Objectives: To evaluate effect of exercise and respiratory training alone or in conjunction with sildenafil on exercise capacity and quality of life (QOL) of patients had pulmonary arterial hypertension (PAH) secondary to idiopathic pulmonary fibrosis (IPF).
Materials & Methods: Patients were categorized into 3 groups (n=12): Group S received sildenafil 25 mg trice/day; Group E underwent exercise intervention and Group ES received both modalities. Exercise consisted of inpatient pulmonary rehabilitation for 30 min/day for 3 weeks (wk) and continued at home for additional 12 wk. Physiologic assessment included determination of FVC, FEV1 and FEV1/FVC using spirometry and arterial blood gases analysis. Outcome was defined as changes from baseline to week 15 in 6-minute walking distance (6MWD), dyspnea scoring by Medical Research Council (MRC), time till exercise intolerance and QOL scoring.
Results: All patients showed improvement of 6MWD, time till exercise intolerance and FVC and FEV1 and ratio FEV1/FVC. Frequency of patients had MRC4 was significantly lower, while MRC2 was significantly higher and post-6MWT Borg exertion score showed progressive decrease in all patients. Improvements were more pronounced in groups ES and E compared to group S with significant difference in favor of group ES. Patients of groups E and ES showed significant improvement of ability to ambulate, while the improvement was non-significant in group S compared to baseline scoring. Emotional behavior and recreation were significantly improved only in group ES.
Conclusion: Combined exercise training and sildenafil therapy improved exercise tolerance of patients with PAH secondary to IPF with improved QOL.
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