Computer Modeling of the Tricuspid Valve in Hypoplastic Left Heart Syndrome

项目总结/摘要 左心发育不良综合征（HLHS）的特征是左心发育不完全， 在美国每年有超过1,000个活产婴儿。分期手术治疗允许患有HLHS的儿童 生存和繁荣，但右心室（RV）仍然是唯一的功能心室和三尖瓣 (TV)是他们唯一的功能性房室瓣因此，三尖瓣返流与心脏 失败和死亡。30%的HLHS患者需要手术干预治疗三尖瓣返流。 尽管取得了进展，但对TV结构与三尖瓣返流之间关系的理解 在HLHS是有限的，手术修复的结果是次优的。3D超声心动图和定量3D 基于超声心动图的分析显著地改善了成人二尖瓣外科手术计划和修复。 然而，尽管初步工作与基本工具建议之间的关系的3D结构的电视 和患者生存率，以及对指导TV修复的精确结构信息的未满足需求， 商业或现成的方法，足以适应建模和量化的独特和高度 HLHS中的可变TV解剖结构。因此，3D超声心动图图像中的信息仍然是潜在的，限制了 了解TV结构与三尖瓣返流之间的关系，以及 通过3D结构分析进行患者特定修复。这一建议建立在乔利博士的经验， 先天性心脏病房室瓣的建模和灵活开源的开发 建模工具。具体目标是：1）研究从3D中量化TV结构的方法 使用基于度量的工具以及新颖的形状参数化和统计形状的超声心动图图像 分析;和2）使用这些工具来确定100例HLHS患者中TV功能障碍的TV结构相关性， 已经完成了分期手术修复开发的方法将被纳入开源3D Slicer成像处理平台将促进先天性和成人结构性心脏病的未来研究， 特别是那些不可能使用现有模式的国家。这些研究是走向个性化的第一步 通过手术对电视修复体进行3D结构分析，并详细了解结构特征 电视功能障碍的案例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 Inc.总的来说，这项研究代表了 在HLHS中对三尖瓣疾病的理解方面取得了重大进展， 为未来先天性和成人结构性心脏病的影像学研究提供了一整套工具。

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.