Acute graft-versus-host disease following lung transplantation in a patient with a novel TERT mutation.

Familial pulmonary fibrosis is associated with loss-of-function mutations in telomerase reverse transcriptase (TERT) and short telomeres. Interstitial lung diseases have become the leading indication for lung transplantation in the USA, and recent data indicate that pathogenic mutations in telomerase may cause unfavourable outcomes following lung transplantation. Although a rare occurrence, solid organ transplant recipients who develop acute graft-versus-host disease (GVHD) have very poor survival. This case report describes the detection of a novel mutation in TERT in a patient who had lung transplantation for familial pulmonary fibrosis and died from complications of acute GVHD.

Approach to Pulmonary Hypertension: From CT to Clinical Diagnosis.

Pulmonary hypertension (PH) is a condition characterized by increased pressure in the pulmonary circulation. It may be idiopathic or arise in the setting of other clinical conditions. Patients with PH tend to present with nonspecific cardiovascular or respiratory symptoms. The clinical classification of PH was recently revised at the World Health Organization symposium in Nice, France, in 2013. That consensus statement provided an updated classification based on the shared hemodynamic characteristics and management of the different categories of PH. Some features seen at computed tomography (CT) can suggest a subtype or probable cause of PH that may facilitate placing the patient in the correct category. These features include findings in the pulmonary arteries (peripheral calcification, peripheral dilatation, eccentric filling defects, intra-arterial soft tissue), lung parenchyma (centrilobular nodules, mosaic attenuation, interlobular septal thickening, bronchiectasis, subpleural peripheral opacities, ground-glass opacities, diffuse nodules), heart (congenital lesions, left heart disease, valvular disease), and mediastinum (hypertrophied bronchial arteries). An approach based on identification of these CT features in patients with PH will allow the radiologist to play an important role in diagnosis and help guide the clinician in management of PH. ©RSNA, 2018.

Cross-sectional Imaging Anatomy and Pathologic Conditions Affecting Thoracic Nerves.

While in many cases they are not directly visualized, awareness of the thoracic nerves and their courses at cross-sectional imaging is important for radiologists. An understanding of the normal function of each nerve is important, as many patients present with neurologic signs and symptoms that can be used to reinforce search patterns for disease and detection of supportive radiologic abnormalities. In the case of primary neoplasms, understanding the expected presence of a nerve in the location of a mass can enhance and improve the accuracy of differential diagnoses. Even in the absence of neurologic symptoms, secondary involvement of these structures by malignancy or close proximity to other intrathoracic disease can prompt significant alterations in oncologic or surgical management. The major components of the thoracic nervous system with which the thoracic radiologist must be familiar are the phrenic, vagus, recurrent laryngeal, intercostal, and long thoracic nerves in addition to the sympathetic chain and brachial plexus. The anatomic structure and course of each component are described including its major functions. Major clinical signs and symptoms attributable to nerve dysfunction or disease are reviewed as well as any associated radiologic signs. Emphasis is placed on primary and secondary malignant involvement of the nerves and iatrogenic and traumatic injuries. Online supplemental material is available for this article. ©RSNA, 2016.

Imaging of Pregnancy-related Vascular Complications.

Pregnancy results in substantial hemodynamic and prothrombotic changes that form the foundation for downstream vascular complications, both during pregnancy and in the postpartum period. In addition, several important risk factors, including older patient age, diabetes, and smoking, can increase the risk for vascular-related pregnancy complications. Because radiologists often play an important role in evaluation of the pregnant patient, understanding the pathophysiology of vascular-related complications in pregnancy and their imaging appearances is essential for diagnostic accuracy. The authors review relevant normal physiologic changes of pregnancy, pathophysiologic changes, and imaging features of vascular conditions that can manifest in conjunction with pregnancy. Particular attention is given to pertinent imaging information that radiologists should provide to referring clinicians to optimally affect the management of pregnant patients as well as women in the peripartum and postpartum stages. Among the complications discussed are preeclampsia; hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome; spontaneous coronary artery dissection; placental abruption; amniotic fluid embolism and acute pulmonary embolism; dural venous sinus thrombosis; ovarian vein thrombophlebitis; vasa previa; uterine arteriovenous malformations; heritable aortopathies; and hereditary hemorrhagic telangiectasia. To help radiologists avoid the use of unnecessary ionizing radiation in pregnancy, imaging examination selection and optimization are also reviewed. Radiologists should be familiar with the physiologic changes of pregnancy, radiation risks during gestation, and pregnancy-related vascular complications to improve imaging examination selection, diagnosis, and clinical management. ©RSNA, 2017.

Accuracy of computed tomography findings in acute pericarditis.

