Functional Cardiovascular 4D MRI in Congenital Heart Disease

描述（由申请人提供）：先天性心脏病（CHD）是最常见的出生缺陷，约占所有活产婴儿的1.2%，是出生缺陷相关死亡的主要原因。冠心病患者需要频繁的诊断检查，以便计划手术修复，评估术前风险，和/或调查重要的长期并发症。双尖瓣主动脉瓣（BAV）是最常见的冠心病形式之一，而单心室生理（SVP）是最严重的冠心病形式之一，这两个患者队列需要进行这种纵向监测。然而，标准诊断工具通常涉及侵入性导管手术、电离辐射和/或长达60-90分钟的磁共振成像（MRI）检查，需要对儿科患者进行镇静或全身麻醉。最近的研究表明，全身麻醉可能对新生儿和幼儿的神经、认知和社会发育产生不利影响。此外，对BAV或SVP等冠心病疾病的常见结局指标比较粗糙，依赖于主动脉根直径或术后临床状态等简化参数，不能反映疾病进展的潜在机制。进一步了解哪些BAV和SVP患者存在主动脉病变或Fontan生理功能衰竭的风险，将通过确定定期随访间隔和制定转诊手术矫正的精确标准来改善患者管理和治疗计划。我们的目标是开发一种新的全面的20分钟心血管功能4D磁共振检查，可以取代长时间的标准磁共振成像方案，减少或消除全身麻醉的暴露。患者特异性的事后分析将允许回顾性量化心功能和血流，而不局限于预定义的二维扫描平面。此外，新的血液动力学生物标志物将被衍生并评估其作为改善BAV和SVP患者预后预测的预后标志物的潜力。

DESCRIPTION (provided by applicant): Congenital heart disease (CHD) represents the most common birth defect and affects approximately 1.2% of all live births and is the leading cause of birth defect-related deaths. CHD patients require frequent diagnostic testing in order to plan surgical repair, assess preoperative risk, and/or survey for important long-term complications. Bicuspid aortic valve (BAV), one of the most common forms of CHD, and single ventricle physiology (SVP), one of the most severe, are two patient cohorts which will require such longitudinal surveillance. Standard diagnostic tools, however, often involve invasive catheter-based procedures, ionizing radiation, and/or lengthy 60-90 minute Magnetic Resonance Imaging (MRI) exams, necessitating sedation or general anesthesia in pediatric patients. Recent studies suggest that general anesthesia could adversely affect neurologic, cognitive, and social development of neonates and young children. In addition, common outcome measures for CHD diseases such as BAV or SVP are coarse and rely on simplified parameters such as the diameter of the aortic root or the post-operative clinical status which do not reflect the underlyig mechanisms of disease progression. Further knowledge of which BAV and SVP patients are at risk for aortopathy or failing Fontan physiology would improve patient management and therapy planning by defining regular follow-up intervals and generating precise criteria for referral to surgical correction. Our goal is to develop and a new comprehensive 20-minute cardiovascular functional 4D MR exam that can replace the long standard MR imaging protocol and reduce or eliminate exposure to general anesthesia. Patient-specific post-hoc analysis will allow retrospective quantification of cardiac function and flow without limitation to predefined 2D scan planes. In addition, new hemodynamic biomarkers will be derived and evaluated for their potential as prognostic markers for improved outcome prediction in patients with BAV and SVP.