Finding the Optimal Timing for Repair of Standard Tetralogy of Fallot: Analysis of Cardiac Magnetic Resonance and Echocardiography Parameters Related to Intermediate Term Outcomes in a Pediatric Population.

INTRODUCTION: Right ventricular (RV) dilatation is the determining prognostic factor in the long-term follow up of patients with repaired Tetralogy of Fallot (TOF). The objective of this study is to analyze whether the results vary depending on the timing of the complete repair and on the surgical technique applied. MATERIAL-METHODS: This is a retrospective longitudinal study in which patients with standard TOF were divided into 3 groups depending on their age at surgical repair: group 1 = Early repair (n = 12,1-8 months), group 2 = Late repair (n = 26, > 8 months), and group 3 = Late repair with previous palliative surgery (n = 17, > 8 months). Clinical, echocardiographic and cardiac magnetic resonance (CMR) data from patients that had received complete reparative surgery in our institution from January 2000 to March 2014 were analyzed and compared. RESULTS: 55 patients with echocardiogram and CMR studies (13.39 ± 3.59 years) were reviewed. All patients had at least moderate pulmonary regurgitation (PR). We observed a positive correlation between PR and right ventricular end-diastolic volume (r2 = 0.418; p = 0,004). Group 3 had more severe right ventricular dilatation than patients in groups 1 and 2 (p = 0.001). No differences in right ventricular end-diastolic volume, PR, and pulmonary trunk dimensions were observed between groups 1 and 2. Patients in group 3 had a longer hospital stay. CONCLUSIONS: Although all patients from our cohort had significant PR, age at surgery was not related to RV or pulmonary trunk dilatation. Previous palliative surgery was associated with more severe right ventricular dilatation and longer hospital stays. No differences were observed between early and late repair groups. Our study suggests that postponing TOF repair to a late stage does not improve the degree of PR or long-term morbidity from RV dilatation. Palliative surgery should be avoided if possible.

Structural network topology correlates of microstructural brain dysmaturation in term infants with congenital heart disease.

Neonates with complex congenital heart disease (CHD) demonstrate microstructural brain dysmaturation, but the relationship with structural network topology is unknown. We performed diffusion tensor imaging (DTI) in term neonates with CHD preoperatively (N = 61) and postoperatively (N = 50) compared with healthy term controls (N = 91). We used network topology (graph) analyses incorporating different weighted and unweighted approaches and subject-specific white matter segmentation to investigate structural topology differences, as well as a voxel-based analysis (VBA) to confirm the presence of microstructural dysmaturation. We demonstrate cost-dependent network inefficiencies in neonatal CHD in the pre- and postoperative period compared with controls, related to microstructural differences. Controlling for cost, we show the presence of increased small-worldness (hierarchical fiber organization) in CHD infants preoperatively, that persists in the postoperative period compared with controls, suggesting the early presence of brain reorganization. Taken together, topological microstructural dysmaturation in CHD infants is accompanied by hierarchical fiber organization during a protracted critical period of early brain development. Our methodology also provides a pipeline for quantitation of network topology changes in neonates and infants with microstructural brain dysmaturation at risk for perinatal brain injury.

Relationship between increases in pancreatic enzymes and cerebral events in children after traumatic brain injury.

OBJECTIVE: To describe the risk factors of early and delayed increases in pancreatic enzymes (PE) in children after severe traumatic brain injury (TBI) and to determine if cerebral events (such as intracranial hemorrhage or intracranial hypertension) are associated with increases in PE. DESIGN AND SETTINGS: Retrospective analysis of prospectively collected Pediatric Neurotrauma Registry for children with severe TBI (GCS ≤ 8). We assessed the association of clinical characteristics with the development of increases in PE using regression analyses. PATIENTS: Fifty-one children with severe TBI were classified into three groups [normal PE; early PE (PE increases within first 24 h); delayed PE (PE increases after 24 h)]. MEASUREMENTS AND MAIN RESULTS: Increases in PE were observed in 29/51 children [57% total; n = 9 (18%) early; n = 20 (39%) delayed]. Multisystem trauma was more prevalent in patients with early increases in PE compared to those without increases in PE (70 vs. 30%, RR = 2.8, 95% CI 1.1-7) but not different between delayed PE and normal PE groups. In the bivariate analyses, increasing age (odds ratios, [95% CI]; 1.2, [1.05-1.4]), intracranial hypertension (14.6, [2.6-80.5]), intracranial hemorrhage (6.2, [1.15-33.7]), receipt of pentobarbital (9.3, [2.1-39.9]), mannitol (13.2, [2.7-62.2]), and vasoactive medications (6.9, [1.5-31.3]) were associated with the development of delayed increases in PE, whereas sex, initial Glasgow Coma Scale, severity of injury (PRISM, Injury Severity Score), therapeutic hypothermia, morphine and furosemide were not associated. Both intracranial hypertension and intracranial hemorrhage independently predicted the development of increases in PE (14.6, [2.6-80.5], and 9.1, [1.31-63.3], respectively). CONCLUSIONS: Increases in PE, often used as the only measures of pancreatitis in children with other severe injuries, are common in children after severe TBI and delayed presentation appears related to intracranial events. This suggests a possible interaction between the brain and the gastrointestinal system, implying that disturbances in cerebral hemodynamics may lead to pancreatic dysfunction.