Anomalous origin of the circumflex artery from the right pulmonary artery.

Anomalous origin of the circumflex artery from the pulmonary artery (ACxAPA) is a rare but clinically significant condition in which the circumflex artery arises from either the main pulmonary artery or one of its main branches. Untreated patients with ACxAPA may develop severe heart failure or sudden cardiac death. Diagnosis is established with either catheter or CT angiography. We present a case of an adult male with no prior known cardiac history who was found to have ACxAPA after presenting to our institution in acute decompensated heart failure.

Dual Energy Technique Adds Value to Solitary Pulmonary Nodule Analysis with Dynamic Contrast-Enhanced CT: A 100 Nodule Experience.

A highly sensitive test for evaluation of solitary pulmonary nodules (SPN) involves a 5-phase, contrast enhanced CT evaluation which can be fraught with technical error. The goal of this study is to determine if qualitative evaluation of iodine maps with dual energy CT (DECT) can add value to SPN evaluation.100 patients had their SPN evaluated with traditional quantitative analysis and qualitative evaluation with iodine maps generated from DECT data acquired at 80 kVp and 140 kVp. Quantitative analysis served as the gold standard. Quantitative analysis was performed at 1, 2, 3, and 4 minutes. Qualitative Iodine maps analysis was performed at 1 and 2 minutes. Sensitivity and specificity were 63% and 95%, and 73% and 91%, respectively, at 1 and 2 minutes. Combined analysis resulted in sensitivity of 67% and specificity of 94%. Six of 7 false negatives on combined analysis were stable for 2 years and the seventh was lost to follow up after 1 year. Of 5 false positives on combined analysis; 2 were due to 'bleed-through of calcium' on iodine maps, 2 were positive on 3 and 4-minute quantitative analysis with one biopsy-proven adenocarcinoma. Qualitative analysis of Iodine maps generated using DECT data can be easily performed and may provide more effective evaluation of the solitary pulmonary nodule when combined with traditional analysis. This method warrants further investigation with larger patient populations, comparison with PET-CT, and evaluation of outcomes including long-term nodule stability and tissue diagnosis.

Neurofibromatosis from Head to Toe: What the Radiologist Needs to Know.

Neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2) are autosomal dominant inherited neurocutaneous disorders or phakomatoses secondary to mutations in the NF1 and NF2 tumor suppressor genes, respectively. Although they share a common name, NF1 and NF2 are distinct disorders with a wide range of multisystem manifestations that include benign and malignant tumors. Imaging plays an essential role in diagnosis, surveillance, and management of individuals with NF1 and NF2. Therefore, it is crucial for radiologists to be familiar with the imaging features of NF1 and NF2 to allow prompt diagnosis and appropriate management. Key manifestations of NF1 include café-au-lait macules, axillary or inguinal freckling, neurofibromas or plexiform neurofibromas, optic pathway gliomas, Lisch nodules, and osseous lesions such as sphenoid dysplasia, all of which are considered diagnostic features of NF1. Other manifestations include focal areas of signal intensity in the brain, low-grade gliomas, interstitial lung disease, various abdominopelvic neoplasms, scoliosis, and vascular dysplasia. The various NF1-associated abdominopelvic neoplasms can be categorized by their cellular origin: neurogenic neoplasms, interstitial cells of Cajal neoplasms, neuroendocrine neoplasms, and embryonal neoplasms. Malignant peripheral nerve sheath tumors and intracranial tumors are the leading contributors to mortality in NF1. Classic manifestations of NF2 include schwannomas, meningiomas, and ependymomas. However, NF2 may have shared cutaneous manifestations with NF1. Lifelong multidisciplinary management is critical for patients with either disease. The authors highlight the genetics and molecular pathogenesis, clinical and pathologic features, imaging manifestations, and multidisciplinary management and surveillance of NF1 and NF2. Online supplemental material is available for this article. ©RSNA, 2022.

Radiologic and Pathologic Correlation in EVALI.

