Right ventricular failure is the most important predictor of mortality for patients with severe pulmonaryarterial hypertension (PAH). Based on the preliminary data of Projects 1, 2, and 3, patients withscleroderma-associated PAH-(SSc) have a significantly higher rate of right ventricular failure for the samelevels of pulmonary artery pressures as patients with IPAH. Since the overall goal of our SCCOR is tounderstand the molecular mechanisms of right ventricular failure, increase the accuracy of phenotyping ofpatients, with severe PAH and to test novel therapies, an imaging core is central for several of the goals ofthis proposal. The primary functions of the Imaging Core are to provide comprehensive analysis andinterpretation of multiple imaging biomarkers, and to develop and test novel imaging biomarkers. Theseimaging parameters will serve to (a) characterize extent and progression of right ventricular diseaseassociated with PAH (with Projects 1,2, 3); (b) phenotype cardiovascular function to facilitate interpretationof serologic and proteomic markers in PAH (with Cores C, D and F and Projects 1, 2 and 3), and (c)develop/ test/ evaluate novel imaging biomarkers in terms of their sensitivity and their predictive potentialfor disease characterization (with Projects 1,2 and 3). Therefore, the Imaging Core will be involved activelyin phenotype characterization, and biomarker discovery and validation.The right ventricle is negatively impacted by pulmonary disease, and deterioration in global RV structureand function have been measured previously using echocardiography, magnetic resonance imaging (MRI)and multi-detector CT (MDCT). The overall strategy of the imaging core will be to (1) provide reliablemeasurements of echocardiographic derived cardiac function, (2) provide reliable measurements of MRIand MDCT derived cardiac and pulmonary vascular function, and (3) implement novel imaging methodsdesigned to measure regional myocardial dysfunction and myocardial fibrosis/ inflammation. Overall, weaim to comprehensively characterize RV status by assessing RV global function, RV regional function,pulmonary distensibility and RV fibrosis using noninvasive imaging methods.
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