Supplemental tube feeding does not mitigate weight loss in infants with shunt-dependent single-ventricle physiology.

Infants with shunt-dependent single-ventricle (SV) physiology are at risk for poor weight gain before superior cavopulmonary connection (SCPC). Lower weight-for-age z-score is a risk factor for prolonged length of stay (LOS) after SCPC. We sought to characterize infant growth and feeding and determine the effect of method of feeding on outcomes. Chart review of infants with shunt-dependent SV physiology born between October 2007 and September 2010 was performed. The cohort was divided into three groups based on feeding method at discharge after initial palliation; 53 in the oral feeding (PO) group, 56 in the nasogastric (NG) tube group, and 26 in the gastrostomy tube (GT) group. Birth weight z-score did not differ among groups (p = 0.39), but infants fed by NG or GT were smaller than PO-fed infants at hospital discharge (p = 0.0001), a difference that persisted through SCPC (p < 0.0001). Predictors of need for GT included Norwood procedure (p = 0.008) and longer LOS after initial palliation (p < 0.001). Interstage mortality and age at SCPC did not differ among groups. Risk factors for longer LOS at SCPC included longer LOS and need for supplemental feeds at discharge from initial palliation as well as lower weight at SCPC. Poor growth is common among infants with shunt-dependent SV physiology. Infants who require GT have lower weight-for-age z-scores at hospital discharge and remain smaller at SCPC than those fed PO. Although GT after initial palliation is associated with longer LOS after SCPC, it is not associated with an increase in interstage morbidity or mortality.

18 years of the Fontan operation at a single institution: results from 771 consecutive patients.

OBJECTIVES: The aim of this study was to evaluate Fontan peri-operative outcomes for 771 consecutive patients. BACKGROUND: Since the initial description by Fontan, mortality associated with the Fontan operation has declined substantially. However, postoperative effusions remain a significant challenge. Effusions are a key determinant of postoperative length-of-stay and have been shown to be associated with the development of protein-losing enteropathy and with decreased survival. METHODS: This study was a single-center, retrospective review of 771 patients who underwent Fontan palliation from 1992 to 2009. RESULTS: Patients were divided into 3 eras dictated by shift in clinical practice. Overall mortality was 3.5%, 1% since 1996. Importantly, age at Stage II palliation decreased from Era 1 to Era 3 (7.1 vs. 5.9 months; p = 0.0001), whereas age at Fontan increased (1.7 vs. 2.8 years; p = 0.0001). The proportion of patients with prolonged hospital stay (46.7% vs. 8.2% vs. 19.5%, p < 0.001) decreased substantially after Era 1. A diagnosis of hypoplastic left heart syndrome and longer operative support times were associated with prolonged pleural drainage (odds ratio [OR]: 2.17, p < 0.001; OR: 1.2, p = 0.001) and hospital stay (OR: 1.48, p = 0.05; OR: 1.34, p < 0.001). In patients who underwent invasive assessment, higher pulmonary artery pressure was associated with death (OR: 1.37, p < 0.001) and prolonged hospital stay (OR: 1.09, p = 0.019). Pulmonary arterial pressure ≥15 mm Hg was 90% specific for discriminating unfavorable outcomes. CONCLUSIONS: Mortality in the modern era is rare, whereas postoperative pleural drainage remains the dominant morbidity. Elevated pulmonary artery pressure seems to be a marker of unfavorable outcome. Continued investigation is warranted to determine whether medical interventions or alterations to operative strategy can alter peri-operative results and improve long-term outcomes.