Neurodevelopmental Outcomes in Infants with HLHS after Hybrid Stage I Palliation

混合 I 期姑息治疗后 HLHS 婴儿的神经发育结果

• 批准号: 8135374
• 负责人: Sharon L. Hill
• 金额: $ 2.68万
• 依托单位: OHIO STATE UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 2009
• 资助国家: 美国
• 起止时间: 2009-09-30 至 2012-09-29
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8135374
• 关键词: Accounting; Age; Age-Months; Behavioral Research; Biomedical Research; Birth; Blood Flow Velocity; Blood flow; Brain; Cardiac Surgery procedures; Cardiopulmonary Bypass; Carotid Arteries; Cause of Death; Cerebrovascular Circulation; Cerebrum; Child; Complex; Congenital Abnormality; Congenital Heart Defects; Coupled; Defect; Development; Family; Gender; Goals; Growth and Development function; Heart; Heart Arrest; Hybrids; Hypoplastic Left Heart Syndrome; Infant; Injury; Intervention; Left ventricular structure; Lesion; Life; Literature; Measures; Minority; Mission; Mitral Valve; Morbidity - disease rate; Motor; Neonatal; Neurocognitive; Neurologic; Operative Surgical Procedures; Outcome; Patent Ductus Arteriosus; Patients; Performance; Perfusion; Play; Population; Population Research; Procedures; Recruitment Activity; Research Proposals; Residual state; Risk; Role; Sampling; Societies; Staging; Stenosis; Technology; Testing; Time; Toddler; United States National Institutes of Health; Western World; Work; anterior cerebral artery; aortic arch; aortic valve; ascending aorta; cohort; congenital heart disorder; design; fetal; improved; infant outcome; innovation; mortality; neonate; neurodevelopment; normal aging; palliation; palliative; repaired; time interval

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.

描述（由申请人提供）：在生命的第一年，左心脏综合征的低塑性左心脏综合征仍然是所有先天性心脏缺陷的主要原因。传统上，在生命的头2 - 3年中进行了三场手术姑息，需要深刻的低温循环停滞和心肺旁路。这一患者队列中的神经发育结果仍然是次优的。混合阶段I程序为第一次阶段palliation提供了另一种方法。混合过程不需要心肺旁路；因此，新生儿没有暴露于循环停滞和心肺旁路的所有风险，并可能导致神经系统损伤。杂交过程后，脑灌注取决于通过支架的专利导管的血液流动和逆行的主动脉弓流到颈动脉动脉，类似于胎儿寿命，大约6个月大约在下一次上演修复时。尚不清楚从主动脉弓向大脑的逆行血流是否足以满足正常生长和发育。这项研究建议的长期目标是检验以下假设：在脑血流速度和神经发育效果之间存在相关性，在患有混合阶段I pAlliation的左心脏综合征婴儿中存在相关性。 HLHS患者以及正常年龄匹配的对照组将被招募参加本研究。将进行经颅多普勒，以测量出生后的中部和前动脉血流，两组年龄在2、4和6个月中。此外，婴儿发育评估将在出生时和2和4个月的婴儿运动表现以及第3版的婴儿和幼儿发育的Bayley量表上进行同一时间间隔进行，该评估将在6个月大时进行。这将与标准参考年龄匹配的对照组进行比较。尽管预计HLHS患者的样本较低，但该研究将包括性别和少数族裔的婴儿。这项研究的经颅多普勒发现可能对神经认知的发展结果产生深远的影响，这可能会对患者，家庭和社会产生巨大的长期影响。这项研究与NIH在生物医学和行为研究中的使命与神经发育结局，再加上当前技术与经颅多普勒的创新，以评估这种独特的HLHS患者在混合阶段I palliation后的独特群体中评估脑灌注。