Hopkins Clinical Center for Pulmonary Vascular Disease Phenomics Program

霍普金斯肺血管疾病临床中心表型组学项目

• 批准号: 8794533
• 负责人: Paul M. Hassoun
• 金额: $ 12.11万
• 依托单位: JOHNS HOPKINS UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 2014
• 资助国家: 美国
• 起止时间: 2014-12-01 至 2019-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8794533
• 关键词: Biological Markers; Biopsy; Blood Vessels; Cardiac; Cardiac Catheterization Procedures; Cardiology; Cardiovascular Diseases; Caring; Cessation of life; Characteristics; Chronic; Classification; Clinical; Clinical Trials; Collaborations; Complex; Comprehensive Health Care; Connective Tissue Diseases; Diagnostic; Disease; Distal; Echocardiography; Enrollment; Ensure; Epidemiology; Equilibrium; Exercise; Failure; Functional disorder; Funding; Genes; Genetic; Genetic Determinism; Genetic Markers; Genomics; Goals; HIV; Heart Diseases; Human; Hypoxia; Image; Industry; Institutional Review Boards; Interstitial Lung Diseases; Knowledge; Left; Link; Lung; Lung diseases; Magnetic Resonance Imaging; Measurement; Measures; Molecular; Morbidity - disease rate; Muscle Cells; Operative Surgical Procedures; Outcome; Pathway interactions; Patients; Performance; Pharmaceutical Preparations; Phenotype; Population; Portal Hypertension; Positioning Attribute; Pre-Clinical Model; Productivity; Public Health; Pulmonary Hypertension; Pulmonology; Radiology Specialty; Recording of previous events; Registries; Research; Research Infrastructure; Research Personnel; Research Project Grants; Rest; Rheumatology; Right Ventricular Dysfunction; Right Ventricular Function; Risk Assessment; Role; Sarcomeres; Schistosomiasis; Scleroderma; Serum; Serum Markers; Services; Severities; Sickle Cell Anemia; Specialist; Specialized Center; Structure; Subgroup; Target Populations; Technology; Testing; Therapeutic; United States National Institutes of Health; Update; Vascular Diseases; Ventricular; Ventricular Remodeling; Work; cohort; congenital heart disorder; disease phenotype; disorder risk; endophenotype; functional genomics; hemodynamics; hypertension treatment; imaging system; molecular imaging; mortality; multidisciplinary; novel; phenomics; pressure; primary outcome; programs; pulmonary arterial hypertension; response; success; tool

DESCRIPTION (provided by applicant): Project Summary: Hopkins Clinical Center for Pulmonary Vascular Disease Phenomics Program The Hopkins Pulmonary Hypertension (PH) program is composed of a highly dedicated, multi-disciplinary group of experts from various institutional divisions and departments, who have a long history of close interaction and rich productivity within the framework of current and previous NIH-supported research projects. Our Program primarily provides comprehensive management of patients from all five groups of the PH World Classification, and also has a long history of participation in industry-sponsored as well as NIH-sponsored multicenter clinical trials for PH treatment. Since right ventricular (RV) function is the main determinant of death in PH, we propose thorough phenotyping of PH patients with a primary focus on the complex relationship between the RV and the pulmonary vasculature (PV) and RV-PV uncoupling, with the goal to further examine their crucial impact on morbidity and mortality in PH. Leveraging our extensive clinical knowledge in PH and our expertise in molecular and diagnostic pulmonary medicine and cardiology, we propose to use 1) echocardiography and cardiac magnetic resonance imaging (cMRI) to identify non-invasive measures of RV performance and their capacity to predict outcomes; 2) invasive hemodynamic assessment of RV/PV interaction for its impact on deep phenotyping and survival; and 3) molecular (genomics, genetics, and serum biomarkers) for endophenotyping. The search for clinical, hemodynamic, imaging, serum and genetic biomarkers proposed in this application can be readily applied to all clinical centers in the context of the PVDOMICs as relevant variables for disease risk assessment, dynamic phenotyping (baseline and in response to treatment), endophenotyping, and as intermediate or primary outcomes in clinical trials. In summary, while the choice of parameters, studies and target populations will be left to the discretion of the DCC, our PH clinical center will offer the unique expertise of a multidisciplinary and highly integrated team of experts in this new endeavor to redefine PH phenotypes from all five groups of PH, thus ensuring success of the PVDOMICs efforts.

描述（由申请人提供）：项目摘要：霍普金斯临床中心肺血管疾病表型组学计划霍普金斯肺动脉高压（PH）计划是由一个高度敬业的，多学科的专家组，来自不同的机构部门和部门，谁有密切的互动和丰富的生产力在当前和以前的NIH支持的研究项目的框架内的悠久历史。我们的项目主要为PH世界分类的所有五组患者提供综合管理，并且长期参与行业赞助以及NIH赞助的PH治疗多中心临床试验。由于右心室（RV）功能是PH患者死亡的主要决定因素，我们建议对PH患者进行彻底的表型分析，主要关注RV与肺血管（PV）之间的复杂关系以及RV-PV解偶联，目的是进一步研究它们对PH发病率和死亡率的重要影响。利用我们在PH方面的广泛临床知识以及我们在分子和诊断肺疾病方面的专业知识，在医学和心脏病学方面，我们建议使用1）超声心动图和心脏磁共振成像（cMRI）来确定RV性能的无创测量及其预测结局的能力; 2）RV/PV相互作用的有创血流动力学评估，以评估其对深层表型和生存率的影响; 3）分子（基因组学、遗传学和血清生物标志物）用于内表型。本申请中提出的临床、血流动力学、成像、血清和遗传生物标志物的检索可作为PVDOMIC的相关变量容易地应用于所有临床中心， 疾病风险评估、动态表型分型（基线和对治疗的反应）、内表型分型以及作为临床试验中的中间或主要结局。总之，虽然参数、研究和目标人群的选择将由DCC自行决定， 我们的PH临床中心将提供多学科和高度集成的独特专业知识， 在这一新的奋进中，我们的专家团队重新定义了所有五组PH的PH表型，从而确保了PVDOMIC工作的成功。