Delivering Serious News in Pediatric Cardiology-A Pilot Program.

Pediatric cardiology fellows receive limited training on delivering serious news. This is a teachable skill through simulation-based communication. While studies have shown the use of communication courses in pediatrics, there have been none in pediatric cardiology. Pediatric cardiologists recognize the importance of good communication and desire further development of these skills. Based on an internal needs assessment, three cases were developed; fetal hypoplastic left heart syndrome, teenager with new hypertrophic cardiomyopathy, and young-adult with Fontan failure. A 4-h simulation course using evidence-based methods to teach delivering serious news was designed, consisting of a didactic session, case demonstration and small group case-based encounters with simulated patients. Trainees completed standardized pre/post-course surveys to assess perception of skill and preparedness. Paired survey responses were compared. Six pediatric cardiology fellows participated. Only 33% had received formal training in delivering serious news and 17% in techniques of responding to patient's emotions. The proportion of participants who felt good about their ability to deliver serious news and deal with a family's emotions increased from 0 to 83%. The proportion of participants who felt prepared to provide serious news about a patient's illness increased from 17 to 67%. Given the small number of participants, results were not statistically significant. All participants felt that the course was valuable in improving communication skills. A formal communication course increased perception of skill and preparedness among trainees. We provide an evidence-based framework and clinical cases for delivering serious news in pediatric cardiology, which is generalizable to other training programs.

Right Atrial Enlargement on Electrocardiogram in Previously Healthy Young Patients.

OBJECTIVES: To assess whether right atrial enlargement (RAE) on electrocardiogram (ECG) correlates with true RAE on echocardiogram in previously healthy young patients and to understand which patients with RAE on ECG may warrant additional testing. STUDY DESIGN: A single-center, retrospective review of previously healthy young patients with (1) ECGs that were read as RAE by a pediatric cardiologist and (2) echocardiograms obtained within 90 days of the ECG. ECGs were reviewed to confirm RAE and determine which leads met criteria. The echocardiograms were then reviewed and RA measurements with z scores obtained. A z score >2 was considered positive for RAE on echocardiogram. RESULTS: In total, 162 patients with median age 10.8 years were included in the study. A total of 23 patients had true RAE on echocardiogram, giving a positive predictive value (PPV) of 14%. In patients <1 year of age, the PPV increased to 35%. In patients older than 1 year, the PPV was low at 7%. Patients with true RAE were more likely to meet criteria for RAE in the anterior precordial leads (V1-V3) (48% vs 5%, P < .001) and meet criteria for right ventricular hypertrophy (22% vs 6%, P = .023). CONCLUSION: Our findings show that RAE on ECG has a low PPV for RAE on echocardiogram in previously healthy young patients. The highest yield for RAE on echocardiogram was observed in patients who were <1 year of age, had RAE in the anterior precordial leads, or displayed right ventricular hypertrophy on ECG.

The Use of Automated Atrial CMR Measures and a Novel Atrioventricular Coupling Index for Predicting Risk in Repaired Tetralogy of Fallot.

Atrial size and function have been recognized as markers of diastolic function, and diastolic dysfunction has been identified as a predictor of adverse outcomes in repaired tetralogy of Fallot (rTOF). This was a retrospective single-center study with the objective of investigating the use of atrial measurements obtained via CMR for predicting outcomes in rTOF patients. Automated contours of the left and right atria (LA and RA) were performed. A novel parameter, termed the Right Atrioventricular Coupling Index (RACI), was defined as the ratio of RA end-diastolic volume to right ventricle (RV) end-diastolic volume. Patients were risk-stratified using a previously validated Importance Factor Score for the prediction of life-threatening arrhythmias in rTOF. Patients with a high-risk Importance Factor Score (>2) had a significantly larger minimum RA volume (p = 0.04) and RACI (p = 0.03) compared to those with scores ≤2. ROC analysis demonstrated RACI to be the best overall predictor of a high-risk Importance Factor Score (AUC 0.73, p = 0.03). Older age at the time of repair and a diagnosis of pulmonary atresia were associated with a larger RACI. Automated atrial CMR measurements are easily obtained from standard CMRs and have the potential to serve as noninvasive predictors of adverse outcomes in rTOF.

