Growth and correlates of nutritional status among infants with hypoplastic left heart syndrome (HLHS) after stage 1 Norwood procedure

Background Protein-energy malnutrition is common among infants with congenital heart disease. We hypothesized that infants with hypoplastic left heart syndrome (HLHS) are at risk for malnutrition. Objective To determine the prevalence of and risk factors for malnutrition in infants undergoing palliative surgery for HLHS. Methods Retrospective chart review of 50 infants with HLHS who underwent both stage 1 Norwood and bidirectional Glenn (BDG) procedures over 4.5 y. Results After a median hospital stay of 21 d, median discharge weight was 3.4 kg, unchanged from admission. Adjusting for weight on admission, children with longer length of hospital stay, longer intensive care unit stay, shorter duration of parental nutrition therapy, and higher diuretic dosage at discharge had a lower weight-for-age Z score at discharge (R2 = 0.85). On admission for BDG, median weight-for-age Z score was −2.0. After adjusting for weight on discharge from the initial hospitalization, children with fewer calories/ounce of their enteral nutrition at discharge, worse right ventricular function, more frequent readmissions, and higher oxygen saturation at discharge had a lower weight-for-age Z score at BDG (R2 = 0.49). Conclusions Malnutrition is common in infants with HLHS after stage 1 palliation. Variables associated with more complex postoperative course and imbalance between systemic and pulmonary blood flow were all associated with poorer nutritional status. When adjusting for these factors, the use of parenteral nutrition and high calorie enteral feeds were associated with improved nutritional status. Aggressive parenteral and enteral nutritional therapy might help reduce the prevalence of growth faltering in infants who have HLHS.