Pulmonary arterial hypertension (PAH) is characterized by right ventricular failure, leading to exertional dyspnea, skeletal muscle weakness, and poor quality of life (QOL). Apart from treatment with PAH-specific drugs, guidelines recommend pulmonary rehabilitation (PR). Clinical PR programs have shown improvement in functional capacity and QOL. However, little is known about the effectiveness of an outpatient PR program. The aim of our study was to assess effectiveness of a multidisciplinary outpatient PR program.
Patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH), who were in a stable condition on optimized drug therapy, followed a 10-wk outpatient program in a rehabilitation center. The PR program was designed to improve exercise capacity and health status by means of low load cycling, walking, and muscle training twice a week combined with psychological counseling. QOL was measured by the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire.
Twenty-one patients (13 women) with PAH (n = 16) or CTEPH (n = 5) completed the study. All patients were in New York Heart Association (NYHA) functional class III, and their mean age was 45 ± 16 yr. After PR, the mean cycling endurance time increased by 4.4 min (P < .001), 6-min walk distance by 12.2 m (P < .05), and maximum inspiratory pressure by 5.8 cm H2O (P = .01). Skeletal muscle function increased significantly. The CAMPHOR questionnaire demonstrated significant decrease in symptoms and improvement in QOL. Soluble biomarkers did not show any change before and after PR.
Outpatient PR could be an effective instrument to improve exercise capacity and health status in patients with PAH or CTEPH.
Pulmonary arterial hypertension (PAH) is an incurable condition of the pulmonary vasculature, leading to right ventricular failure. This study shows significant beneficial effects after a 10-wk outpatient multidisciplinary rehabilitation program on exercise capacity and quality of life. This study can contribute to implementation of a safe outpatient rehabilitation program for PAH patients.
Departments of Pulmonary Medicine (Drs Koudstaal, van den Toorn, Chandoesing, and Boomars and Ms Wapenaar) and Cardiology (Dr van den Bosch), Erasmus MC, University Medical Center Rotterdam, the Netherlands; and Pulmonary Rehabilitation Centre, Revant, Breda, the Netherlands (Dr van Ranst and Mr Beesems). Both T. Koudstaal and M. Wapenaar contributed equally and share first authorship for the article.
Correspondence: Karin Boomars, MD, PhD, Department of Pulmonary Medicine, Erasmus University Medical Center, Doctor Molewaterplein 40, 3015 GD Rotterdam, The Netherlands (email@example.com).
The authors declare no conflicts of interest.
Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.jcrpjournal.com).