RT Journal Article SR Electronic T1 Exercise training in pulmonary arterial hypertension associated with connective tissue diseases JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP P4433 VO 40 IS Suppl 56 A1 Ekkehard Gruenig A1 Felicitas Maier A1 Nicola Ehlken A1 Christine Fischer A1 Mona Lichtblau A1 Norbert Blank A1 Christoph Fiehn A1 Frank Stöckl A1 Felix Prange A1 Gerd Staehler A1 Frank Reichenberger A1 Henning Thide A1 Michael Halank A1 Hans-Jürgen Seyfrath A1 Simone Wagner A1 Christian Nagel YR 2012 UL http://erj.ersjournals.com/content/40/Suppl_56/P4433.abstract AB Background: The objective of this prospective study was to assess short-and long-term efficacy of exercise training(ET) as add-on to medical therapy in patients with connective tissue diseases-associated pulmonary arterial hypertension(CTD-APAH).Patients with invasively confirmed CTD-APAH received ET in-hospital for 3 weeks and continued at home for 15 weeks. Efficacy parameters have been evaluated at baseline and after 15 weeks by blinded-observers. Survival rate has been evaluated in a follow-up period of 2.9±1.9 years.Results: Twenty-one consecutive patients were included and assessed at baseline, and after 3 weeks, 12 after 15 weeks. Patients significantly improved the mean distance walked in 6 minutes compared to baseline by 67±52 meters after 3 weeks(p<0.001) and by 71±35 meters after 15 weeks(p=0.003), scores of quality of life(p<0.05), heart rate at rest and maximal workload. Systolic pulmonary artery pressure and diastolic systemic blood pressure improved significantly after 3 weeks of ET. The 1- and 2-year overall-survival rates were 100%, the 3-year survival 73%. In one patient lung transplantation was performed 6 months after ET.Conclusion: ET as add-on to medical therapy is effective in patients with CTD-APAH to improve work capacity, quality of life and prognostic parameters and improves the 1-, 2- and 3-year survival rate. Further randomized controlled studies are needed to confirm these results.