The Cardiac Atlas Project

摘要 心脏畸形是最常见的出生缺陷类型。在管理方面有所改进 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，患有先天性心脏病的成年人就更多(~1 百万人)，而不是儿童。法洛四联症(TOF)是最常见的青紫型先天性心脏病， 现在有一大批患有TOF的成年人，而且人数还在不断增加。这些病人所需的外科修复 生命早期常常导致残存的肺返流，这可能导致最终的右室增大。 和功能障碍，并易发生心力衰竭、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瓣膜置换以防止室性心动过速 因此，失代偿是至关重要的，需要量化的评估手段。 该项目的目标是扩大目前心脏图谱的先天性心脏病研究 项目数据库，包括大量术后TOF患者，并使用患者的心脏 磁共振(CMR)检查和其他临床数据，以得出形状、生物力学的统计图谱 和电不同步。这些图谱将用于检验假说和发现临床生物标记物。 根据心脏形态、机械状态的变化预测肺瓣膜置换术的结果 特性和机电不同步。 具体目标是：(1)使用来自非侵入性疾病的特定疾病的统计形状图谱 对TOF患者进行影像检查，以验证特定的双心室形态模式可以 根据患者的心功能恢复程度和心脏反转程度来区分患者 瓣膜置换术后的重塑；(2)应用生物力学有限元模型 研究脑室形态模式与临床显著关系的程度。 瓣膜修复术后心功能的改善是由于形状与心肌的差异 材料特性；以及(3)确定不同步电激活模式如何引起 异质性局部收缩影响术后血流动力学改善和逆转心室重构 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的生物力学改变的新见解；对预后的新预测因素 在肺瓣膜置换术后；以及一个大大扩展的网络可访问的CMR检查数据库， 模型和统计图谱，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.