Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. RSNA, 2016.

Purpose To develop and evaluate motion-compensation and compressed-sensing techniques in 4D flow MRI for anatomical assessment in a comprehensive ferumoxytol-enhanced congenital heart disease (CHD) exam. Materials and Methods A Cartesian 4D flow sequence was developed to enable intrinsic navigation and two sampling schemes: VDPoisson and VDRad. Four compressed-sensing methods were developed: A) VDPoisson scan reconstructed using spatial wavelets, B) added temporal total variation to A, C) VDRad scan using the same reconstruction as in B, and D) added motion compensation to C. With IRB approval and HIPAA compliance, 23 consecutive patients (8 females, mean 6.3 years) referred for ferumoxytol-enhanced CHD 3T MRI were recruited. Images were acquired and reconstructed using methods A–D. Two cardiovascular radiologists independently scored the images on a 5-point scale. These readers performed a paired wall motion and functional assessment between method D and 2D bSSFP CINE for 16 cases. Results Method D had higher diagnostic image quality for most anatomical features (mean 3.8–4.8) compared to A (2.0–3.6), B (2.2–3.7), and C (2.9–3.9) with P < 0.05 with good inter-observer agreement (κ ≥ 0.49). Method D had similar or better assessment of myocardial borders and cardiac motion compared to 2D bSSFP (P < 0.05, κ ≥ 0.77). All methods had good internal agreement in comparing aortic with pulmonic flow (BA mean < 0.02%, r > 0.85) and compared to method A (BA mean < 0.13%, r > 0.84) with P < 0.01. Conclusion Flow, functional, and anatomical assessment in CHD with ferumoxytol-enhanced 4D flow is feasible and can be significantly improved using motion compensation and compressed sensing.

Objective: We sought to determine whether patients or their families could identify adverse events in the emergency department (ED), to characterize patient reports of errors and to compare patient reports to events recorded by health care providers. Methods: This was a prospective cohort study in a quaternary care inner city teaching hospital with approximately 40 000 annual visits. ED patients were recruited for participation in a standardized interview within 24 hours of ED discharge and a follow-up interview 3-7 days after discharge. Responses regarding events were tabulated and compared with physician and nurse notations in the medical record and hospital event reporting system. Results: Of 292 eligible patients, 201 (69%) were interviewed within 24 hours of ED discharge, and 143 (71% of interviewees) underwent a follow-up interview 3-7 days after discharge. Interviewees did not differ from the base ED population in terms of age, sex or language. Analysis of patient interviews identified 10 adverse events (5% incident rate; 95% confidence interval [CI] 2.41%-8.96%), 8 near misses (4% incident rate; 95% CI 1.73%-7.69%) and no medical errors. Of the 10 adverse events, 6 (60%) were characterized as preventable (2 raters; κ = 0.78, standard error [SE] 0.20; 95% CI 0.39-1.00; p = 0.01). Adverse events were primarily related to delayed or inadequate analgesia. Only 4 out of 8 (50%) near misses were intercepted by hospital personnel. The secondary interview elicited 2 out of 10 adverse events and 3 out of 8 near misses that had not been identified in the primary interview. No designation (0 out of 10) of an adverse event was recorded in the ED medical record or in the confidential hospital event reporting system. Conclusion: ED patients can identify adverse events affecting their care. Moreover, many of these events are not recorded in the medical record. Engaging patients and their family members in identification of errors may enhance patient safety. RÉSUMÉObjectif : Nous avons cherché à déterminer si les patients ou leurs familles pouvaient détecter des événements indésirables à l'urgence, à caractériser les rapports d'erreurs des patients et à comparer les rapports des patients aux événements consignés par les fournisseurs de soins de santé. Méthodes : Nous avons réalisé une étude de cohorte prospective dans un hôpital universitaire de soins quaternaires du centre-ville qui compte environ 40 000 visites annuellement. Nous avons recruté des patients à l'urgence pour participer à une entrevue normalisée dans les 24 heures suivant ORIGINAL RESEARCH • RECHERCHE ORIGINALE EM ADVANCES

BackgroundAlthough it is known that Anderson-Fabry Disease (AFD) can mimic the morphologic manifestations of hypertrophic cardiomyopathy (HCM) on echocardiography, there is a lack of cardiovascular magnetic resonance (CMR) literature on this. There is limited information in the published literature on the distribution of myocardial fibrosis in patients with AFD, with scar reported principally in the basal inferolateral midwall.MethodsAll patients with confirmed AFD undergoing CMR at our center were included. Left ventricular (LV) volumes, wall thicknesses and scar were analyzed offline. Patients were categorized into 4 groups: 1) no wall thickening; 2) concentric hypertrophy; 3) asymmetric septal hypertrophy (ASH); and 4) apical hypertrophy. Charts were reviewed for clinical information.ResultsThirty-nine patients were included (20 males [51 %], median age 45.2 years [range 22.3–64.4]). Almost half (17/39) had concentric wall thickening. Almost half (17/39) had pathologic LV scar; three quarters of these (13/17) had typical inferolateral midwall scar. A quarter (9/39) had both concentric wall thickening and typical inferolateral scar. A subgroup with ASH and apical hypertrophy (n = 5) had greater maximum wall thickness, total LV scar, apical scar and mid-ventricular scar than those with concentric hypertrophy (n = 17, p < 0.05). Patients with elevated LVMI had more overall arrhythmia (p = 0.007) more ventricular arrhythmia (p = 0.007) and sustained ventricular tachycardia (p = 0.008).ConclusionsConcentric thickening and inferolateral mid-myocardial scar are the most common manifestations of AFD, but the spectrum includes cases morphologically identical to apical and ASH subtypes of HCM and these have more apical and mid-ventricular LV scar. Significant LVH is associated with ventricular arrhythmia.Electronic supplementary materialThe online version of this article (doi:10.1186/s12968-016-0233-6) contains supplementary material, which is available to authorized users.

