title: Effect of exercise and respiratory training on clinical progression and survival in patients with severe chronic pulmonary hypertension.
abstract: BACKGROUND
  Even though specific agents for the treatment of patients with pulmonary hypertension (PH) are available, in PH patients, physical capacity and quality of life (QoL) are often restricted and survival is reduced.
  
  
  OBJECTIVE
  This study prospectively investigated the long-term effects of respiratory and exercise training in patients with severe chronic PH regarding safety, time to clinical worsening and survival.
  
  
  METHODS
  Fifty-eight consecutive patients with severe PH on stable disease-targeted medication received exercise and respiratory training in hospital for 3 weeks and continued at home. They were prospectively followed for 24 ± 12 months. Primary endpoints were time to clinical worsening and survival. Adverse events and changes in the 6-min walking test, QoL, WHO functional class and gas exchange were secondary endpoints and were evaluated at baseline and at weeks 3 and 15.
  
  
  RESULTS
  All patients tolerated the exercise training well without severe adverse events. In week 15, 6-min walking test results were significantly improved compared to baseline (by 84 ± 49 m, p < 0.001), as well as QoL scores, WHO functional class (from 2.9 ± 0.5 to 2.6 ± 0.6, p < 0.01), peak oxygen consumption (from 12.5 ± 3.0 to 14.6 ± 3.9 ml/min/kg, p < 0.001), heart rate at rest (from 75 ± 12 to 61 ± 18 beats/min, p < 0.001) and maximal workload (from 65 ± 21 to 80 ± 25 W, p < 0.001). Survival at 1 and 2 years was 100 and 95%, respectively. Fifteen events occurred during the follow-up.
  
  
  CONCLUSIONS
  This study indicates that exercise and respiratory training as add-on to medical treatment may improve exercise capacity and QoL, and that they have a good long-term safety in the described setting.
authors.affiliation: Center for Pulmonary Hypertension, Thoracic Clinic, University of Heidelberg, Heidelberg, Germany. ekkehard.gruenig@thoraxklinik-heidelberg.de
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grünig
authors.forename: Nicola
authors.initials: N
authors.lastname: Ehlken
authors.forename: Ardeschir
authors.initials: A
authors.lastname: Ghofrani
authors.forename: Gerd
authors.initials: G
authors.lastname: Staehler
authors.forename: F Joachim
authors.initials: FJ
authors.lastname: Meyer
authors.forename: Jana
authors.initials: J
authors.lastname: Juenger
authors.forename: Christian F
authors.initials: CF
authors.lastname: Opitz
authors.forename: Hans
authors.initials: H
authors.lastname: Klose
authors.forename: Heinrike
authors.initials: H
authors.lastname: Wilkens
authors.forename: Stephan
authors.initials: S
authors.lastname: Rosenkranz
authors.forename: Horst
authors.initials: H
authors.lastname: Olschewski
authors.forename: Michael
authors.initials: M
authors.lastname: Halank
mesh_terms: D000328:Adult; D002908:Chronic Disease; D015897:Comorbidity; D018450:Disease Progression; D005081:Exercise Therapy; D005260:Female; D006333:Heart Failure / Q000453:epidemiology; D006339:Heart Rate; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000401:mortality* / Q000534:rehabilitation / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D010100:Oxygen / Q000097:blood; D011446:Prospective Studies; D011788:Quality of Life; D012138:Respiratory Therapy; D016896:Treatment Outcome
chemical_list: D010100:Oxygen