Neurodevelopmental Outcomes in Infants with HLHS after Hybrid Stage I Palliation

描述（由申请人提供）：左心发育不全综合征仍然是所有先天性心脏缺陷在出生后第一年死亡的主要原因。传统上，在生命的前2-3年进行三阶段的手术缓解，需要深度低温循环停止和体外循环。该队列患者的神经发育结果仍不理想。混合I期手术为第一阶段姑息提供了另一种方法。混合型手术不需要体外循环；因此，新生儿不暴露于循环骤停和体外循环的所有风险与潜在的神经损伤。在混合手术后，大约6个月大的婴儿进行下一阶段修复时，脑灌注依赖于血液通过支架状动脉导管未闭和逆行主动脉弓流向颈动脉，类似于胎儿生命。目前尚不清楚从主动脉弓到大脑的逆行血流是否足以维持正常的生长发育。本研究计划的长期目标是检验脑血流速度与左心发育不全综合征婴儿接受混合I期姑息治疗的神经发育结果之间存在相关性的假设。HLHS患者，以及正常年龄匹配的对照组，将被招募参加这项研究。经颅多普勒测量两组婴儿出生后以及2、4、6个月时的大脑中、前动脉血流。此外，婴儿发育评估将在相同的时间间隔进行，使用出生时和2个月和4个月的婴儿运动表现测试，以及6个月大时进行的Bayley婴幼儿发育量表第三版。这将与标准参照的年龄匹配对照进行比较。虽然预计HLHS患者的样本较少，但本研究将包括男女婴儿以及少数民族婴儿。本研究的经颅多普勒结果可能对神经认知发育结果产生深远的影响，这可能对患者、家庭和社会产生巨大的长期影响。这项研究与美国国立卫生研究院在生物医学和行为研究方面的使命保持一致，研究神经发育结果，结合目前经颅多普勒技术的创新，评估混合I期姑息治疗后HLHS患者的脑灌注。

DESCRIPTION (provided by applicant): Hypoplastic Left Heart Syndrome remains the leading cause of death of all congenital heart defects in the first year of life. Traditionally, three staged surgical palliations are performed during the first 2-3 years of life, requiring profound hypothermic circulatory arrest and cardiopulmonary bypass. Neurodevelopmental outcomes in this cohort of patients remain suboptimal. The Hybrid Stage I procedure offers an alternative approach for the first staged palliation. The Hybrid procedure does not require cardiopulmonary bypass; therefore the neonate is not exposed to all the risks of circulatory arrest and cardiopulmonary bypass with potential for neurologic injury. After the Hybrid procedure, cerebral perfusion is dependent upon the blood flow through the stented patent ductus arteriosus and retrograde aortic arch flow to the carotid arteries, similar to fetal life, for approximately 6 months of age when the next staged repair is performed. It remains unknown if the retrograde blood flow from the aortic arch to the brain is adequate for normal growth and development. The long-term objective of this research proposal is to test the hypothesis that a correlation exists between cerebral blood flow velocity and neurodevelopmental outcomes in hypoplastic left heart syndrome babies who have undergone Hybrid Stage I palliation. HLHS patients, along with normal age- matched controls, will be recruited for this study. Transcranial Doppler will be performed to measure the middle and anterior cerebral artery blood flow after birth, and at 2, 4, and 6 months of age in both groups. Additionally, infant developmental assessment will be performed at the same time intervals using the Test of Infant Motor Performance at birth and 2 and 4 months, as well as the Bayley Scale of Infant and Toddler Development, 3rd edition, performed at 6 months of age. This will be compared to norm-referenced age- matched controls. Although the sample of HLHS patients is expected to be low, this study will include infants of both gender, as well as minorities. The transcranial Doppler findings of this study may have a profound impact on neurocognitive developmental outcomes which may have a tremendous long-term impact on patients, families, and society. This study is aligned with the mission of the NIH in biomedical and behavioral research with neurodevelopmental outcomes, coupled with innovation of current technology with transcranial Doppler, to assess cerebral perfusion in this unique cohort of HLHS patients after Hybrid Stage I palliation.