BACKGROUND: Acute pericarditis is a close clinical mimic of pulmonary embolism (PE) in the emergency department, and thus many of these patients are evaluated with chest computed tomography (CT). PURPOSE: To study whether CT findings can be diagnostic of acute pericarditis. MATERIAL AND METHODS: Using the electronic medical record, we retrospectively identified 46 cases of acute pericarditis and 46 control patients with pericardial effusions due to volume overload, all of whom underwent CT examination. Cases were reviewed by two blinded academic thoracic radiologists. RESULTS: The majority, 67%, of the pericarditis cases were evaluated with PE-protocol CTs. Pericardial thickening/enhancement was the most accurate single parameter for pericarditis, with sensitivity of 54-59% and specificity of 91-96%. CONCLUSION: CT findings, while not sensitive for pericarditis, are diagnostic, with few false-positives. Radiologists should be attentive to pericardial thickening or enhancement on CT studies done for chest pain, as they may be able to suggest pericarditis as an alternative diagnosis for the chest pain.

MDCT of trachea and main bronchi.

Tracheobronchial imaging has undergone a major revolution since the advent of MDCT. The improved spatial and temporal resolution not only allows reformatting images that enhance the comprehension of disease before bronchoscopy or surgery, it has introduced newer techniques such as dynamic expiratory imaging to evaluate for tracheomalacia, which can be a subtle, but a confounding entity for patients and clinician alike. Tracheobronchial diseases can be arbitrarily divided into those that cause focal and diffuse narrowing and widening. Such groupings can help develop a practical approach in evaluating diseases of the central airways.

Bronchiectasis.

Bronchiectasis, or the irreversible dilatation of bronchi, can present with a host of nonspecific clinical symptoms, including hemoptysis, cough, and hypoxia. The radiologist, then, can play an important role in its detection and characterization. Bronchiectasis must be differentiated from motion artifact and transient bronchial dilatation in acute lung disease. When diagnosed, a logical approach may allow for proper triage of the patient to prevent progression of disease. The radiologic approach usually begins with CT, which is fast and accurate. The diagnostic approach should be based on the mechanisms of development of bronchiectasis (bronchial wall damage, endobronchial obstruction, and traction) and the location. Once an endobronchial lesion or adjacent fibrosis is excluded, location of the abnormality can be used to help narrow the differential diagnosis. When the bronchiectasis is upper lobe predominant, CF should first be considered but occasionally MAC infection may present with this finding. When the bronchiectasis is mid-upper lobe, then ABPA or chronic hypersensitivity pneumonitis might lead the list of diagnoses. Lower lobe bronchiectasis is usually the sequela of recurrent infection and conditions that predispose to recurrent infections, including Mounier-Kuhn, hypogammaglobulinemia, PCD, and recurrent infections. By using this approach, the radiologist can remain an integral part of the pulmonary team.

Coronary-cameral fistula with double-chambered right ventricle: appearance on cardiac magnetic resonance imaging and 3D printed anatomic modeling.

The present case illustrates cardiac magnetic resonance imaging (MRI) and three-dimensional (3D) printed anatomic model findings of a coronary-cameral fistula (CCF) and double-chambered right ventricle (DCRV). A pregnant woman presented with palpitations and near syncope. A non-contrast cardiac MRI showed CCF connecting to a DCRV. Post-delivery, the patient had a contrast-enhanced MRI and 3D printed anatomic model to better evaluate her aberrant anatomy.

Bronchiectasis.

Bronchiectasis is defined as irreversible bronchial dilatation, leading to chronic cough, sputum formation, and recurrent infections. HRCT plays a major role in diagnosis of bronchiectasis. Most bronchiectasis is either idiopathic or a result of prior infections. Cystic fibrosis, allergic bronchopulmonary aspergillosis, and traction bronchiectasis caused by prior tuberculosis, sarcoidosis, and silicosis with progressive massive fibrosis have an upper lobe distribution. A lower lobe distribution is mostly seen in chronic aspiration, hypogammaglobulinemia, Mounier-Kuhn syndrome, primary ciliary dyskinesia, and traction bronchiectasis caused by usual interstitial pneumonitis and nonspecific interstitial pneumonitis. The right middle lobe and lingula are preferentially involved in atypical mycobacterial infections and sometimes in primary ciliary dyskinesia and Kartagener syndrome. A location-based approach may help lead to a specific diagnosis.

A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR imaging.

