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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Mobile health technology is an emerging tool in interstage home monitoring for infants with single ventricle heart disease or biventricular shunt-dependent defects. This study sought to describe adherence to mobile health monitoring and identify factors and outcomes associated with adherence to mobile health monitoring. This was a retrospective, single-institution study of infants who were followed in a mobile health-based interstage home monitoring programme between February 2016 and October 2020. The analysis included 105 infants and subjects were grouped by frequency of adherence to mobile health monitoring. Within the study cohort, 16 (15.2%) had 0% adherence, 25 (23.8%) had <50% adherence, and 64 (61.0%) had >50% adherence. The adherent groups had a higher percentage of infants who were male (p = 0.02), white race (p < 0.01), non-Hispanic or non-Latinx ethnicity (p < 0.01) and had mothers with primary English fluency (p < 0.01), married marital status (p < 0.01), and a prenatal diagnosis of faetal cardiac disease (p = 0.03). Adherent groups also had a higher percentage of infants with non-Medicaid primary insurance (p < 0.01) and residence in a neighbourhood with a higher median household income (p < 0.04). Frequency of adherence was not associated with interstage mortality, unplanned cardiac reinterventions, or hospital readmissions. Impact of mobile health interstage home monitoring on caregiver stress as well as use of multi-language, low literacy, affordable mobile health options for interstage home monitoring warrant further investigation.

Introduction: Patients with Fontan circulation often develop multiorgan dysfunction. Obesity is a risk factor for systemic complications in the general population, but the impact of obesity in Fontan circulation is unclear. This study investigates the impact of increased BMI on end-organ function in Fontan survivors. Methods: Single center data from patients 13 years and older with Fontan circulation were retrospectively collected from June 2018 to May 2020. Weight at clinic visit was categorized as normal or overweight/obese by WHO BMI-for-age classifications. End-organ function between normal and overweight patients was assessed. The relationship between overweight and measures of end-organ function were modeled via multivariable regression analyses controlling for age, sex, race, heterotaxy, time since Fontan, ventricular function, and atrioventricular valve (AVV) regurgitation. Results: Among 75 patients (median age 18 [15, 26] years), 24 (32%) were overweight or obese. Age, race, duration since Fontan, oxygen saturation, and dominant ventricle did not differ between groups. There were significantly more overweight females (p= 0.02). Overweight patients had significantly lower GFR (p= 0.01), lower predicted peak VO2 (p= 0.02), and higher systemic vascular resistance (SVR) (p= 0.02) than normal weight patients. Duration since Fontan (β= 0.38, p = 0.02) and overweight (β= -0.35, p = 0.03) were independently associated with GFR. Female sex (β= -0.42, p < 0.01) and overweight were independently associated with lower predicted peak VO2. Overweight was independently associated with presence of high SVR (OR = 7.5, p= 0.03). Other tested variables for hepatic (MELD-XI score, liver stiffness), cardiovascular (cardiac index, Fontan pressure, ventricular and AVV function), pulmonary, bone, and hematologic function did not significantly differ between groups. Conclusions: Overweight/obese BMI is common in aging Fontan patients and is significantly associated with worse renal function, exercise capacity, and elevated SVR. These findings suggest a possible relationship between weight, vascular changes, and renal perfusion. Further studies are needed to investigate this mechanism and the impact on Fontan patient outcome.

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