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2-4 Despite having similar measures of right ventricular (RV) load,5,6 patients with SSc-PAH have worse survival and poorer response to treatment compared with patients with IPAH.7,8 In investigating this apparent paradox, we and others have shown that RV function differs between SSc-PAH and IPAH and strongly predicts survival, indicating the importance of the RV in maintaining adequate cardiac output (CO) in the face of increased RV load.9-11 Recent work from our group has investigated direct measurements of RV contractility using pressure volume loops in the RV to compare IPAH with SSc in patients with and without PH.12 In this study, we found the ratio of end-systolic elastance to Anti-diabetic Compound Library effective arterial elastance, a measure of RV-pulmonary arterial coupling, to be significantly decreased in patients with SSc and PAH as compared with patients with IPAH and patients with selleck chemical SSc without PAH. These data strongly suggest differences in RV intrinsic contractility between patients with IPAH and patients with SSc-PAH that become apparent in the setting of an increased afterload. Because SSc is characterized by endothelial dysfunction and microvascular disease, we hypothesized on the basis of microvascular-myocyte imbalance that patients with SSc-PAH, compared with patients with IPAH, may display maladaptive RV hypertrophy as assessed by RV mass in response to increased pulmonary vascular load. Although echocardiography is a useful screening modality in pulmonary hypertension, cardiac magnetic resonance RecBCD imaging (cMRI) has been an increasingly valuable tool in assessing cardiac morphology and function and in determining prognosis in PAH.13,14 Echocardiography is an operator-dependent and two-dimensional method that relies on geometric assumptions for volume and mass calculations. In addition, results are highly dependent on patient and probe position, which is variable between patients.15,16 cMRI provides a true three-dimensional image and is considered the gold standard, with no operator dependence or dependence on optimal imaging windows. Therefore, we used cMRI to test our hypothesis and assess the relationship between RV mass and RV load in two distinct PAH disease states: SSc-PAH and IPAH. Methods This prospective study was Health Insurance Portability and Accountability Act compliant and was approved by the institutional review board at Johns Hopkins Hospital. We performed a cross-sectional analysis of prospectively gathered data from a cohort of 53 patients with SSc-PAH and IPAH. Eighteen patients received a diagnosis of IPAH. Of these 18 patients, 1 patient with known IPAH had a PCWP of 16 at the time of right heart catheterization (RHC). This was attributed to septal interdependence in the setting of significant volume overload. Another patient had a confirmed diagnosis of IPAH but had achieved normalized mPAP (