OBJECTIVE. The purpose of this article is to characterize the appearance on CT of e-cigarette or vaping product use-associated lung injury (EVALI) in a cohort with histopathologic evidence of this disorder. MATERIALS AND METHODS. Twenty-four patients with EVALI were identified. Chest CT examinations were reviewed by two radiologists for various chest CT findings. For comparison with pathologic findings, CT assessments were distilled into previously described patterns of EVALI seen on CT: acute lung injury (ALI), chronic eosinophilic pneumonia (CEP) or organizing pneumonia (OP), acute eosinophilic pneumonia (AEP), alveolar hemorrhage, hypersensitivity pneumonitis (HP), lipoid pneumonia, and mixed or unclassifiable patterns. RESULTS. Sixteen of 24 (67%) patients were men; the mean age was 34.5 years (range, 17-67 years). The most common CT finding was ground-glass opacities, which was present in 23 of 24 (96%) patients and the dominant finding in 18 of 24 (75%) patients. Consolidation was the next most common finding in 42% of patients. Interlobular septal thickening was present in 29%. Lobular low attenuation was conspicuous in six patients. Distribution was multifocal in 54% of patients, peripheral in 17%, and centrally predominant in 8%. Subpleural sparing was seen in 45%. The predominant CT pattern was ALI (42%), concordant with histopathologic findings in 75%; the next most predominant pattern was ground-glass opacity centrilobular nodules resembling HP (33%). A CT pattern of CEP or OP was seen in 13% of patients, all showing ALI on lung biopsy. No patient showed macroscopic lung parenchymal fat. Two patients with CT ALI patterns showed OP on histopathologic examination. Of the eight patients with ground-glass opacity centrilobular nodules resembling HP at CT, none showed HP at histopathologic examination. CONCLUSION. EVALI manifests at CT as ALI with multifocal ground-glass opacity, often with organizing consolidation, and a small centrilobular nodular pattern resembling HP.

Pulmonary Injury Resulting from Vaping or e-Cigarette Use: Imaging Appearances at Presentation and Follow-up.

Electronic cigarette or vaping product use-associated lung injury most frequently presents with an acute lung injury pattern at CT, manifesting as multifocal ground-glass opacity and/or consolidation, typically multifocal and multilobar, possibly with subpleural sparing. Areas of organization, manifesting as contracting consolidation, mild architectural distortion, intralobular lines, lobular distortion, and traction bronchiectasis may occur as the illness evolves. A CT appearance resembling hypersensitivity pneumonitis, reflecting the exquisitely bronchiolocentric micronodular lesions of organizing pneumonia and acute lung injury seen at histopathologic findings in these patients, may be encountered. Less common CT appearances include organizing pneumonia or acute eosinophilic pneumonia patterns, the latter consisting of multifocal opacity and smooth interlobular septal thickening, possibly with small effusions, but without clinical evidence of volume overload. Patients may present with pneumothorax or pneumomediastinum, or these conditions may develop during their illness course. Most patients improve clinically and at imaging on follow-up, particularly following exposure cessation and corticosteroid therapy, but the time course to improvement is variable and most likely related to the severity of the lung injury. Radiologists should be familiar with the imaging manifestations of vaping-associated pulmonary injury, and the possibility of this condition should be considered when the imaging findings reviewed in this article are encountered. © RSNA, 2020.

Pneumocytic adenomyoepithelioma: A case providing support for the benignity of this extremely rare pulmonary neoplasm.

Pneumocytic adenomyoepithelioma is an extremely rare and poorly understood pulmonary neoplasm, so experience with this tumor is limited. Since the initial case series where the lesion was first proposed as a distinctive entity, only one additional report has been described. We present a case of pneumocytic adenomyoepithelioma with clinical and radiologic data that provide the first long-term evidence of the benignity of this extremely rare pulmonary neoplasm. We also review the available literature surrounding pneumocytic adenomyoepitheliomas. Our case provides important new data on the behavior of this lesion, as imaging studies showed essentially stable or very slowly progressive disease over the course of approximately 9 years. Collectively, this rare and poorly described lesion appears to behave in an indolent or benign fashion, a notion that our case further supports.

Left Ventricular Septal Hypertrophy in Elderly Patients With Aortic Stenosis.

