Variation in Growth of Infants with a Single Ventricle
The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. The Journal of pediatrics
(Impact Factor: 3.79).
02/2012; 161(1):16-21.e1; quiz 21.e2-3. DOI: 10.1016/j.jpeds.2012.01.009
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.
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.
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).
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.
Available from: Linda M Lambert
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ABSTRACT: 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.
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ABSTRACT: Infants with single ventricle physiology have a high mortality and poor somatic growth during the interstage period. We retrospectively assessed the impact of pharmacotherapy in this population using a multicenter database.
Records for 395 patients (63.5% boys) with single ventricle were obtained from the National Pediatric Cardiology Quality Improvement Collaborative registry. Median of five medications were prescribed per patient at discharge after stage 1 palliation (interquartile range 3 to 6); the most common medications being aspirin (95.7%), diuretics (90.4%), angiotensin convertase enzyme inhibitors (37.7%), proton pump inhibitors (33.4%), H2 receptor blockers (30.6%), and digoxin (27.6%). Interstage mortality was 9.4%. Digoxin use was associated with lower risk of death (P =.03) on univariable analysis, however no single medication was an independent predictor on regression analysis. Change in weight-for-age z-score was studied as outcome of somatic growth with 36.3% patients showing a decrease during the interstage period. Total number of medications prescribed to a patient showed a negative correlation with the interstage change in z-score (r = −0.19, P =.002). On univariable comparisons, use of metoclopramide and lansoprazole were associated with decreased z-score (P =.004 and.041, respectively) although linear regression failed to identify any agent as independent predictor.
Children with single ventricle have high mortality and a profound medication burden. No individual medication is independently associated with better survival or weight gain during interstage period. Despite widespread use, proton pump inhibitors and prokinetic agents are not associated with better outcomes and may be associated with poor growth.
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ABSTRACT: Purpose of review:
Patients with single-ventricle, shunt-dependent physiology are at increased risk for interstage death due to the inherent instability of parallel circulation. Enhanced surveillance and early identification of deteriorating physiology via interstage home monitoring result in significant reduction in mortality. These programs are an important focus of improving outcomes for patients with single-ventricle heart disease.
In the multi-institutional Pediatric Heart Network Single-Ventricle Reconstruction Trial, interstage mortality was 12%, highlighting the continued opportunity to improve on this metric. A number of single-center series have demonstrated significant benefit of interstage monitoring on survival and growth. The focus on interstage monitoring by the National Pediatric Cardiology Quality Improvement Collaborative of the Joint Council on Congenital Heart Disease should improve our understanding of patients at greatest risk and help establish best practices for interstage care. In addition, a number of pilot projects utilizing newer communication technologies seek to improve the connection between program and patient.
Interstage home monitoring programs are a model of collaborative care that improves outcomes. Continued research in this area will refine the elements of home monitoring programs and continue to guide improved results. In addition, this model may serve as a template for the care of other populations of medically complex infants.
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