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Exercise training in pulmonary arterial hypertension associated with connective tissue diseases

Ekkehard Grünig1*, Felicitas Maier1, Nicola Ehlken1, Christine Fischer2, Mona Lichtblau1, Norbert Blank3, Christoph Fiehn4, Frank Stöckl5, Felix Prange1, Gerd Staehler6, Frank Reichenberger7, Henning Tiede7, Michael Halank8, Hans-Jürgen Seyfarth9, Simone Wagner10 and Christian Nagel1

Author Affiliations

1 Centre for Pulmonary Hypertension at Thoraxclinic Heidelberg, Thoraxclinic at the University Hospital Heidelberg, Amalienstraße 5, Heidelberg, 69126, Germany

2 Department of Human Genetics, University of Heidelberg, Im Neuenheimer Feld 366, Heidelberg, 69120, Germany

3 Department of Rheumatology, University of Heidelberg, Im Neuenheimer Feld 410, Heidelberg, 69120, Gemany

4 Department of Rheumatology, ACURA Centre for Rheumatic Diseases, Rotenbachtalstraße 5, Baden-Baden, 76530, Germany

5 Department of Internal Medicine, Medical Clinic III, Grafenstraße 9, Darmstadt, 64283, Germany

6 Centre for Pulmonary Hypertension, Clinic Löwenstein, Geißhölzle 62, Löwenstein, 74245, Germany

7 Department of Pneumology, University Hospital Gießen, Klinikstraße 33, Giessen, 35392, Germany

8 Department of Pneumology, University Hospital Dresden, Fetscherstraße 74, Dresden, 01307, Germany

9 Department of Pneumology, University Hospital Leipzig, Liebigstraße 20, Leipzig, 04103, Germany

10 Department of Neurology, University Hospital Heidelberg, Im Neuenheimer Feld 400, Heidelberg, 69126, Germany

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Arthritis Research & Therapy 2012, 14:R148  doi:10.1186/ar3883

Published: 18 June 2012



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 disease-associated pulmonary arterial hypertension (CTD-APAH).


Patients with invasively confirmed CTD-APAH received ET in-hospital for 3 weeks and continued at home for 12 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.


Twenty-one consecutive patients were included and assessed at baseline, and after 3 weeks, 14 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, peak oxygen consumption, oxygen saturation 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.


ET as add-on to medical therapy is highly effective in patients with CTD-APAH to improve work capacity, quality of life and further prognostic relevant parameters and possibly improves the 1-, 2- and 3-year survival rate. Further randomized controlled studies are needed to confirm these results.

Trial registration NCT00491309.