OBJECTIVES: Left ventricular (LV) septal hypertrophy in aortic stenosis raises diagnostic and therapeutic questions. However, the etiology and clinical consequences of this finding have not been well studied. The aim of this study was to perform a morphologic evaluation of the LV in aortic stenosis and to investigate the contributing factors and consequences of septal hypertrophy. METHODS: Patients with moderate or severe aortic stenosis were prospectively enrolled. Patients with previous myocardial infarction, wall motion abnormalities, at least moderate valvular regurgitation, known cardiomyopathy, an LV ejection fraction of less than 50%, and age younger than 65 years were excluded. RESULTS: Forty-one patients underwent a final analysis. Septal hypertrophy (LV septal wall thickness ≥15 mm) was confirmed in 21 of 41 patients. The septal hypertrophy group had higher peak aortic valve velocity, a higher diabetes mellitus rate, and a higher rate and longer duration of hypertension than those without septal hypertrophy. The peak aortic valve velocity (odds ratio, 7.1; 95% confidence interval, 1.4-37.1) and diabetes mellitus (odds ratio, 7.4; 95% confidence interval, 1.2-46.2) were the significant factors associated with septal hypertrophy by multivariate analysis. Intraventricular conduction disturbance on electrocardiography was more frequent in the septal hypertrophy group (P = .021). CONCLUSIONS: Left ventricular septal hypertrophy was commonly observed in elderly patients with aortic stenosis, and a higher aortic valve velocity, hypertension, and diabetes mellitus were associated factors. Intraventricular conduction disturbance occurred more often in patients with septal hypertrophy than those without, which implies the pathophysiologic consequence. Further studies are needed to determine the impact of septal hypertrophy and intraventricular conduction disturbance on the prognosis of patients after aortic valve interventions.

Constrictive Pericarditis 5 Months after Radiation Therapy in a 62-Year-Old Woman with Esophageal Cancer.

Most pericardial changes appear within a few weeks in patients who have undergone radiation therapy for thoracic neoplasms. Chronic pericardial constriction typically occurs decades later, consequent to fibrosis. Early constrictive pericarditis after chest irradiation is quite rare. We report the case of a 62-year-old woman who underwent radiation therapy for esophageal cancer and presented with constrictive pericarditis 5 months later. We searched the English-language medical literature from January 1986 through December 2015 for reports of early constrictive pericarditis after irradiation for thoracic malignancies. We defined "early" as a diagnosis within one year after radiation therapy. Five cases fit our criteria, and we summarize the findings here. To our knowledge, ours is the first definitive report of a patient with esophageal cancer to present with early radiation-induced constrictive pericarditis. We conclude that constrictive pericarditis can occur early after radiation for thoracic malignancies, albeit rarely. When planning care for cancer patients, awareness of this sequela is helpful.

Dendriform Pulmonary Ossification in the Absence of Usual Interstitial Pneumonia: CT Features and Possible Association With Recurrent Acid Aspiration.

OBJECTIVE: Dendriform pulmonary ossification (DPO) is a rare lung disease in which mature bone is present in the peripheral interstitium of the lung. It typically occurs in patients with usual interstitial pneumonia (UIP). We assessed patients with CT findings of DPO without UIP to determine possible causative factors and to assess the clinical and CT course. We hypothesized that DPO without UIP would be a unique entity. MATERIALS AND METHODS: We retrospectively reviewed CT reports for the word "ossification." Two observers reviewed each examination for micronodules 1-5 mm in diameter in the peripheral interstitium (subpleural and perifissural spaces and interlobular septa), some of which had high attenuation on mediastinal windows, presence of contiguous clusters of nodules resulting in a branching pattern, and lack of findings of UIP or focal lung disease. We reviewed the electronic medical records and follow-up CT and clinical information in all eligible patients. RESULTS: The study population consisted of 52 men with a median age of 79 years old. Seventy-five percent of the patients had gastroesophageal reflux disease, obstructive sleep apnea, or a chronic neurologic disorder. No progressive pulmonary symptoms were attributed directly to DPO, and no patient developed pulmonary fibrosis or suffered clinical decline from DPO. CT showed minimal progression or remained stable at follow-up (77% for at least 1 year, 25% for over 4 years). CONCLUSION: DPO in the absence of UIP occurs in elderly men and appears to be associated with chronic aspiration of gastric acid. The course is indolent.

Angiosarcoma Causing Cardiac Constriction Late after Radiation Therapy for Breast Carcinoma.

Therapeutic radiotherapy rarely causes sarcoma, and this occurs years after completion of the intended treatment. In treating breast carcinoma, careful planning in the application of modern radiotherapeutic techniques usually can shield the heart and pericardium. We report a rare case of angiosarcoma of the pericardium, which presented in a 41-year-old woman as constrictive pericarditis 8 years after irradiation for cancer of the left breast. To our knowledge, this is only the 2nd report of angiosarcoma of the pericardium after radiotherapy.

Diagnosis of Usual Interstitial Pneumonitis in the Absence of Honeycombing: Evaluation of Specific CT Criteria With Clinical Follow-Up in 38 Patients.

