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Graphical abstract, Highlights • Consider CTD in the cyanotic neonate or infant. • CTD causes obstruction of tricuspid inflow and right-to-left atrial-level shunting. • Focused echocardiography on the area of the tricuspid valve can make the diagnosis. • Operative resection should be pursued for those with persistent hypoxia.

Objective Extremely low gestational age neonates (ELGANs) are at risk for pulmonary hypertension (PH). We hypothesized that PH, defined by echocardiogram at 36 weeks gestational age (GA), would associate with respiratory morbidity, increased oxidant stress, and reduced nitric oxide production. Study design ELGANs in the Vanderbilt fraction of the Prematurity and Respiratory Outcomes Program (PROP) who had echocardiograms at 36 ± 1 weeks GA were studied. Echocardiogram features of PH were compared with clinical characteristics as well as markers of oxidant stress and components of the nitric oxide pathway. Biomarkers were obtained at enrollment (median day 3), 7, 14, and 28 days of life. Results Sixty of 172 infants had an echocardiogram at 36 weeks; 11 had evidence of PH. Infants did not differ by PH status in regards to demographics, respiratory morbidity, or oxidant stress. However, odds of more severe PH were significantly higher in infants with higher nitric oxide metabolites (NOx) at enrollment and with a lower citrulline level at day 7. Conclusions Respiratory morbidity may not always associate with PH at 36 weeks among ELGANs. However, components of nitric oxide metabolism are potential biologic markers of PH in need of further study.

Morbidity and Mortality conference (MMC) serves an important role in medical care and education. We restructured our Department of Pediatrics MMC to focus on multidisciplinary participation and improved communication among disciplines, quality improvement, and system changes for safer clinical care and enhanced learning from adverse outcomes.The structure and philosophy of the Department of Pediatrics MMC was changed. We present guiding principles for the restructuring process and evaluation results postrestructuring, which examined achievement of conference goals, including quality improvement.The MMC led to system changes within the Department of Pediatrics as well as other parts of the hospital. Satisfaction with these changes was high among conference participants, who felt that the conference achieved its goals of including multiple disciplines and creating system changes.The successful change in the focus of the pediatric MMC conference resulted in significant hospital-wide system changes, quality improvements, enhanced education, and departmental satisfaction.

PURPOSE OF REVIEW The following review will focus on the current advances in both echocardiography and cardiovascular MRI (CMRI) in the assessment of cardiac function. RECENT FINDINGS The assessment of cardiac function in pediatric patients and in congenital heart disease (CHD) patients has dramatically improved over the last several years. The advancement of transthoracic echocardiography with tissue Doppler imaging, speckle tracking, and three-dimensional echocardiography has enabled strain assessment and the ability to estimate ventricular volumes in these complex patients. In the last few decades, CMRI has become an imaging modality that is now part of the standard tools used for cardiac assessment. With superb two-dimensional and three-dimensional resolution, CMRI allows clear delineation of cardiac and extracardiac structures as well as accurate and reproducible assessment of ventricular volume and function. The most recent and robust contributions of CMRI are its ability to provide characterization of the myocardium and the development of new measurements of global and regional myocardial mechanics and function. SUMMARY Recent advances in echocardiography and CMRI allow a better understanding of myocardial mechanics and composition as well as accurate assessment of ventricular volume and global and regional function in the complex and unique anatomy often found in CHD patients.

OBJECTIVE The literature suggests pediatric residents are inadequately prepared to perform resuscitation maneuvers when a child suffers a cardiopulmonary arrest (CPA). Our objective was to characterize the resuscitation training and CPA resuscitation experience of residents, including hands on experience with discharging a defibrillator. DESIGN : Cross-sectional survey. SETTING Tertiary care, academic pediatric residency program. SUBJECTS Pediatric residents. MEASUREMENTS AND MAIN RESULTS Seventy-six of 80 (95%) pediatric residents responded. The median (interquartile range) number of CPAs attended increased significantly by level of training, with some attending as many as 20 CPAs during residency (postgraduate year [PGY]1: 2.0 [1.0-3.0] vs. PGY2: 5.0 [3.0-8.0] vs. PGY3: 10.0 [5.0-12.0], p < 0.001). Nine of 25 (36%) senior residents had led a resuscitation. The proportion of third-year residents who had attended at least 1 CPA in the following locations was: general ward 20 of 25 (80%), Emergency Department 18 of 25 (72%), Neonatal intensive care unit 24 of 25 (96%), pediatric intensive care unit 23 of 25 (92%), and secondary training hospital 19 of 25 (76%). Twelve of 76 (16%) residents had discharged a defibrillator on an actual patient; however, 25 of 76 (33%) had never discharged a defibrillator, either on a patient or during training exercises. Although most residents had received required training in American Heart Association Basic Life Support and Pediatric Advance Life Support (i.e., BLS and PALS), 6 of 76 (8%) residents had never taken basic life support and 4 of 48 (8%) of upper level residents had never taken pediatric advanced life support. Multivariate analysis revealed that level of training, pediatric advanced life support training, and attendance at a mock code in the past year were not independently associated with having discharged a defibrillator (i.e., patient, mannequin, etc.), whereas attendance at an institutional Code Team training course was. CONCLUSIONS Almost every pediatric resident was involved in attempting to resuscitate a child suffering a CPA, yet many were inadequately trained to respond. Formal mechanisms are needed to guarantee adequate resuscitation training for pediatric residents, especially regarding participation in basic life support and hands on defibrillator training.

Background The quality of life support delivered during cardiopulmonary resuscitation affects outcomes. However, little data exist regarding the quality of resuscitation delivered to children and factors associated with adherence to American Heart Association (AHA) resuscitation guidelines. Participants Pediatric residents from an academic, tertiary care hospital. Design Prospective, observational cohort study of residents trained in the AHA PALS 2000 guidelines managing a high-fidelity mannequin simulator programmed to develop pulseless ventricular tachycardia (PVT). Main outcome measures Proportion of residents who: (1) started compressions in ≤1min from onset of PVT, (2) defibrillated in ≤3min and (3) factors associated with time to defibrillation. Results Seventy of eighty (88%) residents participated. Forty-six of seventy (66%) failed to start compressions within 1min of pulselessness and 23/70 (33%) never started compressions. Only 38/70 (54%) residents defibrillated the mannequin in ≤3min of onset of PVT. There was no significant difference in time elapsed between onset of PVT and defibrillation by level of post-graduate training. However, residents who had previously discharged a defibrillator on either a patient or a simulator compared to those who had not were 87% more likely to successfully defibrillate the mannequin at any point in time (hazard ratio 1.87, 95% CI: 1.08–3.21, p =0.02). Conclusions Pediatric residents do not meet performance standards set by the AHA. Future curricula should focus training on identified defects including: (1) equal emphasis on "airway and breathing" and "circulation" and (2) hands-on training with using and discharging a defibrillator in order to improve safety and outcomes.