Pulmonary arterial hypertension (PAH) is a relatively poorly understood disease in children and requiresconstant monitoring and chronic treatment to mitigate excess right ventricular afterload effects. Suchmonitoring requires regular and frequently invasive clinical imaging sessions. However, even with invasivetechniques, the extent of clinical information currently obtained is incomplete, involving primarily pulmonaryvascular resistance (PVR) and its component parameters: mean pulmonary artery (PA) pressure and rightsided cardiac output (Qp). Given this paucity of quantitative information currently available to evaluate PAHclinically, opportunities exist to develop and evaluate more comprehensive measures of PAH using acombination of advanced cardiovascular imaging and sophisticated computational modeling. Furthermore,information gained from such endeavors should also assist in the development of novel non-invasivediagnostics, 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 morecomprehensive measure of pulmonary vascular function than PVR alone since impedance includes bothdynamic (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, studiesexploring why impedance is a good reflector of pulmonary vascular hemodynamics and mechanics, andstudies developing novel non-invasive diagnostics that extract the relevant parameters found in invasively-measured impedance, namely PVR and pulmonary vascular stiffness (PVS).
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