OBJECTIVE: We sought to evaluate specific CT criteria for the diagnosis of usual interstitial pneumonitis (UIP) in the absence of honeycombing. These criteria included peripheral reticulation and lobular distortion; some upper lobe involvement, but a lower zone predominance; a heterogeneous appearance with areas of normal lung, minimal reticulation, and substantial distortion alternating throughout the study and often on an individual image; a nonsegmental distribution; and traction bronchiectasis. MATERIALS AND METHODS: We searched reports of CT studies performed between January 1, 2009, and January 1, 2012, to identify patients for whom UIP was a likely or probable diagnosis and reviewed the CT study for each case (n = 106). There were 38 patients who met all CT criteria and who also had a clinical diagnosis of idiopathic UIP (also known as idiopathic pulmonary fibrosis [IPF]) and follow-up of at least 6 months, as determined from the electronic medical record. We reviewed prior and subsequent CT examinations in this cohort. RESULTS: The median age of our patients was 80 years, and the duration of clinical follow-up was 6-104 months (mean, 38 months; median, 37 months). For all patients, a pulmonary medicine physician made a working diagnosis of IPF. Fifteen patients died from pulmonary complications, and 16 of the surviving patients had clinical or functional progression of disease. There were no instances in which the initial diagnosis was revised or reversed. CONCLUSION: Strict application of specific CT criteria may allow a specific diagnosis of UIP in the proper clinical setting in the absence of honeycombing.

The Incremental Value of Magnetic Resonance Imaging for Identification of Apical Pouch in Patients with Apical Variant of Hypertrophic Cardiomyopathy.

BACKGROUND: The development of a left ventricular (LV) apical pouch in patients with apical hypertrophic cardiomyopathy (aHCM) has been thought to be the transition point that can become an apical aneurysm, which is linked to higher risk of adverse events. In our study, we sought to compare the ability of transthoracic echocardiography (echo) and cardiac magnetic resonance imaging (cMRI) to accurately identify the presence of an apical pouch or aneurysm in patients with aHCM. METHODS: We retrospectively reviewed the charts of all consecutive patients that had features of aHCM on imaging. Data from cMRI and echo examinations were abstracted, and the ability of these diagnostic modalities to identify the presence of a LV apical pouch and aneurysm was analyzed. RESULTS: Of 31 patients with aHCM, 17 (54.8%) had an apical pouch and 2 were found to have apical aneurysm (6.5%) on cMRI. Echo with and without perflutren contrast was able to accurately identify both aneurysms, but only 47.1% (8/17) of apical pouches seen by cMRI. Two patients had apical thrombus that was identified by cMRI, but not by echo. CONCLUSION: Our findings indicate that cMRI is superior to echo in identifying apical pouches in patients with aHCM. Our results also suggest that in patients undergoing echo, the use of perflutren contrast for LV opacification increases the diagnostic yield. Further study is necessary to delineate whether earlier identification of an apical pouch will be of clinical benefit for patients with aHCM by altering clinical management and avoiding adverse cardiovascular events.

Risk Stratification for Cardiovascular Disease in Women in the Primary Care Setting.

BACKGROUND: Traditional risk assessment tools classify the majority of middle-aged women at low risk despite cardiovascular (CV) disease's affecting >50% of women and remaining the leading cause of death. Ultrasound-determined carotid intima-media thickness (CIMT) and/or computed tomographic coronary artery calcium score (CACS) quantify subclinical atherosclerosis and add incremental prognostic value. The aim of this study was to assess the utility of CIMT and CACS to detect subclinical atherosclerosis in younger women. METHODS: Asymptomatic women aged 50 to 65 years with at least one CV risk factor and low Framingham risk scores were identified prospectively at primary care and cardiology clinics. Mean intimal thickness, plaque on CIMT, and Agatston calcium score for CACS were obtained. RESULTS: Of 86 women (mean age, 58 ± 4.6 years; mean Framingham risk score, 1.9 ± 1.2; mean low-density lipoprotein cholesterol level, 138.9 ± 37.0 mg/dL), 53 (62%) had high-risk CIMT (51% plaque, 11% CIMT > 75th percentile). In contrast, three women (3.5%) had CACS > 100, all of whom had plaque by CIMT. Of the 58 women with CACS of 0, 32 (55%) had high-risk CIMT (48% plaque, 7% CIMT > 75th percentile). CONCLUSIONS: In patients referred by their physicians for assessment of CV risk, CIMT in asymptomatic middle-aged women with at least one CV risk factor and low risk by the Framingham risk score identified a large number with advanced subclinical atherosclerosis despite low CACS. Our results suggest that CIMT may be a more sensitive method for CV risk assessment than CACS or traditional risk tools in this population. Further studies are needed to determine if earlier detection would be of clinical benefit.

Hypoplasia, pseudocoarctation and coarctation of the aorta - a systematic review.

