The Cardiac Atlas Project

摘要 心脏畸形是最常见的出生缺陷类型。改进对 复杂的先天性心脏病导致>90%的先天性心脏病患者现在能够 才能活到成年早期仅在美国，就有更多的成年人患有先天性心脏病（约1 （百万人）比孩子。法洛四联症（TOF）是最常见的紫绀型先天性心脏病， 并且现在有大量且不断增长的成人TOF患者。这些病人需要的手术修复 在生命的早期常常会导致残余肺动脉回流，最终会导致右心室扩大 和功能障碍，易患心力衰竭、心律失常和猝死。在临床过程中， 管理，决定是否以及何时进行肺动脉瓣置换术，以防止心室 因此，失代偿是至关重要的，并保证了可量化的评估手段。 这个项目的目标是扩大目前的先天性心脏病研究的心脏图谱 项目数据库包括一个大型术后TOF患者队列，并使用患者心脏 磁共振（CMR）检查和其他临床数据，以获得形状、生物力学 和电不同步。这些地图集将用于测试假设和发现临床生物标志物 根据心室形状、机械强度、 性能和机电不同步。 具体目标是：（1）使用非侵入性的疾病特异性统计形状图谱， 对TOF患者进行成像检查，以测试特定双心室形状模式可以 根据心室功能恢复程度和心脏逆转程度区分患者 瓣膜置换术后重构;（2）利用双心室生物力学有限元模型， 研究心室形状模式与 瓣膜修复后心室功能的改善是由于形状单独与心肌 材料特性;以及（3）为了确定如何不同步的电激活模式，引起 非均匀性局部收缩对血流动力学改善和逆转心室重构影响 TOF患者的肺动脉瓣置换术。 这项工作将对先天性心脏病的临床管理产生重大影响， 提供：对术后TOF生物力学变化的新见解;新的结局预测因子 肺动脉瓣置换术后;以及一个大大扩展的CMR检查网络访问数据库， 模型和统计地图集，以促进先天性心脏病的临床研究和培训。

ABSTRACT Cardiac malformations are the most common type of birth defect. Improvements in the management of complex congenital heart disease have resulted in >90% of those born with congenital heart disease now able to survive into early adulthood. In the U.S. alone, there are more adults with congenital heart disease (~1 million individuals) than children. Tetralogy of Fallot (TOF) is the commonest cyanotic congenital heart lesion, and there is now a large and growing population of adults with TOF. The surgical repairs these patients require early in life often lead to residual pulmonary regurgitation that can cause eventual right ventricular enlargement and dysfunction, and pre-dispose to heart failure, arrhythmias, and sudden death. In the course of clinical management, deciding whether and when to perform pulmonary valve replacement to prevent ventricular decompensation is therefore critical and warrants quantifiable means of assessment. The goal of this project is to expand the current Congenital Heart Disease study of the Cardiac Atlas Project database to include a large cohort of patient with post-operative TOF, and to use the patient cardiac magnetic resonance (CMR) exams and other clinical data to derive statistical atlases of shape, biomechanics and electrical dyssynchrony. These atlases will be used to test hypotheses and discover clinical biomarkers that predict outcomes of pulmonary valve replacement based on variations in ventricular shape, mechanical properties and electromechanical dyssynchrony. The specific aims are: (1) To use disease-specific statistical shape atlases derived from non-invasive imaging exams of patients with TOF to test the hypothesis that specific biventricular shape modes can discriminate patients by their degree of recovery of ventricular function and their extent of reverse cardiac remodeling after valve replacement; (2) To use finite element models of biventricular biomechanics to investigate the extent to which clinically significant relationships between ventricular shape modes and improvements in ventricular function post-valve repair are due to shape alone vs. differences in myocardial material properties; and (3) To determine how dyssynchronous electrical activation patterns that give rise to heterogeneous regional contraction affect hemodynamic improvement and reverse ventricular remodeling after pulmonary valve replacement in TOF patients. This work will have a significant impact on the clinical management of congenital heart disease by providing: new insights into biomechanical alterations in post-operative TOF; new predictors of outcomes following pulmonary valve replacement; and a greatly expanded web-accessible database of CMR exams, models and statistical atlases to facilitate clinical research and training in congenital heart disease.