ENERGY EXPENDITURE IN INFANTS WITH HYPOPLASTIC LEFT HEART SYNDROME (HLHS)

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。患有先天性心脏病的婴儿经常出现生长迟缓。有几个因素可能导致这种生长迟缓，包括代谢率和热量摄入的减少。本研究的目的是确定单心室生理学（左心发育不全综合征或HLHS）和紫绀对3-4个月大婴儿和年龄匹配的健康对照组的总能量消耗和静息能量消耗和热量摄入的影响。我们将患者人群限制在孤立性HLHS的婴儿中，以便仅检查该病变的影响，而不检查其他因素引起的并发症。我们将研究那些接受了诺伍德手术1期的患者，这是三个手术阶段中的第一个完全修复。根据婴儿是否有轻微或明显的三尖瓣反流，对患者组进行细分。了解这些患者的能量消耗对于准确测量他们的能量需求，提供最佳的营养支持和优化手术矫正的时机是必要的。通过优化营养需求，可以提高婴儿在手术修复下一阶段的存活率。

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.