TY - JOUR T1 - Cardiopulmonary Exercise Testing with Right-heart Catheterization in Patients with Systemic Sclerosis JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.091424 SP - jrheum.091424 AU - Allan J. Walkey AU - Michael Ieong AU - Mir Alikhan AU - Harrison W. Farber Y1 - 2010/06/15 UR - http://www.jrheum.org/content/early/2010/06/13/jrheum.091424.abstract N2 - Objective To examine the role of cardiopulmonary exercise testing with right-heart catheterization (CPET/RHC) in patients with systemic sclerosis (SSc) with potentially multifactorial exertional limitation. Methods This was a single-center retrospective cohort study of patients with SSc referred for CPET/RHC. Results A total of 19 patients with SSc [subtypes: 10 limited, 5 diffuse, 1 systemic lupus erythematosus (SLE)/SSc overlap, and 3 with no subtype specified in the medical record] underwent CPET/RHC testing from February 2003 to February 2008. Of these patients, the primary limitations to exercise were found to be ventilatory (n = 6), deconditioning/cardiovascular (n = 6), pulmonary vascular (PVL; n = 3), and exercise-induced left ventricular diastolic dysfunction (exercise-LVDD; n = 4). No prior physical examination, pulmonary function test, imaging, or echocardiographic data reliably predicted the etiology of exercise limitation determined by CPET/RHC. Vital capacity and ventilatory equivalent for CO2 did not differ during CPET testing between PVL and exercise–LVDD, limiting the utility of CPET alone for discriminating these etiologies of dyspnea. Exercise alveolararterial oxygen gradient was elevated in subjects shown to have PVL [median 48 mm Hg (interquartile range 45.3, 62.0)] compared to those with exercise-LVDD [26.0 (IQR 10.6, 36.0)] and deconditioning [13.9 (IQR 4.0, 16.4); p = 0.02]. Major therapeutic changes occurred in 11/19 (58%) subjects after CPET/RHC testing. Conclusion CPET/RHC testing in subjects with SSc and potentially multifactorial dyspnea adds potentially useful diagnostic information unavailable from noninvasive testing. ER -