Faculty Bibliography

Primary immunodeficiency disorders (PIDs), which are humoral, combined, and innate defects of the immune system, are relatively uncommon and may go undiagnosed in patients experiencing recurrent infections, resulting in increased morbidity and mortality. PIDs are clinically characterized by a broad spectrum of disorders, including repeated infections, autoimmune disorders, lymphoproliferative diseases, congenital anomalies, and increased risk of malignancy. Cardiothoracic imaging plays a crucial role in the diagnosis of PIDs owing to the high rates of repeated respiratory infections leading to bronchiectasis and other forms of chronic lung disease. Although PIDs as a group may seem similar in terms of radiologic features and clinical manifestations, there are specific entities that are pertinent to each PID on an individual level. For example, patients with common variable immunodeficiency may develop a unique granulomatous lymphocytic interstitial lung disease, and Good syndrome is associated with thymoma. Familiarity with the imaging characteristics of these disorders may expedite diagnosis and prognostication, and better direct therapy. Reviewing the thoracic manifestations of all PIDs is beyond the scope of this article; thus, the focus herein is on discussing the thoracic manifestations of the most common PIDs and their imaging features. © RSNA, 2021An earlier incorrect version appeared online. This article was corrected on March 25, 2021.

Myocardial infarction with nonobstructive coronary arteries (MINOCA) occurs when a patient presents with positive cardiac enzymes in the absence of obstructive atherosclerosis on coronary angiography. Several hypotheses for the pathogenesis of MINOCA have been suggested and multiple potential underlying etiologies have been reported. This review will outline the reported causes of MINOCA and associated major imaging features. In doing so, it will increase awareness of this entity and equip cardiac imagers with the knowledge to appropriately tailor imaging to make a prompt and accurate diagnosis.

Rationale: Chronic hypersensitivity pneumonitis (CHP) is caused by an immune response to antigen inhalation and is characterized by variable histopathological and clinical features. A subset of subjects with CHP have usual interstitial pneumonia and appear to be clinically similar to subjects with idiopathic pulmonary fibrosis (IPF).Objectives: To determine the common and unique molecular features of CHP and IPF.Methods: Transcriptome analysis of lung samples from CHP (n = 82), IPF (n = 103), and unaffected controls (n = 103) was conducted. Differential gene expression was determined adjusting for sex, race, age, and smoking history and using false discovery rate to control for multiple comparisons.Measurements and Main Results: When compared with controls, we identified 413 upregulated and 317 downregulated genes in CHP and 861 upregulated and 322 downregulated genes in IPF. Concordantly upregulated or downregulated genes in CHP and IPF were related to collagen catabolic processes and epithelial development, whereas genes specific to CHP (differentially expressed in CHP when compared with control and not differentially expressed in IPF) were related to chemokine-mediated signaling and immune responsiveness. Using weighted gene coexpression network analysis, we found that among subjects with CHP, genes involved in adaptive immunity or epithelial cell development were associated with improved or reduced lung function, respectively, and that MUC5B expression was associated with epithelial cell development. MUC5B expression was also associated with lung fibrosis and honeycombing.Conclusions: Gene expression analysis of CHP and IPF identified signatures common to CHP and IPF, as well as genes uniquely expressed in CHP. Select modules of gene expression are characterized by distinct clinical and pathological features of CHP.

Cardiovascular imaging plays a central role in the diagnosis, management, and follow-up of congenital and acquired cardiovascular disease in patients with Turner syndrome. Cardiovascular defects in this population may affect a single component of the cardiovascular system or exist in combination with other anomalies, and, they may present early in life or remain occult into adulthood. Careful screening and surveillance imaging are necessary for the early detection and management of cardiovascular defects, especially in cases wherein early intervention may be necessary to prevent a serious cardiovascular outcome. It is critical that these patients are followed-up by specialists aware of their unique cardiovascular risk factors and that imaging examinations are interpreted by cardiovascular imagers familiar with the variety of anomalies that may be present and/or warrant imaging follow-up. Herein, we review common and uncommon cardiovascular anomalies associated with Turner syndrome and provide an image-based approach to analyzing key cardiothoracic findings that should be assessed in this patient population. Current imaging recommendations and guidelines for various anomalies will also be reviewed.

Congenital pulmonary artery anomalies represent a diverse group of abnormalities, ranging from asymptomatic incidental findings to causes of sudden cardiac death. While some may be recognized in childhood, others may be found incidentally in adulthood. We review the clinical and imaging findings in patients with congenital anomalies of the pulmonary arteries, including valvular and perivavular anomalies as well as abnormal narrowing, course and communications of the pulmonary arteries. We also discuss the role of various imaging modalities in the evaluation of these patients. It is vital to be aware of the key radiologic manifestations and associated haemodynamic consequences in these conditions in order to facilitate accurate diagnosis and prognostic stratification.

Aortic intramural hematoma (IMH) is a pathologic process with a clinical presentation identical to aortic dissection and associated with significant morbidity and mortality. Radiologists must be familiar with the imaging appearances of IMH as computed tomography (CT) plays a critical role in both diagnosis and patient management. The course of IMH is variable and the process may regress, remain stable, or progress in extent and therefore imaging findings associated with a negative prognosis must be recognized and included in the formal radiology report. Potentially life-threatening complications and findings associated with IMH include hemopericardium and cardiac tamponade, coexisting aortic dissection, ulcer-like projection, intramural blood pool, and extension of hematoma along the pulmonary or coronary arteries, which are identifiable with aortic protocol CT. The purpose of this pictorial review is to provide the reader with an image-based review of the diagnostic criteria, related complications, and associated critical prognostic features in patients presenting with aortic IMH.

Common variable immunodeficiency (CVID) is associated with significant chronic lung disease. The purpose of this paper was to describe the clinical, radiologic, and pathologic findings of CVID-associated lung diseases. These include airways' disease, interstitial lung disease, lymphoma, and mucosa-associated lymphoid tissue lymphoma. In addition, a genetic syndrome termed Kabuki syndrome results in CVID-like immune abnormalities. These patients may also present with CVID-associated lung disease. Awareness and precise identification of CVID-associated lung disease may allow for better assessment of prognosis and direction of therapy.

Chest radiographs are obtained as a standard part of clinical care. Rapid advancements in medical technology have resulted in a myriad of new medical devices, and familiarity with their imaging appearance is a critical yet increasingly difficult endeavor. Many modern thoracic medical devices are new renditions of old designs and are often smaller than older versions. In addition, multiple device designs serving the same purpose may have varying morphologies and positions within the chest. The radiologist must be able to recognize and correctly identify the proper positioning of state-of-the-art medical devices and identify any potential complications that could impact patient care and management. To familiarize radiologists with the arsenal of newer thoracic medical devices, this review describes the indications, radiologic appearance, complications, and magnetic resonance imaging safety of each device. ^©RSNA, 2018.

We developed a faculty professional development seminar series in order to facilitate the integration of our numerous new faculty into academics. The changing nature of the healthcare system, increasing clinical and administrative responsibility, and lack of access to senior mentors can hinder junior faculty productivity and possibly increase attrition. Given that no ready-made resources existed to address these issues we established a Professional Development Committee, developed a curriculum that covers relevant topics including promotion, mentorship, conflict management and feedback, and effective presentation of scientific data, and instituted changes iteratively based upon feedback. We used surveys from successive years of this seminar series to assess effectiveness, and our data demonstrate that our Professional Development Seminar Series was valued by its participants and that individual lectures improved from year to year. While it is too early to determine whether our efforts will lead to long-term changes in promotion success or faculty retention, our initial data are promising.