TY - JOUR T1 - Exercise training in pulmonary arterial hypertension associated with connective tissue diseases JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - P4433 AU - Ekkehard Gruenig AU - Felicitas Maier AU - Nicola Ehlken AU - Christine Fischer AU - Mona Lichtblau AU - Norbert Blank AU - Christoph Fiehn AU - Frank Stöckl AU - Felix Prange AU - Gerd Staehler AU - Frank Reichenberger AU - Henning Thide AU - Michael Halank AU - Hans-Jürgen Seyfrath AU - Simone Wagner AU - Christian Nagel Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/P4433.abstract N2 - 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. ER -