Advanced Imaging and Diagnostics for Pediatric Pulmonary Hypertension

肺动脉高压（PAH）是一种儿童疾病， 持续监测和长期治疗以减轻过度右心室后负荷效应。等 监测需要定期和频繁的侵入性临床成像会话。然而，即使有侵入性 技术，目前获得的临床信息的程度是不完整的，主要涉及肺 血管阻力（PVR）及其组成参数：平均肺动脉（PA）压和右 侧心输出量（Qp）。鉴于目前可用于评价PAH的定量信息缺乏 在临床上，有机会开发和评价更全面的PAH指标， 结合先进的心血管成像和复杂的计算建模。此外，委员会认为， 从这些努力中获得的信息也应该有助于开发新的非侵入性药物。 诊断，通过允许更容易地获取肺血管特征，连续监测， 和床旁反应性评价，应扩大临床医生的能力，以确定这种复杂的疾病。 这些研究的总体假设是肺血管输入阻抗提供了更多的 肺血管功能的综合测量优于单独的PVR，因为阻抗包括 血管回路的动态（刚度或顺应性）和稳态（阻力）组件。的 因此，本项目的目标分为建立阻抗临床应用的研究、 探讨为什么阻抗是肺血管血流动力学和力学的良好反映，以及 研究开发新的非侵入性诊断，提取相关参数发现在侵入性- 测量阻抗，即PVR和肺血管硬度（PVS）。

Pulmonary arterial hypertension (PAH) is a relatively poorly understood disease in children and requires constant monitoring and chronic treatment to mitigate excess right ventricular afterload effects. Such monitoring requires regular and frequently invasive clinical imaging sessions. However, even with invasive techniques, the extent of clinical information currently obtained is incomplete, involving primarily pulmonary vascular resistance (PVR) and its component parameters: mean pulmonary artery (PA) pressure and right sided cardiac output (Qp). Given this paucity of quantitative information currently available to evaluate PAH clinically, opportunities exist to develop and evaluate more comprehensive measures of PAH using a combination of advanced cardiovascular imaging and sophisticated computational modeling. Furthermore, information gained from such endeavors should also assist in the development of novel non-invasive diagnostics, which by allowing easier acquisition of pulmonary vascular characteristics, serial monitoring, and bedside evaluation of reactivity, should widen the clinician's ability to characterize this complex disease. The overall hypothesis for these studies is that pulmonary vascular input impedance provides a more comprehensive measure of pulmonary vascular function than PVR alone since impedance includes both dynamic (stiffness or compliance) and steady-state (resistance) components of the vascular circuit. The aims of this project are therefore divided into studies establishing the use of impedance clinically, studies exploring why impedance is a good reflector of pulmonary vascular hemodynamics and mechanics, and studies developing novel non-invasive diagnostics that extract the relevant parameters found in invasively- measured impedance, namely PVR and pulmonary vascular stiffness (PVS).