Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure.

Neonates who present in high output heart failure secondary to vein of Galen aneurysmal malformation can be difficult to manage medically due to the complex physiology that results from the large shunt through the malformation. Though the cardiac function is often normal, right ventricular dilation, severe pulmonary hypertension, and systemic steal can result in inadequate organ perfusion and shock. This report recommends medical management for stabilization of neonates prior to definitive management with endovascular embolization. IMPACT: Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial arteriovenous malformation, which can present in the neonatal period with high output heart failure. Heart failure secondary to VGAM is often difficult to manage and is associated with high mortality and morbidity. Despite optimal medical management, many patients require urgent endovascular embolization for stabilization of their heart failure. This report offers discrete recommendations that can be used by clinicians as guidelines for the medical management of heart failure in newborns with VGAM.

Developmental changes of the fetal and neonatal thyroid gland and functional consequences on the cardiovascular system.

Thyroid hormones play an important role in the development and function of the cardiac myocyte. Dysregulation of the thyroid hormone milieu affects the fetal cardiac cells via complex molecular mechanisms, either by altering gene expression or directly by affecting post-translational processes. This review offers a comprehensive summary of the effects of thyroid hormones on the developing cardiovascular system and its adaptation. Furthermore, we will highlight the gaps in knowledge and provide suggestions for future research.

Rapid Cycle Deliberate Practice Versus Reflective Debriefing for Pediatric Septic Shock Training.

OBJECTIVES: Rapid cycle deliberate practice is a simulation training method that cycles between deliberate practice and directed feedback to create perfect practice; in contrast to reflective debriefing where learners are asked to reflect on their performance to create change. The aim of this study is to compare the impact of rapid cycle deliberate practice versus reflective debriefing training on resident application and retention of the pediatric sepsis algorithm. DESIGN: Prospective, randomized-control study. SETTING: A tertiary care university children's hospital simulation room, featuring a high-fidelity pediatric patient simulator. SUBJECTS: Forty-six upper-level pediatric residents. INTERVENTIONS: Simulation training using rapid cycle deliberate practice or reflective debriefing. MEASUREMENTS AND MAIN RESULTS: Knowledge was assessed with a quiz on core sepsis management topics. The application of knowledge was assessed with a sepsis management checklist during the simulated scenario. The residents were assessed before and after the intervention and again at a follow-up session, 3-4 months later, to evaluate retention. Both groups had similar pre-intervention scores. Post-intervention, the rapid cycle deliberate practice group had higher checklist scores (rapid cycle deliberate practice 18 points [interquartile range, 18-19] vs reflective debriefing 17 points [interquartile range, 15-18]; p < 0.001). Both groups had improved quiz scores. At follow-up, both groups continued to have higher scores compared with the pre-intervention evaluation, with the rapid cycle deliberate practice group having a greater change in checklist score from pre-intervention to follow-up (rapid cycle deliberate practice 5 points [interquartile range, 3.5-7] vs reflective debriefing 3 points [interquartile range, 1.5-4.5]; p = 0.019). Both groups reported improved confidence in diagnosing and managing septic shock. CONCLUSIONS: Both rapid cycle deliberate practice and reflective debriefing are effective in training pediatric residents to apply the sepsis algorithm and in improving their confidence in the management of septic shock. The rapid cycle deliberate practice method was superior immediately post-training; however, it is unclear if this advantage is maintained over time. Both methods should be considered for training residents.

Reintervention Is Associated With Improved Survival in Pediatric Patients With Pulmonary Vein Stenosis.

OBJECTIVES: The aim of this study was to evaluate survival following catheter intervention in pediatric patients with pulmonary vein stenosis (PVS). BACKGROUND: Despite aggressive surgical and catheter intervention on PVS in children, recurrence and progression of stenosis can lead to right heart failure and death. Clinicians continue to seek effective treatment options for PVS. METHODS: A single-center, retrospective study was performed including all patients <18 years of age who underwent catheter intervention (balloon angioplasty and bare-metal stent and drug-eluting stent insertion) on PVS. Endpoints included death, vein loss, and rate of reintervention. RESULTS: Thirty patients underwent intervention (balloon angioplasty, n = 9; bare-metal stent, n = 5; drug-eluting stent, n = 16) at a median age of 6.4 months (4.3 to 9.9 months). Median follow-up duration was 30.6 months (77 days to 10.5 years). Fourteen patients (47%) died at a median of 2.0 months (0.4 to 3.2 months) following intervention. There was no association between DES placement and survival (p = 0.067). Reintervention (catheter or surgical) was associated with improved survival (p = 0.001), with a 1-year survival rate of 84% compared with 25% for no reintervention. Vein loss occurred in 34 of 58 (59%) veins at a median of 3.3 months (1.0 to 5.0 months). One-year vein survival was higher with DES implantation (p = 0.031) and with reintervention (p < 0.001). CONCLUSIONS: DES implantation at first catheter intervention appears to be associated with improved vein survival but may not result in improved patient survival. However, reintervention appears to be associated with improved patient survival and vein patency, suggesting that despite mode of treatment, frequent surveillance is important in the care of these patients.