Although delayed contrast material-enhanced cardiac magnetic resonance (MR) imaging has traditionally been used to evaluate ischemic disease and myocardial viability, it is increasingly being used in the evaluation of nonischemic cardiomyopathies. Unlike myocardial infarction, which demonstrates subendocardial or transmural delayed contrast enhancement in a vascular distribution, nonischemic cardiomyopathies demonstrate enhancement that is not limited to a vascular territory. In combination with other cardiac MR imaging features, the location (subendocardial, transmural, subepicardial, or mesocardial) and pattern (patchy or diffuse) of abnormal delayed myocardial enhancement allow differentiation between ischemic (infarct-related) and nonischemic cardiomyopathies and, in cases of nonischemic cardiomyopathy, narrowing of the differential diagnosis. With use of a structured approach, delayed contrast-enhanced cardiac MR imaging can be helpful in the early detection and appropriate treatment of nonischemic cardiomyopathies.

ACR Appropriateness Criteria® Dyspnea-Suspected Cardiac Origin.

This article discusses imaging guidelines for five dyspnea variants: (1) dyspnea due to heart failure, ischemia not excluded; (2) dyspnea due to suspected nonischemic heart failure, ischemia excluded; (3) dyspnea due to suspected valvular heart disease, ischemia excluded; (4) dyspnea due to suspected cardiac arrhythmia, ischemia excluded; and (5) dyspnea due to suspected pericardial disease, ischemia excluded. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

ACR Appropriateness Criteria® Acute Chest Pain-Suspected Pulmonary Embolism.

Pulmonary embolism (PE) remains a common and important clinical condition that cannot be accurately diagnosed on the basis of signs, symptoms, and history alone. The diagnosis of PE has been facilitated by technical advancements and multidetector CT pulmonary angiography, which is the major diagnostic modality currently used. Ventilation and perfusion scans remain largely accurate and useful in certain settings. Lower-extremity ultrasound can substitute by demonstrating deep vein thrombosis; however, if negative, further studies to exclude PE are indicated. In all cases, correlation with the clinical status, particularly with risk factors, improves not only the accuracy of diagnostic imaging but also overall utilization. Other diagnostic tests have limited roles. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer-reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Non-coronary cardiac CT imaging.

Cardiac multidetector CT (MDCT) has moved from purely anatomic imaging, to assessment of cardiac function. Significant advances since the advent of multidetector CT now make it feasible to assess not only the coronary arteries, but also ejection fraction, ventricular volumes, myocardial mass and the presence of wall-motion abnormalities. Advances include improvements in EKG-gating, including improvements in temporal resolution, as well as the addition of delayed contrast-enhanced methods. Anatomic imaging has improved as well, with thinner collimation and better reconstruction methods. Three-dimensional software programs now permit excellent surface rendered displays and multiplanar reconstructions suitable as surgical and procedural "road maps."

Imaging of Pericardial Diseases.

Given the widespread use of cross-sectional imaging modalities, specifically multidetector computed tomography and magnetic resonance, to evaluate thoracic disease, the pericardium is frequently imaged. Knowledge of the normal appearance and anatomical boundaries is vital for radiologists to avoid confusion with more sinister pathology. A variety of disorders and diseases of the pericardium can bring a patient to clinical attention from inflammatory conditions, resulting in pericarditis and pericardial effusion, to malignancy. This article discusses the anatomy and conditions that affect the pericardium, emphasizing the role imaging plays in diagnosis and management.

A Diet Rich in Medium-Chain Fatty Acids Improves Systolic Function and Alters the Lipidomic Profile in Patients With Type 2 Diabetes: A Pilot Study.

CONTEXT: Excessive cardiac long-chain fatty acid (LCFA) metabolism/storage causes cardiomyopathy in animal models of type 2 diabetes. Medium-chain fatty acids (MCFAs) are absorbed and oxidized efficiently. Data in animal models of diabetes suggest MCFAs may benefit the heart. OBJECTIVE: Our objective was to test the effects of an MCFA-rich diet vs an LCFA-rich diet on plasma lipids, cardiac steatosis, and function in patients with type 2 diabetes. DESIGN: This was a double-blind, randomized, 2-week matched-feeding study. SETTING: The study included ambulatory patients in the general community. PATIENTS: Sixteen patients, ages 37-65 years, with type 2 diabetes, an ejection fraction greater than 45%, and no other systemic disease were included. INTERVENTION: Fourteen days of a diet rich in MCFAs or LCFAs, containing 38% as fat in total, was undertaken. MAIN OUTCOME MEASURES: Cardiac steatosis and function were the main outcome measures, with lipidomic changes considered a secondary outcome. RESULTS: The relatively load-independent measure of cardiac contractility, S', improved in the MCFA group (P < .05). Weight-adjusted stroke volume and cardiac output decreased in the LCFA group (both P < .05). The MCFA, but not the LCFA, diet decreased several plasma sphingolipids, ceramide, and acylcarnitines implicated in diabetic cardiomyopathy, and changes in several sphingolipids correlated with improved fasting insulins. CONCLUSIONS: Although a diet high in MCFAs does not change cardiac steatosis, our findings suggest that the MCFA-rich diet alters the plasma lipidome and may benefit or at least not harm cardiac function and fasting insulin levels in humans with type 2 diabetes. Larger, long-term studies are needed to further evaluate these effects in less-controlled settings.

