Non-invasive Assessment of Pulmonary Vascular Function by MRI in Children with Pulmonary Arterial Hypertension Correlate with Invasive Measurements

通过 MRI 对肺动脉高压儿童的肺血管功能进行无创评估与有创测量相关

• 批准号: 10872008
• 负责人: Uyen T Truong
• 金额: $ 16.94万
• 依托单位: CHILDREN'S RESEARCH INSTITUTE
• 依托单位国家: 美国
• 财政年份: 2017
• 资助国家: 美国
• 起止时间: 2017-08-14 至 2024-08-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/10872008
• 关键词: Non; invasive; Assessment; Pulmonary; Vascular

Project Summary Pulmonary arterial hypertension (PAH) is an incurable, progressive disease that affects both adults and children. While many pathophysiologic similarities exist between adult and pediatric pulmonary hypertension, the number of life years lost in children is much greater given limited long-term survival. Pediatric PAH is distinct from adult disease in the fact that the initial insult occurs during a critical period of maturation and lung development. Hemodynamic abnormalities in the developing lung may impact treatment response and the natural history of the disease in ways that have not been shown; and can remain a strong contributing factor in disease progression. Despite significant unique factors in pediatric PAH, research of this group is lacking, forcing the use of adult-based guidelines and therapies. As a result, management of pediatric PAH includes using invasive right heart catheterization (RHC) for serial diagnostic/prognostic evaluation. Serial RHC is costly, exposes children to repeated anesthesia and excessive radiation, and importantly, children undergoing catheterization for PAH may have catastrophic events including cardiorespiratory arrest and death. Despite these challenges inherent to an invasive procedure, there is no alternative for evaluating therapeutic direction for pediatric patients. This is a significant factor for the lack of research and targeted therapies for this vulnerable population. Cardiac MRI is the single imaging modality that allows assessment of both the ventricle and the vasculature involved in PAH pathophysiology, without radiation exposure or anesthesia in older children. In adult studies, MRI is emerging as a technique for evaluating vascular function, including, recently, vorticity and helicity by 4-dimensional (4D) velocity- encoded MRI. MRI also has the ability to evaluate the pulmonary vascular structure with high spatial resolution and may demonstrate developmental vascular changes in PAH. Our overall hypothesis is that MRI will reflect pulmonary hemodynamic status in patients with PAH similar to conventional RHC. Our long-range goal is to show that non-invasive imaging modality can diagnose and serially monitor PAH children and possibly minimize the need for RHC. Furthermore, novel vascular imaging by 4D MRI, along with conventional MRI, in the proximal vasculature will quantify significant alterations in maturing vascular function in children with PAH. MRI may be a powerful in identifying targeted therapy unique for children.

项目摘要 肺动脉高压(PAH)是一种无法治愈的进行性疾病，它影响 无论是成人还是儿童。虽然成体和成体之间存在许多病理生理上的相似之处 儿童肺动脉高压，儿童寿命损失年数大得多 考虑到有限的长期生存能力。儿童PAH与成人疾病的不同之处在于 最初的侮辱发生在成熟和肺发育的关键时期。血流动力学 发育中的肺的异常可能影响治疗反应和自然病程。 这种疾病的方式还没有表现出来，而且可能仍然是 疾病的发展。尽管儿童PAH存在显著的独特因素，但这一组的研究 缺乏，迫使使用以成人为基础的指南和疗法。因此，管理层 儿童PAH包括使用有创右心插管(RHC)进行系列治疗 诊断/预后评估。连续RHC成本高昂，使儿童暴露在重复的环境中 麻醉和过度辐射，重要的是，正在接受导尿术的儿童 PAH可能会发生灾难性事件，包括心跳呼吸骤停和死亡。尽管 这些是侵入性手术固有的挑战，没有其他方法可以评估 儿科患者的治疗指导。这是缺乏研究的一个重要因素。 以及针对这一弱势群体的针对性治疗。 心脏mri是一种可以评估两个脑室的单一成像方式。 和参与PAH病理生理学的血管系统，没有辐射暴露或 年龄较大的儿童的麻醉。在成人研究中，核磁共振成像正在成为一种评估 血管功能，包括最近4维(4D)速度的涡度和螺旋度- 编码的核磁共振成像。MRI还具有评价高密度肺血管结构的能力。 空间分辨率，并可显示PAH的发育血管改变。我们的整体 假设MRI将反映类似于PAH患者的肺血流动力学状态 到传统的RHC。我们的长期目标是证明非侵入性成像方式可以 诊断和连续监测PAH儿童，并尽可能减少对RHC的需求。 此外，新的4D MRI血管成像，以及传统的MRI，在近端 血管系统将量化儿童成熟血管功能的显著变化 啊哈。MRI可能在确定儿童独特的靶向治疗方面具有强大的作用。