title: Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease.
abstract: BACKGROUND
  The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD-APAH).
  
  
  METHODS
  Patients with invasively confirmed CHD-APAH received in-hospital exercise training for 3 weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15 weeks. Medical treatment remained unchanged. Worsening events and survival rate were assessed in a follow-up period of 21 ± 14 months.
  
  
  RESULTS
  Twenty consecutive CHD-APAH patients (16 female, 4 male, mean pulmonary arterial pressure 60 ± 23 mm Hg) were included. Patients significantly improved the mean distance walked in 6 min compared to baseline by 63 ± 47 m after 3 weeks (p<0.001) and by 67 ± 59 m after 15 weeks (p=0.001). Quality of life-score (p=0.05), peak oxygen consumption (p=0.002) and maximal workload (p=0.003) improved significantly by exercise training after 15 weeks. The 1- and 2-year survival rates were 100%, the transplantation-free survival rate was 100% after 1 year and 93% after 2 years.
  
  
  CONCLUSIONS
  Exercise training as add-on to medical therapy may be effective in patients with CHD-APAH and improved work capacity, quality of life and further prognostic relevant parameters. It was associated with an excellent long-term survival. Further randomized controlled studies are needed to confirm these results.
authors.affiliation: Centre for Pulmonary Hypertension at Thoraxclinic Heidelberg, University of Heidelberg, Germany.
authors.forename: Tabea
authors.initials: T
authors.lastname: Becker-Grünig
authors.forename: Hans
authors.initials: H
authors.lastname: Klose
authors.forename: Nicola
authors.initials: N
authors.lastname: Ehlken
authors.forename: Mona
authors.initials: M
authors.lastname: Lichtblau
authors.forename: Christian
authors.initials: C
authors.lastname: Nagel
authors.forename: Christine
authors.initials: C
authors.lastname: Fischer
authors.forename: Matthias
authors.initials: M
authors.lastname: Gorenflo
authors.forename: Henning
authors.initials: H
authors.lastname: Tiede
authors.forename: Dietmar
authors.initials: D
authors.lastname: Schranz
authors.forename: Alfred
authors.initials: A
authors.lastname: Hager
authors.forename: Harald
authors.initials: H
authors.lastname: Kaemmerer
authors.forename: Oliver
authors.initials: O
authors.lastname: Miera
authors.forename: Silvia
authors.initials: S
authors.lastname: Ulrich
authors.forename: Rudolf
authors.initials: R
authors.lastname: Speich
authors.forename: Sören
authors.initials: S
authors.lastname: Uiker
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grünig
mesh_terms: D000328:Adult; D015444:Exercise / Q000502:physiology*; D005081:Exercise Therapy / Q000379:methods*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy*; D006760:Hospitalization / Q000639:trends; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D010101:Oxygen Consumption / Q000502:physiology; D011446:Prospective Studies; D016896:Treatment Outcome