Cerebral Blood Flow in Single Ventricle and Normal Children Utilizing MRI

利用 MRI 研究单心室和正常儿童的脑血流

• 批准号: 7666774
• 负责人: Mark A Fogel
• 金额: $ 60.65万
• 依托单位: CHILDREN'S HOSP OF PHILADELPHIA
• 依托单位国家: 美国
• 财政年份: 2008
• 资助国家: 美国
• 起止时间: 2008-09-01 至 2013-12-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7666774
• 关键词: Acidosis; Adverse effects; Affect; Age; Altitude; Alveolar; Anatomy; Aorta; Blood; Blood Pressure; Blood flow; Brain; Bypass; Carbon Dioxide; Cerebrovascular Circulation; Cerebrum; Child; Clinical; Cohort Studies; Common Ventricle; Coronary; Cross-Sectional Studies; Data; Defect; Development; Diastolic blood pressure; Diffusion Magnetic Resonance Imaging; Economics; Feedback; Fontan Procedure; General Population; Heart; Hypercapnia; Hypercapnic respiratory failure; Hypoxia; Image; Imaging Techniques; Inferior; Intelligence; Lead; Lesion; Literature; Lung; Magnetic Resonance Imaging; Measures; Medical; Modification; Morbidity - disease rate; Nervous System Physiology; Neurodevelopmental Deficit; Neurologic; Operative Surgical Procedures; Outcome; Pathology; Pathway interactions; Patients; Phase; Physiology; Publishing; Pulmonary Circulation; Pulmonary artery structure; Pulse Pressure; Pump; Randomized; Recruitment Activity; Regulation; Route; Series; Shunt Device; Side; Source; Spin Labels; Staging; Superior vena cava structure; Surgical Management; System; United States; Upper Extremity; Vasoconstrictor Agents; Vasodilator Agents; Venous; Venous Pressure level; Ventricular; Weight; brain volume; cohort; congenital heart disorder; meetings; mortality; normal aging; operation; palliative; prospective; rapid growth; reconstruction; repaired; response; sucking

DESCRIPTION (provided by applicant): Children who are born with a heart containing only one usable pumping chamber (single ventricle) are the leading cause of morbidity and mortality from congenital heart disease (CHD) in the United States. The most common repair is the modified Fontan operation in which the right side of the heart is bypassed and blood is channeled directly to the lungs without a pumping chamber. This surgery is performed in stages. In the initial operation, a connection is created from the aorta to the pulmonary arteries and the outflow from the heart is reconstructed (Stage I). In an intermediate stage, flow from the superior vena cava is channeled directly into the pulmonary arteries while the inferior vena caval flow enters the single ventricle directly. The Fontan completion involves routing inferior vena caval blood to the pulmonary arteries via a baffle or conduit. Patients with single ventricle are also known to have greater neurological problems than the general population and other forms of CHD. One contributing factor is cerebral blood flow (CBF); evidence from preliminary data from our lab as well as in the published literature suggests that the amount of CBF is related to intelligence. There are reasons to believe that CBF in single ventricle patients is different from normal children and changes throughout staged surgical reconstruction. At Stage I, the connection created between the aorta and pulmonary arteries decrease blood pressure and suck blood into the lungs. In the second stage, the brain and lungs are hooked up directly in series with each other and only with each other (blood flows from the aorta to the brain and then directly into the superior vena cava to the lungs). Finally, after complete Fontan reconstruction, brain venous pressures are elevated. The amount of CBF during these stages remain unknown along with the change in CBF with increased carbon dioxide which increases brain flow and is a marker for how well the brain can adapt to meet its blood flow needs. Indeed, there is no data on CBF in normal children in the literature. This is especially important at this age since this rapid growth stage of brain development may be related to ultimate neurological function. Elucidating CBF in single ventricles may ultimately lead to changes in management of these patients; if one stage has an adverse effect on CBF for example and is not normal, it might be prudent to complete the Fontan earlier rather than the typical 18 month timeframe. By accurately measuring CBF which our lab has done previously, magnetic resonance imaging (MRI) offers a unique opportunity to measure CBF non-invasively, CBF response to carbon dioxide as well as brain volumes and abnormalities. Understanding how CBF changes in single ventricles with the stage of surgery as compared to normal children may lead to understanding why they have neurological problems and possibly change medical or surgical management. This is a prospective, non-randomized, single center study built upon preliminary data on CBF in single ventricle patients and other forms of CHD via MRI and seeks to determine CBF changes in single ventricles during staged surgical reconstruction as compared with normal children. Project Narrative: Children who are born with a heart containing only one usable pumping chamber (single ventricle lesion) are the leading cause of morbidity and mortality from congenital heart disease in the United States and suffer from neurological problems. By understanding how blood flow to the brain is different from normals and how it changes during surgery in these patients, we may better be able to understand why they have neurological problems and what can be done to intervene. Changing the way the blood flows in these patients by surgery may alter the way we ultimately manage these patients medically and surgically.

