Julie Slicker

Nutrition algorithms for infants with hypoplastic left heart syndrome; birth through the first interstage period.

Failure to thrive is common in infants with hypoplastic left heart syndrome and its variants and those with poor growth may be at risk for worse surgical and neurodevelopmental outcomes. The etiology of growth failure in this population is multifactorial and complex, but may be impacted by nutritional intervention. There are no consensus guidelines outlining best practices for nutritional monitoring and intervention in this group of infants. The Feeding Work Group of the National Pediatric Cardiology Quality Improvement Collaborative performed a literature review and assessment of best nutrition practices from centers participating in the collaborative in order to provide nutritional recommendations and levels of evidence for those caring for infants with single ventricle physiology.

Variation in Growth of Infants with a Single Ventricle

OBJECTIVE The study goal was to evaluate interstage growth variation among sites participating in the National Pediatric Cardiology Quality Improvement Collaborative registry caring for infants with hypoplastic left heart syndrome and to identify nutritional practices common among sites achieving best growth outcomes. STUDY DESIGN This was a retrospective analysis of infants in the registry who had presented due to their superior cavopulmonary connection (SCPC) and whose surgical site had enrolled ≥ 4 eligible patients in the registry. The primary outcome variable was weight-for-age z-score (WAZ) change between Norwood discharge and presentation for SCPC (interstage period). Blinded, structured interviews were performed with each site regarding site-specific nutritional practices. Practices common among sites with positive interstage WAZ changes were identified. RESULTS Sixteen centers enrolled 132 infants from December 2008 through December 2010. Median age at SCPC was 5 months (2.6-12.6), and median interstage WAZ change was -0.29 (-3.2 to 2.3). Significant variation in WAZ changes among sites was demonstrated (P < .001). Sites that used standard feeding evaluation prior to Norwood discharge and that closely monitored for specific weight gain/loss red flags in the interstage period demonstrated significantly better patient growth than those that did not use these practices (P = .002). CONCLUSIONS Considerable variation exists in interstage growth among patients receiving care at these 16 surgical sites. Standardization of interstage nutritional management with focus on best nutritional practices may lead to improved growth in this high-risk population of infants.

Improving interstage survival after Norwood operation: outcomes from 10 years of home monitoring.

OBJECTIVE Infants who undergo Norwood stage 1 palliation (S1P) continue with high-risk circulation until stage 2 palliation (S2P). Routine care during the interstage period is associated with 10% to 20% mortality. This report illustrates the sustained reduction of interstage mortality over 10 years associated with use of home monitoring. METHODS Daily monitoring of oxygen saturation and weight was done for all patients discharged to home after S1P. Notification of the care team occurred for oxygen saturation<75% or >90%, weight gain<20 g over 3 days, weight loss>30 g, or intake<100 cc/kg/d. Breach of these criteria marked an interstage event. Interstage outcomes are reported. Patient characteristics and perioperative variables were compared between patients with and without interstage events. RESULTS Over 10 years, 157 patients were discharged after S1P with home monitoring. Interstage survival was 98%. Breach of home criteria occurred in 59% (93 out of 157), with violation of oxygen saturation<75% the most common event. Patient characteristics, operative data, and early postoperative morbidity did not differ between patients with and without events. CONCLUSIONS Home monitoring after S1P is associated with excellent interstage survival. Although a breach of monitoring criteria occurred in more than half of patients, our analysis failed to identify independent predictors of interstage events. Analysis of variables predicting mortality could not be assessed due to the low frequency of death in this cohort. Failure to identify specific variables for interstage events suggests that home monitoring, as part of an interstage surveillance program, should be applied to all S1P hospital survivors.

Pediatric Parenteral Nutrition: Putting the Microscope on Macronutrients and Micronutrients

Parenteral nutrition can be a life-saving therapy, but its benefits need to be balanced with a unique set of risks and complications. Methods of practice vary because there is a dearth of research in the area of pediatric parenteral nutrition. This article reviews the available literature on parenteral nutrition in children and provides suggestions on prevention and management of parenteral nutrition-associated liver disease. Some of the issues discussed in this article include glucose infusion rates, cycling of parenteral nutrition, copper and manganese toxicity, and the provision of glutamine, selenium, and carnitine.

Effect of feeding modality on interstage growth after stage I palliation: A report from the National Pediatric Cardiology Quality Improvement Collaborative

OBJECTIVES Achieving adequate growth after stage 1 palliation for children with single-ventricle heart defects often requires supplemental nutrition through enteral tubes. Significant practice variability exists between centers in the choice of feeding tube. The impact of feeding modality on the growth of patients with a single ventricle after stage 1 palliation was examined using the multiinstitutional National Pediatric Cardiology Quality Improvement Collaborative data registry. METHODS Characteristics of patients were compared by feeding modality, defined as oral only, nasogastric tube only, oral and nasogastric tube, gastrostomy tube only, and oral and gastrostomy tube. The impact of feeding modality on change in weight for age z-score during the interstage period, from stage 1 palliation discharge to stage 2 palliation, was evaluated by multivariable linear regression, adjusting for important patient characteristics and postoperative morbidities. RESULTS In this cohort of 465 patients, all groups demonstrated improved weight for age z-score during the interstage period with a mean increase of 0.3±0.8. In multivariable analysis, feeding modality was not associated with differences in the change in weight for age z-score during the interstage period (P=.72). Risk factors for poor growth were a diagnosis of hypoplastic left heart syndrome (P=.003), vocal cord injury (P=.007), and lower target caloric goal at discharge (P=.001). CONCLUSIONS In this large multicenter cohort, interstage growth improved for all groups and did not differ by feeding modality. With appropriate caloric goals and interstage monitoring, adequate growth may be achieved regardless of feeding modality and therefore local comfort and complication risk should dictate feeding modality.

