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Open in new tab Download slide OBJECTIVES Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery. METHODS This was a retrospective review of 69 patients with Alagille syndrome who underwent congenital heart surgery at our institution. The underlying diagnoses included PPAS (n  = 29), tetralogy of Fallot with PPAS (n  = 14), tetralogy with PA (n  = 3), PA with ventricular septal defect and major aortopulmonary collateral arteries (n  = 21) and one each with D-transposition and supravalvar aortic stenosis. RESULTS The median age at surgery was 16 months (range 0–228 months). Procedures performed included PPAS repair (n  = 43), tetralogy with PA repair (n  = 3), unifocalization procedures (n  = 21) and other (n  = 2). Forty-two (61%) patients had mild or no liver dysfunction, while 26 (38%) had moderate or severe liver dysfunction. The median cardiopulmonary bypass time was 345 min (341 with liver dysfunction, 345 without liver dysfunction). There were a total of 8 operative (12%) deaths and 3 late (4%) deaths. Six operative and 2 late deaths occurred in patients with liver dysfunction (combined 30.7%) versus 2 operative and 1 late death (combined 7.1%) for patients without liver dysfunction (P  < 0.05). CONCLUSIONS These results suggest that liver dysfunction has a profound impact on survival in children with Alagille syndrome undergoing congenital heart surgery. [ABSTRACT FROM AUTHOR]

Background: Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality.Methods: This was a retrospective review of 938 consecutive patients undergoing congenital heart surgery at a single institution over a 2-year timeframe. The number of postoperative surgical and unplanned diagnostic procedures were counted and the impact on hospital length of stay and mortality was assessed.Results: 581 of the 938 (62%) patients had zero postoperative diagnostic or surgical procedures. These patients had a median length of stay of 6 days with a single operative mortality (0.2%). 357 of the 938 (38%) patients had one or more postoperative diagnostic or surgical procedures. These patients had a total of 1586 postoperative procedures. There was a significant correlation between the number of postoperative procedures and both hospital length of stay and mortality (p < .001). Patients who required 10 or more postoperative procedures had a median hospital length of stay of 89 days and had a 50% mortality. There were no survivors in patients who had 15 or more postoperative procedures.Conclusions: The data demonstrate that the number of postoperative procedures was highly correlated with both hospital length of stay and mortality. [ABSTRACT FROM AUTHOR]

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a relatively rare and complex form of congenital heart disease. Unifocalization of MAPCAs has been advocated by some groups for the treatment of this condition. The purpose of this study was to assess the midterm fate of unifocalized MAPCAs in a cohort of patients with retroesophageal MAPCAs. This was a retrospective review of 37 patients who underwent a unifocalization procedure. All patients in this study had 1 or more retroesophageal MAPCAs, and detailed mapping of the MAPCAs was made based on a combination of the cardiac catheterization and surgical findings. The 37 patients had a total of 166 MAPCAs, or 4.5 MAPCAs per patient. One hundred twenty-nine (78%) MAPCAs were unifocalized, whereas 37 (22%) were ligated because they were dual supply. Median follow-up was 69 months. At follow-up cardiac catheterization, evaluation of the 129 unifocalized MAPCAs demonstrated that 123 (95%) had antegrade flow, whereas 6 were occluded. For the 123 MAPCAs with antegrade flow, 97 (80%) were widely patent, whereas 26 were stenotic. Thirteen of the 37 patients have subsequently undergone reintervention on MAPCAs that were determined to be stenotic following unifocalization. Seven of these patients had mild disease and had complete resolution with balloon (n = 5) or surgical revision (n = 2). Six patients with moderate or severe disease underwent surgical revision with confirmed resolution in 4 of 6. The data demonstrate that the majority of unifocalized MAPCAs remain widely patent following unifocalization. However, one-quarter of unifocalized MAPCAs develop stenoses or occlusion. These results suggest the fate for most unifocalized MAPCAs is favorable but highlight the need for close vigilance. Midterm fate of unifocalized MAPCAs. Ninety-five percent of unifocalized MAPCAs had antegrade flow at follow-up evaluation. Of these, 80% were widely patent, whereas 20% had some degree of stenosis. Reinterventions on MAPCAs with stenoses were 100% successful. [Display omitted] [ABSTRACT FROM AUTHOR]

Introduction: We sought to describe characteristics of children admitted with pericardial effusion (PCE) and Systemic Lupus Erythematosus (SLE) and determine the association between PCE and outcomes of interest. Methods: We performed a retrospective cohort study of all patients 18 years of age or younger with an International Classification of Disease, Ninth Revision code for SLE from 2004 to 2015 in the Pediatric Health Information System (PHIS). Children with congenital heart disease or who had undergone heart surgery were excluded. Multivariable analysis evaluated the effect of PCE on mortality, length of stay (LOS), and readmission within 30 days. Results: There were 5679 admissions, in which 705 (12.4%) had PCE. Median age at admission was 15 years (interquartile range: 13-17). There were no differences in age or sex between patients with or without PCE. The percentage of black children was higher in the PCE group (43% vs. 31%, p < 0.001). The odds of a black patient having PCE were 1.7 higher than non-black patients (p < 0.001). In-hospital mortality was 2.5 times higher in children with PCE (p = 0.027). Those with PCE had 1.5 greater odds of readmission within 30 days (p < 0.001). PCE was not associated with increased LOS (0.99, p = 0.753). Conclusion: PCE is common in admissions of children with SLE. There are disproportionately more black patients with SLE affected by PCE. PCE is associated with significantly higher mortality and rates of readmission. [ABSTRACT FROM AUTHOR]

Introduction: Congenital heart disease (CHD) is the most common birth defect and leading cause of neonatal and infant death. CHD with single ventricle anatomy, such as double-inlet left ventricle, also known as common ventricle (CV), are the most complex with the highest mortality. In contrast to other forms of CHD, prenatal risk factors associated with CV have not been previously studied. Methods: We analyzed 200 CV cases and 11,063 nonmalformed controls in the National Birth Defects Prevention Study, a case-control, multicenter population-based study of birth defects among 32,200 cases from 9 United States. Mothers completed surveys by trained interviewers 6 weeks to 24 months after the estimated date of delivery (October 1997 to December 2011). Random forest analysis identified potential predictor variables for CV among >200 variables. These potential predictor variables were included in multivariable models to estimate effects. Odds ratios (OR) and 95% confidence intervals were estimated using logistic regression for CV among all cases, cases with isolated CV, and non-isolated cases, defined as CV with at least 1 additional major non-cardiac malformation. Results: Random forest analysis identified pre-pregnancy diabetes, any history of maternal insulin use, maternal total lipid intake, and paternal race as potential predictors of CV. Table 1 reports risk factors identified via logistic regression. Higher maternal fat intake was associated with reduced risk of CV. Conclusions: Maternal pre-pregnancy diabetes and history of insulin use were associated with increased risk of having an infant with CV while maternal lipid intake had an inverse association. These novel data provide multiple metabolic pathways for investigation to better identify the developmental etiologies of CHD and suggest that public health interventions targeting balanced metabolism in women of childbearing age could reduce CHD risk. [ABSTRACT FROM AUTHOR]