Ambrisentan and Tadalafil Upfront Combination Therapy in Scleroderma-Associated PAH (original) (raw)

American journal of respiratory and critical care medicine, 2015

Abstract

Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is a rare disease characterized by very dismal response to therapy and poor survival. We assessed the effects of upfront, combined PAH therapy in SSc-PAH. In this prospective multicenter open label trial, 24 treatment naïve SSc-PAH patients received ambrisentan (10 mg) and tadalafil (40 mg) daily for 36 weeks. Functional, hemodynamic and imaging (cardiac magnetic resonance and echocardiography) assessment at baseline and 36 weeks included changes in right ventricular mass and pulmonary vascular resistance (RV mass and PVR) as co-primary end-points, and stroke volume/pulmonary pulse pressure (SV/PP), tricuspid annular plane systolic excursion (TAPSE), six-minute walk distance (6MWD) and N-terminal pro-brain natriuretic peptide (NT-proBNP), all secondary end-points. Treatment resulted in significant reductions in median RV mass (28.0 IQR 20.6-32.9 versus 32.5 IQR 23.2-41.4 gm; P<0.05) and PVR (3.1 IQR 2.0-5.7 versus 6...

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