title: The combination of exercise and respiratory training improves respiratory muscle function in pulmonary hypertension.
abstract: OBJECTIVE
  Increased dyspnea and reduced exercise capacity in pulmonary arterial hypertension (PAH) can be partly attributed to impaired respiratory muscle function. This prospective study was designed to assess the impact of exercise and respiratory training on respiratory muscle strength and 6-min walking distance (6MWD) in PAH patients.
  
  
  METHODS
  Patients with invasively confirmed PAH underwent 3 weeks of in-hospital exercise and respiratory training, which was continued at home for another 12 weeks. Medication remained constant during the study period. Blinded observers assessed efficacy parameters at baseline (I) and after 3 (II) and 15 weeks (III). Respiratory muscle function was assessed by twitch mouth pressure (TwPmo) during nonvolitional supramaximal magnetic phrenic nerve stimulation.
  
  
  RESULTS
  Seven PAH patients (4 women; mean pulmonary artery pressure 45 ± 11 mmHg, median WHO functional class 3.1 ± 0.4, idiopathic/associated PAH n = 5/2) were included. The training program was feasible and well tolerated by all patients with excellent compliance. TwPmo was I: 0.86 ± 0.37 kPa, II: 1.04 ± 0.29 kPa, and III: 1.27 ± 0.44 kPa, respectively. 6MWD was I: 417 ± 51 m, II: 509 ± 39 m, and III: 498 ± 39 m, respectively. Both TwPmo (+0.41 ± 0.34 kPa, +56 ± 39 %) and 6MWD (+81 ± 30 m, +20 ± 9 %) increased significantly in the period between baseline and the final assessment (pairwise comparison: p = 0.012/<0.001; RM-ANOVA considering I, II, III: p = 0.037/<0.001).
  
  
  CONCLUSIONS
  Exercise and respiratory training as an adjunct to medical therapy may be effective in patients with PAH to improve respiratory muscle strength and exercise capacity. Future, randomized, controlled trials should be carried out to further investigate these findings.
authors: Department of Pneumology, University Hospital Freiburg, Killianstrasse 5, 79106, Freiburg, Germany, hans-joachim.kabitz@uniklinik-freiburg.de.
authors: Hans-Joachim
authors: HJ
authors: Kabitz
authors: Hinrich-Cordt
authors: HC
authors: Bremer
authors: Anja
authors: A
authors: Schwoerer
authors: Florian
authors: F
authors: Sonntag
authors: Stephan
authors: S
authors: Walterspacher
authors: David Johannes
authors: DJ
authors: Walker
authors: Nicola
authors: N
authors: Ehlken
authors: Gerd
authors: G
authors: Staehler
authors: Wolfram
authors: W
authors: Windisch
authors: Ekkehard
authors: E
authors: Grünig
mesh_terms: D000368:Aged; D001945:Breathing Exercises; D003131:Combined Modality Therapy; D005080:Exercise Test; D005081:Exercise Therapy / Q000379:methods*; D017079:Exercise Tolerance; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength; D011446:Prospective Studies; D020127:Recovery of Function; D012132:Respiratory Muscles / Q000503:physiopathology*; D013997:Time Factors; D016896:Treatment Outcome