Multimodality Imaging in Ebstein Anomaly.

Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.

Occlusion of the os of the left coronary artery by dysplastic aortic valve tissue presenting as progressive mitral insufficiency and cardiac arrest.

Mitral regurgitation in the neonatal period is relatively rare. It can be secondary to a congenital malformation of the valve apparatus or mitral valve dysfunction and deformation secondary to myocardial dysfunction or volume load of the left ventricle. Less commonly, it can be due to coronary artery abnormalities leading to mitral valve papillary muscle ischaemia and subsequent dysfunction. Such coronary artery abnormalities include anomalous left coronary artery from pulmonary artery, left main coronary artery atresia, or a thromboembolic phenomenon. In this study, we describe a newborn with a dysplastic aortic valve causing obstruction of the os of the left coronary artery leading to progressive mitral insufficiency.

Impaired Neurovascular Function Underlies Poor Neurocognitive Outcomes and Is Associated with Nitric Oxide Bioavailability in Congenital Heart Disease.

We use a non-invasive MRI proxy of neurovascular function (pnvf) to assess the ability of the vasculature to supply baseline metabolic demand, to compare pediatric and young adult congenital heart disease (CHD) patients to normal referents and relate the proxy to neurocognitive outcomes and nitric oxide bioavailability. In a prospective single-center study, resting-state blood-oxygen-level-dependent (BOLD) and arterial spin labeling (ASL) MRI scans were successfully obtained from 24 CHD patients (age = 15.4 ± 4.06 years) and 63 normal referents (age = 14.1 ± 3.49) years. Pnvf was computed on a voxelwise basis as the negative of the ratio of functional connectivity strength (FCS) estimated from the resting-state BOLD acquisition to regional cerebral blood flow (rCBF) as estimated from the ASL acquisition. Pnvf was used to predict end-tidal CO2 (PETCO2) levels and compared to those estimated from the BOLD data. Nitric oxide availability was obtained via nasal measurements (nNO). Pnvf was compared on a voxelwise basis between CHD patients and normal referents and correlated with nitric oxide availability and neurocognitive outcomes as assessed via the NIH Toolbox. Pnvf was shown as highly predictive of PETCO2 using theoretical modeling. Pnvf was found to be significantly reduced in CHD patients in default mode network (DMN, comprising the ventromedial prefrontal cortex and posterior cingulate/precuneus), salience network (SN, comprising the insula and dorsal anterior cingulate), and central executive network (CEN, comprising posterior parietal and dorsolateral prefrontal cortex) regions with similar findings noted in single cardiac ventricle patients. Positive correlations of Pnvf in these brain regions, as well as the hippocampus, were found with neurocognitive outcomes. Similarly, positive correlations between Pnvf and nitric oxide availability were found in frontal DMN and CEN regions, with particularly strong correlations in subcortical regions (putamen). Reduced Pnvf in CHD patients was found to be mediated by nNO. Mediation analyses further supported that reduced Pnvf in these regions underlies worse neurocognitive outcome in CHD patients and is associated with nitric oxide bioavailability. Impaired neuro-vascular function, which may be non-invasively estimated via combined arterial-spin label and BOLD MR imaging, is a nitric oxide bioavailability dependent factor implicated in adverse neurocognitive outcomes in pediatric and young adult CHD.

Interpretation errors in focused cardiac ultrasound by novice pediatric emergency medicine fellow sonologists.

BACKGROUND: Focused cardiac ultrasound (FOCUS) is a core competency for pediatric emergency medicine (PEM) fellows. The objectives of this study were (1) to evaluate test characteristics of PEM-fellow-performed FOCUS for pericardial effusion and diminished cardiac function and (2) to assess image interpretation independent of image acquisition. METHODS: PEM fellows performed and interpreted FOCUS on patients who also received cardiology service echocardiograms, the reference standard. Subsequently, eight different PEM fellows remotely interpreted a subset of the PEM-acquired and cardiology-acquired echocardiograms. RESULTS: Eight PEM fellows performed 54 FOCUS exams, of which two had pericardial effusion and four had diminished function. PEM fellow FOCUS had a sensitivity of 50.0% (95% CI 9.19-90.8) and specificity of 100.0% (95% CI 91.1-100.0) for detecting diminished function, and sensitivity of 50.0% (95% CI 2.67-97.33) and specificity of 98.1% (95% CI 88.42-99.9) for detecting pericardial effusions. When PEM fellows remotely interpreted 15 echocardiograms, the sensitivity was 81.3% (95% CI 70.7-88.8) and specificity 75% (95% CI 67.0-81.0) for detecting diminished function, and sensitivity of 76.3% (95% CI 65.0-85.0) and specificity 94.4% (95% CI 89.0-97.0) for detecting pericardial effusion. There were no differences in sensitivity and specificity of PEM fellows' interpretation of FOCUS studies compared to their interpretation of cardiology echocardiograms. Interrater reliability for interpretation of remote images (kappa) was 0.66 (95% CI 0.59-0.73) for effusion and 0.31 (95% CI 0.24-0.38) for function among the fellows. CONCLUSION: Novice PEM fellow sonologists (a physician who performs and interprets ultrasound) in the majority of instances were able to acquire and remotely interpret FOCUS images with limited training. However, they made real-time interpretation errors and likely need further training to incorporate real-time image acquisition and interpretation into their practice.

Surgical ligation of patent ductus arteriosus in premature infants: trends and practice variation.

OBJECTIVE: We sought to analyse the variation in the incidence of patent ductus arteriosus over three recent time points and characterise ductal ligation practices in preterm infants in the United States, adjusting for demographic and morbidity factors. METHODS: Using the Kids' Inpatient Database from 2003, 2006, and 2009, we identified infants born at ⩽32 weeks of gestation with International Classification of Diseases, Ninth Revision diagnosis of patent ductus arteriosus and ligation code. We examined patient and hospital characteristics and identified patient and hospital variables associated with ligation. RESULTS: Of 182,610 preterm births, 30,714 discharges included a patent ductus arteriosus diagnosis. The rate of patent ductus arteriosus diagnosis increased from 14% in 2003 to 21% in 2009 (p<0.001). A total of 4181 ligations were performed, with an overall ligation rate of 14%. Ligation rate in infants born at ⩽28 weeks of gestation was 20% overall, increasing from 18% in 2003 to 21% in 2009 (p<0.001). The ligation rate varied by state (4-28%), and ligation was associated with earlier gestational age, associated diagnoses, hospital type, teaching hospital status, and region (p<0.001). CONCLUSION: The rates of patent ductus arteriosus diagnosis and ligation have increased in the recent years. Variation exists in the practice of patent ductus arteriosus ligation and is influenced by patient and non-patient factors.