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The contemporaneous presentation of transposition of the great arteries and hypertrophic cardiomyopathy is rare and complicates optimal surgical timing. We present a newborn with transposition and severe hypertrophic cardiomyopathy with a postnatal course complicated by persistent pulmonary hypertension who was supported with extracorporeal membrane oxygenation until successful arterial switch operation on the day of life 8.

Simulation is a key component of training in the pediatric cardiac intensive care unit (CICU), a complex environment that lends itself to virtual reality (VR)-based simulations. However, VR has not been previously described for this purpose. Two simulations were developed to test the use of VR in simulating pediatric CICU clinical scenarios, one simulating junctional ectopic tachycardia and low cardiac output syndrome, and the other simulating acute respiratory failure in a patient with suspected coronavirus disease 2019. Six attending pediatric cardiac critical care physicians were recruited to participate in the simulations as a pilot test of VR's feasibility for educational and practice improvement efforts in this highly specialized clinical environment. All participants successfully navigated the VR environment and met the critical endpoints of the two clinical scenarios. Qualitative feedback was overall positive with some specific critiques regarding limited realism in some mechanical aspects of the simulation. This is the first described use of VR in pediatric cardiac critical care simulation.

Assess the effect of a simulation "boot camp" on the ability of pediatric nurse practitioners to identify and treat a low cardiac output state in postoperative patients with congenital heart disease. Additionally, assess the pediatric nurse practitioners' confidence and satisfaction with simulation training.Prospective pre/post interventional pilot study.University simulation center.Thirty acute care pediatric nurse practitioners from 13 academic medical centers in North America.We conducted an expert opinion survey to guide curriculum development. The curriculum included didactic sessions, case studies, and high-fidelity simulation, based on high-complexity cases, congenital heart disease benchmark procedures, and a mix of lesion-specific postoperative complications. To cover multiple, high-complexity cases, we implemented Rapid Cycle Deliberate Practice method of teaching for selected simulation scenarios using an expert driven checklist.Knowledge was assessed with a pre-/posttest format (maximum score, 100%). A paired-sample t test showed a statistically significant increase in the posttest scores (mean [SD], pre test, 36.8% [14.3%] vs post test, 56.0% [15.8%]; p0.001). Time to recognize and treat an acute deterioration was evaluated through the use of selected high-fidelity simulation. Median time improved overall "time to task" across these scenarios. There was a significant increase in the proportion of clinically time-sensitive tasks completed within 5 minutes (pre, 60% [30/50] vs post, 86% [43/50]; p = 0.003] Confidence and satisfaction were evaluated with a validated tool ("Student Satisfaction and Self-Confidence in Learning"). Using a five-point Likert scale, the participants reported a high level of satisfaction (4.7 ± 0.30) and performance confidence (4.8 ± 0.31) with the simulation experience.Although simulation boot camps have been used effectively for training physicians and educating critical care providers, this was a novel approach to educating pediatric nurse practitioners from multiple academic centers. The course improved overall knowledge, and the pediatric nurse practitioners reported satisfaction and confidence in the simulation experience.

HPS is a major complicating feature of end-stage liver disease. Diagnosis is clinical, and LT is the only definitive treatment. While the general impression is that HPS improves quickly after transplantation, it may not always be the case. We describe the smallest reported child with HPS prior to LT and requiring prolonged venoarterial extracorporeal membrane oxygenation after LT; especially as it is a rare occurrence, physician managing such cases should be aware of the circumstances under which HPS may require specific treatment.

Thirteen structural analogs of the potent nonpolyglutamatable dihydrofolate reductase inhibitor N α -(4-amino-4-deoxypteroyl)- N δ -hemiphthaloyl- l -ornithine (PT523) with modifications in the side chain, the para -aminobenzoyl moiety, or the 9,10-bridge were evaluated for the ability to inhibit human recombinant dihydrofolate reductase (DHFR), to utilize the reduced folate carrier (RFC) for influx, and to inhibit the growth of CCRF-CEM human leukemia cells in culture. In spectrophotometric assays of the kinetics of the reduction of dihydrofolate by DHFR in the presence of NADPH, these compounds had K i values ranging from 0.2 to 1.3 pM, and thus were not greatly different in potency from the parent drug PT523. By comparison, the K i values of aminopterin (AMT), methotrexate (MTX), and 10-ethyl-10-deazaaminopterin (EDX) were 3.7, 4.8, and 11 pM. In assays of competitive inhibition of [ 3 H ]MTX influx into CCRF-CEM cells, the K i values ranged from 0.21 to 7.3 μM, as compared with 0.71, 5.4, and 1.1 μM for PT523, AMT, and EDX. The K t for MTX was also re-analyzed and found to be 4.7 μM, in better agreement with the literature than our previously reported value of 7.1 μM. The ic 50 values of these compounds as inhibitors of the growth of CCRF-CEM cells after 72 hr of drug exposure ranged from 0.53 to 55 nM, and were qualitatively consistent with the other results.

To describe great-vessel dimensions in patients with D-loop transposition of the great arteries (TGA) who have undergone atrial switch operation (ATSO). Patients who have undergone arterial switch operation for TGA have a high incidence of dilation of the neoaortic root. The incidence and degree of great artery dilation in patients who have undergone ATSO for TGA has not previously been described. A retrospective database review identified patients with TGA and intact ventricular septum who underwent ATSO at

Patients who have undergone the arterial switch operation for d-loop transposition of the great arteries (d-TGA) have a high incidence of dilation of the neo-aortic (native pulmonary) root. The incidence and degree of native pulmonary and aortic root dilation in patients who have undergone the