing pulmonary arterial and venous enhancement kinetics. MATERIALS AND METHODS: Seven patients with eight documented patent PAVMs underwent a 4D-MRA with keyhole and viewsharing compression at 3T with the following parameters: spatial resolution 0.87 × 0.87 × 1.4 mm(3); field of view 500 × 350 × 238 mm(3); dynamic scan time (temporal resolution) 1.2 seconds; total acquisition time 18.1 seconds for six dynamic datasets (6 × 1.2 sec + reference scan: 10.9 sec). All images were reviewed by two experienced radiologists. Image quality was rated on a qualitative 5-point scale (1: not assessable to 5: excellent). Signal value was measured on cross-sectional planes for the afferent arteries and efferent veins of the PAVM, and for normal reference healthy arteries and veins. The difference in time to peak for each coupled artery/vein (dTTPav) was calculated and compared with a Mann-Whitney test between PAVMs and reference vessels. RESULTS: Mean image quality was 3.2 ± 0.9. dTTPav was significantly smaller in PAVMs (0.15 ± 0.76 sec) than in reference vessels (3.75 ± 1.62 sec), P < 0.001. CONCLUSION: 4D-MRA is a promising tool for noninvasive assessment of PAVM patency.

Rapid-clearance iron nanoparticles for inflammation imaging of atherosclerotic plaque: initial experience in animal model.

PURPOSE: To evaluate the use of a recently developed fast-clearing ultrasmall superparamagnetic iron oxide (USPIO) for detection of vascular inflammation in atherosclerotic plaque. MATERIALS AND METHODS: The study protocol was approved by the animal experimentation ethics committee. A recently introduced USPIO, P904, and a reference-standard USPIO, ferumoxtran-10, were tested in a rabbit model of induced aortic atherosclerosis. In vivo magnetic resonance (MR) angiography and T2*-weighted plaque MR imaging were performed at baseline and after administration of P904 and ferumoxtran-10 (administered dose for both, 1000 micromol of iron per kilogram of body weight) in 26 hyperlipidemic New Zealand white rabbits. The variation in vessel wall area over time was evaluated with nonparametric testing. Ex vivo MR imaging findings were compared with iron content at linear regression analysis. RESULTS: With in vivo MR imaging, plaque analysis was possible as early as 24 hours after P904 injection. The authors observed a 27.75% increase in vessel wall area due to susceptibility artifacts on day 2 (P = .04) and a 38.81% increase on day 3 (P = .04) after P904 administration compared with a 44.5% increase in vessel wall area on day 7 (P = .04) and a 34.8% increase on day 10 (P = .22) after ferumoxtran-10 administration. These susceptibility artifacts were correlated with intraplaque iron uptake in the corresponding histologic slices. The number of pixels with signal loss on the ex vivo MR images was linearly correlated with the logarithm of the iron concentration (P = .0001; R(2) = 0.93). CONCLUSION: Plaque inflammation in rabbits can be detected earlier with P904 than with ferumoxtran-10 owing to the faster blood pharmacokinetics and the early uptake of P904 in the reticuloendothelial system. SUPPLEMENTAL MATERIAL: http://radiology.rsnajnls.org/cgi/content/full/252/2/401/DC1.

12q13.12q13.13 microdeletion encompassing ACVRL1 and SCN8A genes: Clinical report of a new contiguous gene syndrome.

Hereditary hemorrhagic telangiectasia is usually linked to the presence of a pathogenic mutation ACVRL1 or ENG. Thus, apparently there is no benefit to perform an array CGH in case of HHT. However, ENG has been involved in a contiguous gene syndrome due to a de novo 9q33.3q34.11 microdeletion. We describe here a new contiguous gene syndrome involving ACVRL1 gene. A 50-year-old female patient had a typical clinical presentation of hereditary hemorrhagic telangiectasia (HHT) with epistaxis, cutaneous-mucous telangiectases, arteriovenous malformation. She also presented a cognitive disability. Cognitive assessment showed a heterogeneous cognitive disorder predominating in the executive sphere without intellectual deficiency. She had no peculiar morphological feature. Neurological examination disclosed the presence of contralateral mirror movements during voluntary movement of each hand. A heterozygous deletion of the whole ACVRL1 gene (exons 1 to 10) was found to be responsible for the HHT features. To investigate further the dysexecutive syndrome and the mirror movements, we performed oligonucleotide array comparative genomic hybridization (array CGH) study (180K, Agilent, Santa-Clara, CA, USA). This study revealed a de novo 1.58 Mb deletion on chromosome 12q13.12q13.13 encompassing the ACVRL1 and SCN8A genes. To our knowledge, this deletion has not been previously reported and defines a new contiguous gene syndrome. The loss of one ACVRL1 allele is likely to be responsible for the HHT phenotype, while the deletion of the SCN8A gene is likely to be the cause of the mild cognitive disorder. SCN8A haploinsufficiency might also be involved in the occurrence of mirror movements. This report highlights the benefit of searching for large rearrangements in cases including unusual symptoms in association with HHT. On the other hand, an early diagnosis of 12q13.12q13.13 microdeletion based on the presence of a dysexecutive syndrome and/or mirror movement may allow to prevent HHT complications.

Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients.

BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) has recently been defined by an international Taskforce to characterize interstitial lung disease associated with systemic manifestations limited to subtle serological and clinical autoimmune abnormalities and not fulfilling the international criteria for the diagnosis of a given connective tissue disease. OBJECTIVE: to report on a series of patients with IPAF, and to compare their outcome to that of a cohort of patients with idiopathic pulmonary fibrosis (IPF). METHODS: Retrospective analysis of consecutive patients in a single institution over a 3-year period. RESULTS: Out of 778 consecutive patients with interstitial lung disease, 55% had idiopathic interstitial pneumonia (including 20.1% with IPF), 21.5% had connective tissue disease, and 7.3% had IPAF. Patients (49% of females) had a mean FVC of 64% and a mean DLco of 49%. Serologic criteria for IPAF were the most frequent (93%), followed by "morphologic" criteria (79%), and clinical criteria (47%). Fifty three percent of patients had a NSIP pattern on CT. Nailfold capillaroscopy found giant capillaries in 13/30 patients tested (23%). No significant was found in overall survival between patients with IPAF and those with IPF. CONCLUSION: The recently defined criteria for IPAF are fulfilled by a significant proportion of patients referred for interstitial lung disease. As compared to those with IPF, patients with IPAF are more frequently females, have distinctive characteristics, have relatively frequent abnormalities at nailfold capillaroscopy, with no difference in age or in overall survival. Prospective studies are needed to guide the management of IPAF.

Pathology-specific late outcome after endovascular repair of thoracic aorta: a single-centre experience.

OBJECTIVES: Endovascular treatment of thoracic aortic lesions appears to be advantageous. However, long-term outcomes remain poorly reported. This retrospective study reported 6-year outcomes of thoracic endovascular aortic repair. METHODS: A total of 74 patients underwent endovascular thoracic aorta treatments between 1999 and 2007; 13 had thoracic aortic dissections, 19 had traumatic aortic injuries, 35 had aneurysms, 6 had pseudoaneurysms and 1 had a penetrating ulcer. The mean follow-up was 66 months after 30 perioperative days. Yearly follow-ups included computed tomography angiography or magnetic resonance angiography. Patient demographics, mortality, complications and reinterventions were analysed. RESULTS: The early 30-day mortality and the overall late mortality were 9.5 (7/74) and 37.8% (28/74), respectively. Late mortality was higher in patients with aneurysms than in the other groups (20/35; 57% vs 8/39; 20.5%; P = 0.002). Aortic-related mortality occurred in 5/35 (14%) patients with aneurysms, but not in other groups (P = 0.02). No relationships among late complications were found for traumatic aortic injuries. The most common complication was an endoleak (21/74, 28.4%), which occurred more frequently with aneurysms than other disorders (18/35, 51.4% vs 3/39, 7.7%; P < 0.001). Endoleaks also occurred most frequently in aortic-related deaths (16/69 vs 5/5; P = 0.001). Type 1 endoleaks occurred significantly more often with aneurysms (13/35) than with other disorders (P = 0.004). Reintervention was required in 9 patients (12%); 8 with atherosclerotic aneurysms (8/35; 23%). A false lumen was thrombosed in 54% of dissections (7/13), and shrank in 39% (5/13). CONCLUSIONS: Long-term outcomes depended on aortic pathology. Aortic aneurysms were the most complicated and caused the highest mortality, probably due to atherosclerotic disease evolution. Patients with traumatic aortic injuries appeared to have the best long-term outcomes.

Negative Predictive Value of Transthoracic Core-Needle Biopsy: A Multicenter Study.

BACKGROUND: Specimens collected by CT scan-guided transthoracic core-needle biopsy (TTNB) are frequently used for the diagnosis of lung nodules, but the clinical value of negative results has not been sufficiently investigated. We sought to determine the negative predictive value (NPV) of TTNB specimens and investigate predictive factors of negative results. METHODS: All consecutive TTNBs performed in three centers between 2006 and 2012 were included. The medical charts of patients with nonmalignant TTNB specimens were reviewed and classified as true or false negatives. Binary logistic regression was used for multivariate analysis. RESULTS: Overall, findings from 980 TTNB specimens were included. Malignant disease was found in 79% (n = 777) of the cases, nonmalignant disease in 6% (n = 54), and "negative" results in 15% (n = 149). For the diagnosis of malignant disease, NPV was 51%. Estimated sensitivity, specificity, and accuracy were 89%, 99%, and 90%, respectively. The complication rate was 34% (life-threatening complication in 6%). In multivariate analysis, predictive factors for a false-negative result were radiologist experience (adjusted OR [AOR], 0.996; 95% CI, [0.994-0.998]), occurrence of a complication during the procedure (AOR, 1.958; 95% CI, [1.202-3.187]), and moderate to high maximum standardized uptake value on PET scan (AOR, 7.657; 95% CI, [1.737-33.763]). In 24 cases, a second TTNB was performed at the same target. The complication rate was 33%, and TTNB specimens provided diagnosis in 95% of cases with a 67% NPV. CONCLUSIONS: One-half of all "negative" TTNB specimen results were falsely negative for malignant diagnosis. Findings in tissue collected from a second TTNB at the same target provided a final diagnosis in most cases without increasing complication rates.

Synergy between stents and extracorporeal membrane oxygenation in multitrauma patients with inferior vena cava injury.

Despite the lack of evidence in the literature, we report the case of a 25-year-old man involved in a road traffic accident, who had an inferior vena cava (IVC) injury and severe lung contusion with parenchymal bleeding requiring an extracorporeal membrane oxygenation (ECMO). An emergency procedure to implant a stent graft was successful in repairing the IVC injury. Moreover, we think that ECMO, in addition to providing oxygenation, reduced bleeding by creating a negative pressure along the injured IVC. The patient was decannulated on the eighth day and discharged 31 days after the accident.