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.

The Single Ventricle Program improved interstage weight gain, thereby allowing for early second-stage palliation at an equivalent patient weight. Interstage mortality was not significantly reduced by our program. However, 1-year transplant-free survival was significantly improved in patients in the Single Ventricle Program.

3b. Laryngoscope, 127:167-172, 2017.

The reported incidence of VFP following cardiac surgery via median sternotomy ranges between 1.7% and 67% depending on the type of surgery and the weight of the infant at the time of surgery. In our cohort, 19.7% had VFP. Surgery requiring aortic arch manipulation had a higher incidence of complications and required longer hospitalizations. These results may be used to improve informed consent and to manage postoperative expectations by identifying patients who are at higher risk for complications.

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Postoperative nutrition fails to meet the needs of infants with hypoplastic left heart syndrome despite increased focus on nutritional support. Modifiable factors (eg, nutritional intake) and hemodynamic factors (eg, tricuspid regurgitation) may play roles in the poor weight gain of these infants.

Background Feeding dysfunction occurs commonly in infants with single ventricle heart disease and impacts growth and long-term outcomes. Little evidence exists to guide safe feeding in this population. This study surveyed centers participating in the National Pediatric Cardiology Quality Improvement Collaborative to assess prevailing feeding practices amongthose caring for single ventricle neonates. Methods Web-based survey of 56 pediatric cardiac surgical centers was conducted. Questions addressed peri-operative feeding approaches and responses were presented and analyzed descriptively. Results Of 56 centers, 46 (82%) completed a survey. Preoperative feeding was common in single ventricle infants (30/46; 65%), routes varied. Centers who did not feed infants preoperatively cited the risk of necrotizing enterocolitis (16/16; 100%), presence of umbilical artery catheter (12/16; 75%), and prostaglandin infusion (9/16; 56%) as main concerns. 67% of centers reported no specific vital sign thresholds for withholding enteral feedings. In the postoperative period, most centers used an "internal guideline" (21/46; 46%) or an "informal practice" (15/46; 33%) to determine feeding readiness. Approaches to findings were significantly different among centers. About 40% of centers did not send patients home with feeding tubes, and there was no clear consensus between preferred feeding tube modality at discharge. Conclusion Considerable variation exists in feeding practices for infants with single ventricle congenital heart disease among 46 centers participating in a quality improvement collaborative. Although most centers generally feed infants preoperatively, feeding practices remain center-specific. Variability continues in the immediate post-operative and interstage periods. Further opportunities exist for investigation, standardization and development of best-practice feeding guidelines.

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