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: 8208093
• 负责人: Mark A Fogel
• 金额: $ 65.92万
• 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 2014-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8208093
• 关键词: Acidosis; Adverse effects; Affect; Age; Altitude; Alveolar; Anatomy; Aorta; Blood; Blood Circulation; 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; 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; Recruitment Activity; Regulation; 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; abstracting; brain volume; cohort; congenital heart disorder; meetings; mortality; normal aging; operation; palliative; prospective; rapid growth; reconstruction; repaired; response; sucking

Project Summary/Abstract: 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.

项目摘要/摘要： 出生时心脏只有一个可用的泵室(单心室)的儿童 在美国，先天性心脏病(CHD)是导致发病率和死亡率的主要原因。这个 最常见的修复手术是改良的Fontan手术，在该手术中，心脏的右侧被绕过，而 血液在没有泵室的情况下直接输送到肺部。这种手术是分阶段进行的。在 最初的手术是建立一个从主动脉到肺动脉的连接，然后从心脏流出 被重建(阶段I)。在中间阶段，从上腔静脉流出的血液被直接导入 当下腔静脉流出时，肺动脉直接进入单心室。方坦家族 完成手术需要通过挡板或管道将下腔静脉的血液输送到肺动脉。 众所周知，单脑室患者的神经系统问题也比一般患者更严重。 人口和其他形式的冠心病。其中一个因素是脑血流量(CBF)；证据来自 来自我们实验室的初步数据以及发表的文献表明，CBF的量与 为了情报。有理由相信单心室患者的脑血流量不同于正常 在整个手术过程中儿童和变化分期手术重建。在第一阶段，建立在 大动脉和肺动脉可以降低血压，并将血液吸入肺部。在第二阶段， 大脑和肺是直接串联在一起的，而且只有彼此相连(血液从 主动脉到脑，然后直接进入上腔静脉到肺)。最后，在完成方坦之后 重建后，脑静脉压升高。这些阶段的脑血流量仍不清楚。 随着二氧化碳含量的增加，脑血流量发生变化，这增加了大脑流量，是 大脑适应得有多好，以满足其血流需求。事实上，没有关于正常儿童脑血流量的数据 在文学作品中。这在这个年龄尤其重要，因为大脑发育的这个快速增长阶段可能 与终极神经功能有关。阐明单个脑室的CBF可能最终导致变化 在这些患者的管理中；例如，如果一个阶段对CBF有不良影响，并且不正常，它 提前完成丰坦项目可能是谨慎的，而不是典型的18个月时间框架。 通过准确测量脑血流量，我们的实验室以前已经做过，磁共振成像(MRI) 提供了一个独特的机会来非侵入性地测量CBF，CBF对二氧化碳的反应以及大脑 体积和异常。了解单心室血流量随手术分期的变化 与正常儿童相比，可能会导致理解为什么他们会有神经问题，并可能 改变内科或外科治疗。这是一项基于前瞻性、非随机、单中心的研究 通过MRI对单心室患者和其他形式的冠心病患者脑血流量的初步数据，并试图确定 与正常儿童相比，分期手术重建时单侧脑室血流量的变化。项目说明： 出生时心脏只包含一个可用的泵室(单心室)的儿童 病变)是美国先天性心脏病发病率和死亡率的主要原因， 患有神经问题。通过了解血液流入大脑的方式不同于正常和 在这些患者的手术中，它是如何变化的，我们或许能够更好地理解为什么他们会有 神经问题以及可以做些什么来干预。改变这些患者的血液流动方式 手术可能会改变我们最终在医学上和手术上管理这些患者的方式。