Aortic arch abnormalities are uncommon and may be seen in association with other congenital cardiac anomalies. Coarctation, pseudocoarctation and hypoplastic aortic arch are known aortic arch abnormalities, with the former being well studied, whilst for the latter two, much less is known. There are similarities and differences that are important to distinguish among these three conditions in order to avoid errors in diagnosis that may result in unnecessary investigations, which may in turn result in physical or emotional harm to the patient. For this reason, we present a systematic review of the published literature providing an evidence-based overview that may be helpful to clinicians when faced with this diagnostic dilemma.

Non-calcified coronary atherosclerotic plaque characterization by dual energy computed tomography.

Coronary heart disease (CHD) is the most prevalent cause of death worldwide. Atherosclerosis which is the condition of plaque buildup on the inside of the coronary artery wall is the main cause of CHD. Rupture of unstable atherosclerotic coronary plaque is known to be the cause of acute coronary syndrome. Vulnerability of atherosclerotic plaque has been related to a large lipid core covered by a fibrous cap. Non-invasive assessment of plaque characterization is necessary due to prognostic importance of early stage identification. The purpose of this study is to use the additional attenuation data provided by dual energy computed tomography (DECT) for plaque characterization. We propose to train supervised learners on pixel values recorded from DECT monochromatic X-ray and material basis pairs images, for more precise classification of fibrous and lipid plaques. The interaction of the pixel values from different image types is taken into consideration, as single pixel value might not be informative enough to separate fibrous from lipid. Organic phantom plaques scanned in a fabricated beating heart phantom were used as ground truth to train the learners. Our results show that support vector machines, artificial neural networks and random forests provide accurate results both on phantom and patient data.

Coronary calcium quantification using contrast-enhanced dual-energy computed tomography scans.

The purpose of this study is to evaluate a direct measure of calcium burden by using dual-energy computed tomography (DECT) during contrast-enhanced coronary imaging, potentially eliminating the need for an extra noncontrast X-ray acquisition. The ambiguity of separation of calcium from contrast material on contrast-enhanced images was solved by using virtual noncontrast images obtained by DECT. A new threshold CT number was required to detect the calcium carrying potential risk for adverse coronary events on virtual noncontrast images. Two methods were investigated to determine the 130 HU threshold for DECT scoring. An in vitro anthropomorphic phantom with 29 excised patient calcium plaques inserted was used for both a linear and a logistic regression analysis. An IRB approved in vivo prospective study of six patients was also performed to be used for logistic regression analysis. The threshold found by logistic regression model to define the calcium burden on virtual noncontrast images detects the calcium carrying potential risk for adverse coronary events correctly (2.45% error rate). DECT calcium mass and volume scores obtained by using the threshold correlates with both conventional Agatston and volume scores (r = 0.98, p < 0.001). A conventional CT cardiac exam requires two scans, including a noncontrast scan for calcium quantification and a contrast-enhanced scan for coronary angiography. With the ability to quantify calcium on DECT contrast-enhanced images, a DECT cardiac exam could be accomplished with one contrast-enhanced scan for both calcium quantification and coronary angiography. 

Usual interstitial pneumonia-pattern fibrosis in surgical lung biopsies. Clinical, radiological and histopathological clues to aetiology.

Pulmonary fibrosis in surgical lung biopsies is said to have a 'usual interstitial pneumonia-pattern' (UIP-pattern) of disease when scarring of the parenchyma is present in a patchy, 'temporally heterogeneous' distribution. These biopsies are one of the more common non-neoplastic specimens surgical pathologists encounter and often pose a number of challenges. UIP is the expected histopathological pattern in patients with clinical idiopathic pulmonary fibrosis (IPF), but the UIP-pattern can be seen in other conditions on occasion. Most important among these are the rheumatic interstitial lung diseases (RILD) and chronic hypersensitivity pneumonitis (CHrHP). Because theses entities have different mechanisms of injury, approach to therapy, and expected clinical progression, it is imperative for the surgical pathologist to correctly classify them. Taken in isolation, the UIP-pattern seen in patients with IPF may appear to overlap with that of RILD and CHrHP, at least when using the broadest definition of this term (patchy fibrosis). However, important distinguishing features are nearly always present in our experience, and the addition of a multidisciplinary approach will often resolve the critical differences between these diseases. In this manuscript, we review the distinguishing clinical, radiologic and histopathological features of UIP of IPF, RILD and CHrHP, based, in part, on the existing literature, but also lessons learned from a busy lung biopsy consultation practice.