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: 8011215
• 负责人: Mark A Fogel
• 金额: $ 66.73万
• 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/8011215
• 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; 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）：在美国，出生时心脏仅包含一个可用泵室（单心室）的儿童是先天性心脏病 (CHD) 发病和死亡的主要原因。最常见的修复是改良的 Fontan 手术，其中绕过心脏的右侧，将血液直接输送到肺部，而无需泵室。该手术分阶段进行。在初始手术中，建立从主动脉到肺动脉的连接，并重建心脏的流出道（第一阶段）。在中间阶段，来自上腔静脉的血流直接进入肺动脉，而下腔静脉血流直接进入单心室。 Fontan 完成涉及将下腔静脉血液通过挡板或导管输送至肺动脉。众所周知，单心室患者比普通人群和其他形式的先心病患者有更严重的神经系统问题。影响因素之一是脑血流量 (CBF)；我们实验室的初步数据以及已发表的文献表明，CBF 的量与智力有关。有理由相信单心室患者的 CBF 与正常儿童不同，并且在分期手术重建过程中会发生变化。在第一阶段，主动脉和肺动脉之间建立的连接降低了血压并将血液吸入肺部。在第二阶段，大脑和肺部直接相互串联并且仅相互连接（血液从主动脉流向大脑，然后直接进入上腔静脉到达肺部）。最后，在完成 Fontan 重建后，脑静脉压升高。这些阶段的 CBF 量仍然未知，随着二氧化碳增加，CBF 发生变化，从而增加脑流量，并且是大脑适应满足其血流需求的能力的标志。事实上，文献中没有关于正常儿童CBF的数据。这在这个年龄段尤其重要，因为大脑发育的这个快速生长阶段可能与最终的神经功能有关。阐明单心室 CBF 可能最终导致这些患者治疗方法的改变；例如，如果某个阶段对 CBF 产生不利影响并且不正常，则明智的做法是提前完成 Fontan，而不是典型的 18 个月时间范围。通过我们实验室之前精确测量 CBF，磁共振成像 (MRI) 提供了一个独特的机会来非侵入性测量 CBF、CBF 对二氧化碳的反应以及脑容量和异常情况。与正常儿童相比，了解单心室的 CBF 如何随着手术阶段的变化而变化，可能有助于理解他们为什么会出现神经系统问题，并可能改变医疗或手术治疗。这是一项前瞻性、非随机、单中心研究，基于通过 MRI 获得的单心室患者和其他形式的 CHD 的 CBF 初步数据，旨在确定分阶段手术重建期间单心室与正常儿童相比的 CBF 变化。项目叙述：出生时心脏仅包含一个可用泵室（单心室病变）的儿童是美国先天性心脏病发病和死亡的主要原因，并患有神经系统问题。通过了解流向大脑的血流与正常人的不同以及这些患者在手术过程中的变化，我们也许能够更好地理解他们为什么会出现神经系统问题以及可以采取哪些干预措施。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对这些患者进行医疗和手术的方式。