With improvements in surgical and medical management, patients with congenital heart disease (CHD) are often living well into adulthood. MRI provides critical data for diagnosis and monitoring of these patients, yielding information on cardiac anatomy, blood flow, and cardiac function. Though historically these exams have been complex and lengthy, four-dimensional (4D) flow is emerging as a single fast technique for comprehensive assessment of CHD. The 4D flow consists of a volumetric time-resolved acquisition that is gated to the cardiac cycle, providing a time-varying vector field of blood flow as well as registered anatomic images. In this article, we provide an overview of MRI evaluation of congenital heart disease by means of example of three relatively common representative conditions: tetralogy of Fallot, aortic coarctation, and anomalous pulmonary venous drainage. Then 4D flow data acquisition, data correction, and postprocessing techniques are reviewed. We conclude with several examples that highlight the comprehensive nature of the evaluation of congenital heart disease with 4D flow.

The pattern of myocardial injury in patients after COVID-19 vaccination at MRI was similar to other causes of myocarditis, but with less severity. Key Results:1. In a retrospective study of 92 patients with myocarditis, cardiac MRI demonstrated a similar pattern of injury in 21 patients with myocarditis following COVID-19 vaccination compared to other causes, including subepicardial LGE.2. Myocardial abnormalities were less severe in patients with vaccine-associated myocarditis (eg, less functional impairment, lower native T1, and less frequent involvement of the septum) compared to other forms of myocarditis.

Purpose To compare the precision and inter-observer agreement of ventricular volume, function and mass quantification by three-dimensional time-resolved (4D) flow MRI relative to cine steady state free precession (SSFP). Materials and Methods With research board approval, informed consent, and HIPAA compliance, 22 consecutive patients with congenital heart disease (CHD) (10 males, 6.4±4.8 years) referred for 3T ferumoxytol-enhanced cardiac MRI were prospectively recruited. Complete ventricular coverage with standard 2D short-axis cine SSFP and whole chest coverage with axial 4D flow were obtained. Two blinded radiologists independently segmented images for left ventricular (LV) and right ventricular (RV) myocardium at end systole (ES) and end diastole (ED). Statistical analysis included linear regression, ANOVA, Bland-Altman (BA) analysis, and intra-class correlation (ICC). Results Significant positive correlations were found between 4D flow and SSFP for ventricular volumes (r = 0.808–0.972, p<0.001), ejection fraction (EF) (r = 0.900–928, p<0.001), and mass (r = 0.884–0.934, p<0.001). BA relative limits of agreement for both ventricles were between −52% to 34% for volumes, −29% to 27% for EF, and −41% to 48% for mass, with wider limits of agreement for the RV compared to the LV. There was no significant difference between techniques with respect to mean square difference of ED-ES mass for either LV (F=2.05, p=0.159) or RV (F=0.625, p=0.434). Inter-observer agreement was moderate to good with both 4D flow (ICC 0.523–0.993) and SSFP (ICC 0.619–0.982), with overlapping confidence intervals. Conclusion Quantification of ventricular volume, function and mass can be accomplished with 4D flow MRI with precision and inter-observer agreement comparable to that of cine SSFP.

All patients surviving an acute aortic dissection require continued lifelong surveillance of their diseased aorta. Late complications, driven predominantly by chronic false lumen degeneration and aneurysm formation, often require surgical, endovascular, or hybrid interventions to treat or prevent aortic rupture. Imaging plays a central role in the medical decision-making of patients with chronic aortic dissection. Accurate aortic diameter measurements and rigorous, systematic documentation of diameter changes over time with different imaging equipment and modalities pose a range of practical challenges in these complex patients. Currently, no guidelines or recommendations for imaging surveillance in patients with chronic aortic dissection exist. In this document, we present state-of-the-art imaging and measurement techniques for patients with chronic aortic dissection and clarify the need for standardized measurements and reporting for lifelong surveillance. We also examine the emerging role of imaging and computer simulations to predict aortic false lumen degeneration, remodeling, and biomechanical failure from morphological and hemodynamic features. These insights may improve risk stratification, individualize contemporary treatment options, and potentially aid in the conception of novel treatment strategies in the future.