This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Infants with congenital heart disease frequently experience growth retardation. Several factors may contribute to this growth retardation, including metabolic rate and decrease caloric intake. The purpose of the proposed research is to determine the effect of single ventricle physiology (hypoplastic left heart syndrome or HLHS) and cyanosis on total and resting energy expenditure and caloric intake in a group of 3-4 month old infants and group of healthy age-matched controls. We have restricted our patients population to those infants with isolated HLHS so as to examine the effect of only this lesion without complication arising from other factors. We will study those patients who have undergone Stage 1 Norwood procedure, which is the first of three surgical stages to full repair. The patient group will be subdivided as to whether the infant has minimal or significant tricuspid valve reflux. Knowledge of the energy expenditures of these patients is necessary to accurately gauge their energy requirements, provide optimal nutritional support and optimize the timing of surgical correction. Survival of the infant to the next stage of surgical repair may be improved by optimizing nutritional requirements.
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