Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease

Int J Cardiol. 2013 Sep 20;168(1):375-81. doi: 10.1016/j.ijcard.2012.09.036. Epub 2012 Oct 5.


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.

Conclusion: 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.

Keywords: Congenital heart disease; Exercise training; Pulmonary hypertension; Rehabilitation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Exercise / physiology*
  • Exercise Therapy / methods*
  • Familial Primary Pulmonary Hypertension
  • Female
  • Follow-Up Studies
  • Heart Defects, Congenital / epidemiology*
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / therapy*
  • Hospitalization / trends
  • Humans
  • Hypertension, Pulmonary / epidemiology*
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy*
  • Male
  • Middle Aged
  • Oxygen Consumption / physiology
  • Prospective Studies
  • Treatment Outcome