Stabilization of disease after targeted therapy in a thymic carcinoma with KIT mutation detected by clinical next-generation sequencing.

Precision medicine uses individually determined genomic information to guide treatment in cancer and other diseases. We have implemented a clinical genomics assay that uses targeted next-generation sequencing of 25 cancer-related genes to guide the use of targeted therapies in diverse malignancies. We report the case of a 55-year-old woman with a poorly differentiated squamous cell carcinoma of thymic origin, with disease progression after standard treatment. Targeted tumor sequencing revealed the presence of a KIT codon 579 deletion (p.D579del). This specific mutation has not previously been associated with thymic tumors, but has been reported in gastrointestinal stromal tumors and has been associated with response to imatinib. Imatinib therapy was instituted for and resulted in stabilization of disease. This case illustrates the potential of clinical next-generation sequencing to open unexpected avenues for treatment and thereby improve patient outcomes.

Segmental analysis of congenital heart disease: putting the "puzzle" together with computed tomography.

Advances in surgical and medical treatment for congenital heart disease have resulted in greater life expectancy. As a result, there has been an increase in the utilization of cross-sectional imaging for diagnosis and management of complex congenital heart disease. This manuscript describes a morphological and sequential segmental approach to deciphering the code of complex congenital heart defects in cross-sectional imaging, mostly computed tomography. This manuscript will review approaches to differentiate types of transposition, the anatomic relationships of cardiac structures, and the application of these relationships in the description of complex congenital heart disease.

ACR appropriateness criteria® nonischemic myocardial disease with clinical manifestations (ischemic cardiomyopathy already excluded).

Nonischemic myocardial disease or cardiomyopathy can present as arrhythmia, palpitations, heart failure, dyspnea, lower extremity edema, ascites, syncope, and/or chest discomfort and can be classified as either systolic, diastolic, or a combination of both. Echocardiography is the mainstay of evaluating left ventricular function. However, cardiac magnetic resonance imaging (MRI) is now considered the reference standard imaging technique to assess myocardial anatomy, function, and viability. Advanced MRI techniques with delayed myocardial enhancement, especially, can provide information beyond echocardiography for tissue characterization in CM and can assist in determining specific etiology or in narrowing the differential. Often imaging enhancement patterns, signal characteristics, and morphology on MRI can lead to specific diagnoses such as amyloidosis, hypertrophic CM, or iron deposition. Cardiac computed tomography is usually used in excluding coronary artery disease but can also be used in some patients unable to undergo cardiac MRI to assess arrhythmogenic right ventricular dysplasia. Both 18-F-fluoro-2-deoxyglucose positron emission tomography and delayed contrast-enhanced MRI can be used to assess for cardiac sarcoidosis. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 3 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.

Cardiac imaging in adults with congenital heart disease: unknowns and issues related to diagnosis.

OPINION STATEMENT: Many adults with simple and complex congenital heart disease (CHD) survive to adulthood. The goal of imaging is to diagnose the underlying anomalies and to detect late complications of their CHD and past surgical repair, in order to assess the need for further intervention and better prepare for endovascular or open-heart surgery. Cardiac magnetic resonance imaging (MRI) and computerized tomography (CT) are increasingly utilized in this patient population, due to the technical advances made to these modalities in the past decade regarding image acquisition and reconstruction, spatial and temporal resolution, and radiation dose reduction. Here, we aim to review the role of cardiac MR in initial diagnosis, pre-treatment planning and post-surgical follow-up of adults with CHD, and to discuss the ancillary role of cardiac CT in these patients.

ACR Appropriateness Criteria chronic chest pain-low to intermediate probability of coronary artery disease.

Chronic chest pain can arise from a variety of etiologies. However, of those potential causes, the most life-threatening include cardiac disease. Chronic cardiac chest pain may be caused either by ischemia or atherosclerotic coronary artery disease or by other cardiac-related etiologies, such as pericardial disease. To consider in patients, especially those who are at low risk for coronary artery disease, are etiologies of chronic noncardiac chest pain. Noncardiac chest pain is most commonly related to gastroesophageal reflux disease or other esophageal diseases. Alternatively, it may be related to costochondritis, arthritic or degenerative diseases, old trauma, primary or metastatic tumors, or pleural disease. Rarely, noncardiac chest pain may be referred pain from organ systems below the diaphragm, such as the gallbladder. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every 2 years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment.