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

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。患有先天性心脏病的婴儿经常出现发育迟缓。几个因素可能导致这种生长迟缓，包括代谢率和卡路里摄入量减少。本研究的目的是在3-4个月大的婴儿和年龄匹配的健康对照组中，确定单心室生理学(左心发育不良综合征)和发紫对婴儿总能量消耗和静息能量消耗以及热量摄入量的影响。我们将我们的患者群体限制在那些患有孤立性HLHS的婴儿身上，以便只检查这种病变的效果，而不会因为其他因素而产生并发症。我们将研究那些已经接受了第一阶段Norwood手术的患者，这是三个手术阶段中的第一个阶段，完全修复。根据婴儿是否有轻微或明显的三尖瓣反流，患者组将被细分。了解这些患者的能量消耗对于准确评估他们的能量需求、提供最佳营养支持和优化手术矫正时机是必要的。通过优化营养需求，可以提高婴儿到下一阶段手术修复的存活率。

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.