Subjective Global Nutritional Assessment in Critically Ill Children

BACKGROUND Underweight children admitted to the pediatric intensive care unit (PICU) have a higher risk of mortality than normal-weight children. The authors hypothesized that subjective global nutrition assessment (SGNA) could identify malnutrition in the PICU and predict nutrition-associated morbidities. METHODS The authors prospectively evaluated the nutrition status of 150 children (aged 31 days to 5 years) admitted to the PICU with the use of SGNA and commonly used objective anthropometric and laboratory measurements. Each child was administered the SGNA by a dietitian while anthropometric measurements were performed by an independent assessor. To test interrater reproducibility, 76 children had SGNA performed by another dietitian. Occurrence of nutrition-associated complications was documented for 30 days after admission. RESULTS SGNA ratings of well nourished, moderately malnourished, or severely malnourished demonstrated moderate to strong correlation with several standard anthropometric measurements (P < .05). The laboratory markers did not demonstrate any correlation with SGNA. Interrater agreement showed moderate reliability (κ = 0.671). Length of stay, pediatric logistic organ dysfunction, and Pediatric Risk of Mortality III were not significantly different across the groups and did not correlate with SGNA.

Feeding Dysfunction in Children with Single Ventricle Following Staged Palliation

OBJECTIVE To determine the prevalence of feeding dysfunction in children with single ventricle defects and identify associated risk factors. STUDY DESIGN Patients aged 2-6 years with single ventricle physiology presenting for routine cardiology follow-up at the Childrens Hospital of Wisconsin were prospectively identified. Parents of the patients completed 2 validated instruments for assessment of feeding dysfunction. Chart review was performed to retrospectively obtain demographic and diagnostic data. RESULTS Instruments were completed for 56 patients; median age was 39 months. Overall, 28 (50%) patients had some form of feeding dysfunction. Compared with a normal reference population, patients with single ventricle had statistically significant differences in dysfunctional food manipulation (P < .001), mealtime aggression (P = .002), choking/gagging/vomiting (P < .001), resistance to eating (P < .001), and parental aversion to mealtime (P < .001). Weight and height for age z-scores were significantly lower in subjects with feeding dysfunction (-0.84 vs -0.33; P < .05 and -1.46 vs -0.56; P = .001, respectively). Multivariable analysis identified current gastrostomy tube use (P = .02) and a single parent household (P = .01) as risk factors for feeding dysfunction. CONCLUSION Feeding dysfunction is common in children with single ventricle defects, occurring in 50% of our cohort. Feeding dysfunction is associated with worse growth measures. Current gastrostomy tube use and a single parent household were identified as independent risk factors for feeding dysfunction.

Using statistical process control to identify early growth failure among infants with hypoplastic left heart syndrome.

Although interventions to improve outcomes for children with congenital heart disease may be designed and tested, the rarity of any one specific defect presents a barrier to using traditional statistical methods to measure the effects of these interventions. The purpose of this report is to describe the innovative statistical approach taken by the Joint Council on Congenital Heart Disease (JCCHD) National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) to measure outcomes for infants with hypoplastic left heart syndrome—a relatively rare disease. We report our experience with the application of statistical process control methods to generate measures capable of identifying statistically significant change in the incidence of early growth failure—a clinically important outcome in this relatively small patient population.

Utility of speckle tracking echocardiography in measuring systemic right ventricular systolic function for patients with d-transposition of the great arteries status post atrial switch procedure: a comparison with cardiac magnetic resonance imaging

Background Patients with d-transposition of the great arteries (D-TGA), status post atrial switch, are at risk for developing systemic right ventricular (RV) dysfunction. Echocardiographic (Echo) assessment of RV function is subjective because complex RV geometry does not allow accurate determination of ejection fraction (EF). RVEF measured by cardiac magnetic resonance imaging (MRI) is the gold standard for quantitative assessment of systemic RV function. New Echo measures of ventricular deformation allow for quantitative assessment of RV function. The primary aim was to explore the correlation of global peak longitudinal strain (GPLS) of the systemic RV with MRI RVEF for patients with D-TGA status post atrial switch. The secondary aim was to characterize MRI and clinical findings in this population.

Growth Trajectory in Children with Trisomy 21 with and without Atrioventricular Septal Defect

OBJECTIVE Trisomy 21 is associated with poor weight gain and atrioventricular septal defects. The impact of atrioventricular septal defects on weight gain in the setting of Trisomy 21 has not previously been described in the recent era. This study aimed to determine if such an association is present. DESIGN Patients with Trisomy 21 with and without atrioventricular septal defects were identified. Clinical, surgical, and postoperative data were collected were for these patients and then compared between patients. Specifically, weight for age z-scores were compared at various time points in a univariate and multivariate fashion. Effect of timing of surgery in those with an atrioventricular septal defect was also studied. RESULTS A total of 86 patients were identified, 42 with an atrioventricular septal defect. There was a difference in weight for age z-scores between patients with and without atrioventricular septal defects only at 2 months (P value .038) and 6 months (P value .003) of age. This persisted after multivariate regression which demonstrated atrioventricular septal defects as an independent risk factor. There was no difference noted in weight at 2 years of age in patients undergoing atrioventricular septal defect repair before and 150 days of life. CONCLUSION There was a statistically significant, but not clinically relevant, difference in weight between the patients with Trisomy 21 with and without atrioventricular septal defects in our cohort. Those with atrioventricular septal defects required more nutritional intervention, such as gastrostomy tube placement. Timing of repair did not alter outcomes at midterm follow-up.