TY - JOUR T1 - Presence of right ventricular dysfunction predicts dyspnea and quality of life improvements with sildenafil in IPF JF - European Respiratory Journal JO - Eur Respir J VL - 38 IS - Suppl 55 SP - 176 AU - MeiLan Han AU - David Bach AU - Peter Hagan AU - Shelley Schmidt AU - Kevin Flaherty AU - Galen Toews AU - Rex Edwards AU - Eric Yow AU - Kevin Anstrom AU - Fernando Martinez Y1 - 2011/09/01 UR - http://erj.ersjournals.com/content/38/Suppl_55/176.abstract N2 - Background: IPF is a progressive lung disease with impaired gas exchange. The STEP-IPF trial was conducted to determine if sildenafil would increase 6-minute walk distance by ≥ 20%. The primary endpoint was not reached, but a substudy tested whether baseline evidence of pulmonary hypertension would predict treatment response.Methods: Echocardiograms were available for review in 119/180 subjects and read by two cardiologists. Right ventricular hypertrophy (RVH), RV systolic dysfunction (RVSD) and RV systolic pressure (RVSP) were assessed. A general linear model determined the relationship between cardiac abnormality, sildenafil treatment and improvement in quality of life (QOL) as measured by EuroQol EQ-5D, EuroQol thermometer and SGRQ at 12 weeks.Results: Mean FVC% predicted was 57%; DLCO% predicted 26%. Prevalence of RVH was 13%; RV systolic dysfunction 19%. Mean RVSP was 42.5, measurable in 71/119 subjects. Mean EuroQoL thermometer score was 69.1, EuroQol EQ-5D 0.73, and SGRQ 53.1. Significant interactions between RV systolic dysfunction and sildenafil treatement were seen for SGRQ (p=0.048), SGRQ symptom score (p=0.002) and EuroQol Thermometer (p=0.05). Sildenafil treated subjects with RVSD improved by 13.4 SGRQ points, 28.0 SGRQ symptom score points, and 17.9 EuroQol Thermometer points vs placebo. Those with RVSD treated with placebo increased their SGRQ by 2.9 points, SGRQ symptom score by 3.8 points, and dropped their EuroQoL Thermometer score by 1.4 points vs placebo.Conclusions: In IPF patients with RV systolic dysfunction, sildenafil treatment is associated with improvements in QOL as measured by SGRQ total and symptom scores and EuroQol Thermometer score. ER -