Background: Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is a rare disease characterized by a very dismal response to therapy and poor survival. We assessed the effects of up-front combination PAH therapy in patients with SSc-PAH.

Rationale: Pulmonary arterial hypertension is a progressive disease with high morbidity and mortality despite advances in medical therapy. The relationship between patient-related outcomes, such as healthrelated quality of life (HRQOL), and survival is not well described.Objective: To assess the relationship between HRQOL and outcomes in patients with pulmonary arterial hypertension.Methods: Consecutive patients with right heart catheterization-proven pulmonary arterial hypertension who completed the Medical Outcomes Survey Short Form-36 survey (SF-36) were included. Demographic, clinical, physiological, and hemodynamic data were collected at baseline. Survival was assessed from the time of diagnosis of pulmonary arterial hypertension. Cox proportional hazard models were constructed to assess the relationship between HRQOL and transplant-free survival.Measurements and Main Results: Eighty-seven patients with pulmonary arterial hypertension were enrolled and followed prospectively for a median of 3.8 years. At baseline, HRQOL was significantly worse than U.S. normal values for six of eight domains of the SF-36. Several domains demonstrated moderate correlation (r value > 0.40) with 6-minute-walk distance and World Health Organization functional class; there were no significant associations with hemodynamics. In univariable Cox proportional hazard models, six of eight domains and both summary scores were significantly associated with survival. In multivariable models, adjusted for age, disease type, and cardiac function, these relationships largely persisted. Conclusions:In this cohort of patients with pulmonary arterial hypertension, HRQOL, as assessed by the SF-36, was strongly associated with transplant-free survival. These relationships persisted when controlling for potential confounders such as disease type and disease severity. These findings suggest that HRQOL may be an important predictor of outcomes in pulmonary arterial hypertension and therefore a target for future therapeutic interventions.

Using distributional and anchor-based methods, we estimate the minimum important difference for Borg dyspnea scale in pulmonary arterial hypertension is approximately 0.9 units. Using distributional methods only, we estimate the minimum important difference for the Borg fatigue scale is around 1 unit. Further studies are needed to determine the clinical utility of these scores in patients with pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that leads to right ventricular (RV) failure and premature death. Because RV failure is the leading cause of mortality in PAH, the prognostic value of cardiac magnetic resonance (CMR) imaging, a powerful tool for assessing the RV, has been the subject of much recent investigation. Several studies have demonstrated that CMR measures of ventricular volumes and right ventricular ejection fraction (RVEF) predict mortality in PAH [1-5]. Fewer studies, however, have examined the prognostic value of ventricular mass metrics in PAH. RAJARAM et al. [5] demonstrated significant associations between RV mass and ventricular mass index (VMI), the ratio of RV mass to left ventricular (LV) mass, and mortality in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). LV mass has been shown to predict time to clinical worsening in idiopathic pulmonary arterial hypertension (IPAH) [6]. Small studies have shown that VMI correlates more strongly with invasive haemodynamics