Longitudinal Assessment of Cardiac Function Following Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Multisystem inflammatory syndrome in children (MIS-C) after COVID-19 is commonly associated with cardiac involvement. Studies found myocardial dysfunction, as measured by decreased ejection fraction and abnormal strain, to be common early in illness. However, there is limited data on longitudinal cardiac outcomes. We aim to describe the evolution of cardiac findings in pediatric MIS-C from acute illness through at least 2-month follow-up. A retrospective single-center review of 36 patients admitted with MIS-C from April 2020 through September 2021 was performed. Echocardiographic data including cardiac function and global longitudinal strain (GLS) were analyzed at initial presentation, discharge, 2-4-week follow-up, and at least 2-month follow-up. Patients with mild and severe disease, normal and abnormal left ventricular ejection fraction (LVEF), and normal and abnormal GLS at presentation were compared. On presentation, 42% of patients with MIS-C had decreased LVEF < 55%. In patients in whom GLS was obtained (N = 18), 44% were abnormal (GLS < |- 18|%). Of patients with normal LVEF, 22% had abnormal GLS. There were no significant differences in troponin or brain natriuretic peptide between those with normal and abnormal LVEF. In most MIS-C patients with initial LVEF < 55% (90%), LVEF normalized upon discharge. At 2-month follow-up, all patients had normal LVEF with 21% having persistently abnormal GLS. Myocardial systolic dysfunction and abnormal deformation were common findings in MIS-C at presentation. While EF often normalized by 2 months, persistently abnormal GLS was more common, suggesting ongoing subclinical dysfunction. Our study offers an optimistic outlook for recovery in patients with MIS-C and carditis, however ongoing investigation for longitudinal effects is warranted.

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.

Prediction of Future Epilepsy in Neonates With Hypoxic-Ischemic Encephalopathy Who Received Selective Head Cooling.

Epilepsy outcomes after therapeutic hypothermia for neonates with hypoxic-ischemic encephalopathy are understudied. The authors used multivariable logistic regression to predict epilepsy in neonates after selective head cooling. Sensitivity analyses used magnetic resonance imaging (MRI) and electroencephalogram (EEG) interpretations by different clinicians. Fifty neonates had 2-year follow-up. Nine developed epilepsy. Predictors included pH ≤6.8 on day of birth (adjusted odds ratio [OR] 19 [95% confidence interval (CI) 1-371]), burst suppression on EEG on day 4 (8.2 [1.3-59]), and MRI deep gray matter injury (OR 33 [2.4-460]). These factors stratify neonates into low (0-1 factors; 3% [0%-14%] risk), medium (2 factors; 56% [21%-86%] risk), and high-risk groups (3 factors; 100% [29%-100%] risk) for epilepsy. The stratification was robust to varying clinical interpretations of the MRI and EEG. Neonates with hypoxic-ischemic encephalopathy who undergo selective head cooling appear at risk of epilepsy if they have 2 to 3 identified factors. If validated, this rule may help counsel families and identify children for close clinical follow-up.

Engineering extracellular matrix structure in 3D multiphase tissues.

In native tissues, microscale variations in the extracellular matrix (ECM) structure can drive different cellular behaviors. Although control over ECM structure could prove useful in tissue engineering and in studies of cellular behavior, isotropic 3D matrices poorly replicate variations in local microenvironments. In this paper, we demonstrate a method to engineer local variations in the density and size of collagen fibers throughout 3D tissues. The results showed that, in engineered multiphase tissues, the structures of collagen fibers in both the bulk ECM phases (as measured by mesh size and width of fibers) as well as at tissue interfaces (as measured by density of fibers and thickness of tissue interfaces) could be modulated by varying the collagen concentrations and gelling temperatures. As the method makes use of a previously published technique for tissue bonding, we also confirmed that significant adhesion strength at tissue interfaces was achieved under all conditions tested. Hence, this study demonstrates how collagen fiber structures can be engineered within all regions of a multiphase tissue scaffold by exploiting knowledge of collagen assembly, and presents an approach to engineer local collagen structure that complements methods such as flow alignment and electrospinning.