Computer Modeling of the Tricuspid Valve in Hypoplastic Left Heart Syndrome

项目摘要/摘要 左心发育不全综合征(HLHS)的特征是左心发育不全并影响 在美国，每年有1000多名活产婴儿。分期手术治疗允许患有HLHS的儿童 为了生存和繁荣，但右心室(RV)仍然是唯一起作用的心室和三尖瓣 (TV)是他们唯一起作用的房室瓣膜。因此三尖瓣返流与心脏高度相关。 失败和死亡。30%的HLHS患者需要手术干预来治疗三尖瓣反流。 尽管循序渐进，但对TV结构和三尖瓣返流关系的理解 HLHS的手术效果有限，手术修复效果不佳。三维超声心动图与定量三维 基于超声心动图的分析极大地改进了成人二尖瓣手术的计划和修复。 然而，尽管用基本工具进行的初步工作表明电视的3D结构之间的关系 和患者的生存，以及对准确的结构信息的需求，以指导电视维修，没有 商业化或现成的方法，充分适用于建模和量化独一无二的高度 HLHS中的可变电视解剖。因此，3D超声心动图图像中的信息仍然是潜在的，有限的 对电视结构与三尖瓣返流关系的认识及三尖瓣反流设计 通过3D结构分析了解患者特定的修复情况。这一建议是基于乔利博士在 先天性心脏病房室瓣膜模型及灵活开源软件的开发 建模工具。具体目标是1)研究从3D中量化电视结构的方法 使用基于度量工具的超声心动图图像以及新的形状参数化和统计形状 分析；以及2)使用这些工具来确定100名HLHS患者电视功能障碍的结构相关性 已经完成了阶段性手术修复。开发的方法将被合并到开放源代码的3D 切片成像处理平台，催化未来对先天性和成人结构性心脏病的研究， 尤其是那些不可能使用当前模式的患者。这些研究是迈向个性化的第一步。 通过3D结构分析，对电视机进行外科修复，并详细了解其结构特点 HLHS的电视功能障碍。该提案利用了现有的基础设施和广泛的专业知识 费城儿童医院、女王大学和Kitware公司。总体而言，这项研究代表了一项 在HLHS中对三尖瓣病变的认识方面取得了重大进展，以及灵活、可扩展的 为未来先天性和成人结构性心脏病的影像研究提供工具。

PROJECT SUMMARY/ABSTRACT Hypoplastic left heart syndrome (HLHS) is characterized by incomplete development of the left heart and affects over 1,000 live born infants in the United States per year. Staged surgical treatment allows children with HLHS to survive and flourish, but the right ventricle (RV) remains the sole functioning ventricle and the tricuspid valve (TV) is their only functional atrioventricular valve. Tricuspid regurgitation is thus highly associated with heart failure and death. Thirty percent of HLHS patients require surgical intervention to treat tricuspid regurgitation. Despite incremental progress, understanding of the relationship between TV structure and tricuspid regurgitation in HLHS is limited, and results of surgical repair are suboptimal. 3D echocardiography and quantitative 3D echocardiography based analysis have dramatically improved adult mitral valve surgical planning and repair. However, despite preliminary work with basic tools suggesting relationships between the 3D structure of the TV and patient survival, and the unmet need for precise structural information to guide TV repair, there is no commercial or readily available method sufficiently adaptable to model and quantify the unique and highly variable TV anatomy in HLHS. As a result, the information in 3D echocardiography images remains latent, limiting understanding of the relationship between TV structure and tricuspid regurgitation, as well as the design of patient-specific repairs informed by 3D structural analysis. This proposal builds upon Dr. Jolley’s experience with modeling of atrioventricular valves in congenital heart disease and the development of flexible open-source modeling tools. The specific goals are to 1) investigate methods for quantifying TV structure from 3D echocardiography images using metric based tools as well as novel shape parameterization and statistical shape analysis; and 2) use these tools to identify TV structural correlates of TV dysfunction in 100 HLHS patients who have completed staged surgical repair. The methods developed will be incorporated into the open-source 3D Slicer imaging processing platform to catalyze future studies of congenital and adult structural heart disease, particularly those not possibly using current modalities. These studies are the first step toward individualized surgical repair of the TV informed by 3D structural analysis, and a detailed understanding of structural features of TV dysfunction in HLHS. The proposal leverages the existing infrastructure and extensive expertise at the Children’s Hospital of Philadelphia, Queen’s University, and Kitware Inc. Overall, this study represents a significant advance in the understanding of tricuspid valve disease in HLHS, as well as a flexible, and extensible armamentarium of tools for the future image-based investigation of congenital and adult structural heart disease.