描述（由申请人提供）：天生的儿童只有一个只有一个可用的抽水室（单个心室）是美国先天性心脏病（CHD）发病和死亡的主要原因。最常见的修复是修改的Fontan操作，其中绕过心脏的右侧，并将血液直接引导到肺部，而无需抽水室。该手术分阶段进行。在最初的操作中，从主动脉到肺动脉创建了连接，并且从心脏的流出进行了重建（i阶段I）。在中间阶段，从上腔静脉的流动直接引导到肺动脉中，而下腔静脉流动直接进入单个心室。 Fontan的完成涉及通过挡板或导管将下腔静脉血液路由到肺动脉。与普通人群和其他形式的CHD相比，单个心室患者的神经系统疾病也更大。一个促成因素是脑血流（CBF）；来自我们实验室以及已发表的文献的初步数据的证据表明，CBF的数量与智能有关。有理由认为，单脑室患者的CBF与正常儿童不同，并且整个分期手术重建的变化。在第一阶段，主动脉和肺动脉之间的联系降低了血压并吸收血液进入肺部。在第二阶段，大脑和肺部彼此之间直接连接起来，仅相互连接（血液从主动脉流向大脑，然后直接进入上腔静脉到肺部）。最后，经过完整的Fontan重建后，大脑静脉压力升高。这些阶段的CBF量以及CBF的变化随着二氧化碳的增加而尚不清楚，这增加了脑流量，并且是大脑能够适应以满足其血液流动需求的标志。确实，文献中普通儿童中没有关于CBF的数据。这在这个时代尤其重要，因为大脑发育的快速生长阶段可能与最终的神经功能有关。阐明单个心室中的CBF最终可能导致这些患者的管理变化；如果一个阶段对CBF产生不利影响，并且不正常，那么较早而不是典型的18个月时间表可能是谨慎的。通过准确测量我们实验室以前做过的CBF，磁共振成像（MRI）提供了一个独特的机会，可以非侵入性地测量CBF，CBF对二氧化碳以及脑量和异常的反应。与正常儿童相比，了解单个心室的CBF如何变化可能会导致理解他们出现神经系统问题的原因，并可能改变医疗或手术治疗。这是一项前瞻性的，非随机的单中心研究，基于单个心室患者的CBF和其他形式的CHD的初步数据，并试图确定与正常儿童相比，在上演手术重建过程中单脑室的CBF变化。项目叙述：天生的儿童只有一个只有一个可用的抽水室（单个心室病变）是美国先天性心脏病的发病率和死亡率的主要原因，并且患有神经系统问题。通过了解血液流向大脑的血液与正常性的不同以及在这些患者手术过程中的变化如何不同，我们可以更好地理解为什么它们存在神经问题以及可以采取什么措施进行干预。通过手术改变这些患者的血液流动方式可能会改变我们最终通过医学和外科手术管理这些患者的方式。