RT Journal Article SR Electronic T1 Effects of exercise training in patients with idiopathic pulmonary arterial hypertension JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 669 OP 675 DO 10.1183/09031936.00027909 VO 34 IS 3 A1 F. S. de Man A1 M. L. Handoko A1 H. Groepenhoff A1 A. J. van 't Hul A1 J. Abbink A1 R. J. H. Koppers A1 H. P. Grotjohan A1 J. W. R. Twisk A1 H-J. Bogaard A1 A. Boonstra A1 P. E. Postmus A1 N. Westerhof A1 W. J. van der Laarse A1 A. Vonk-Noordegraaf YR 2009 UL http://erj.ersjournals.com/content/34/3/669.abstract AB We determined the physiological effects of exercise training on exercise capacity and quadriceps muscle function in patients with idiopathic pulmonary arterial hypertension (iPAH). In total, 19 clinically stable iPAH patients (New York Heart Association II-III) underwent a supervised exercise training programme for the duration of 12 weeks. Maximal capacity, endurance capacity and quadriceps function were assessed at baseline and after 12 weeks. In 12 patients, serial quadriceps muscle biopsies were obtained. 6-min walk distance and peak exercise capacity did not change after training. However, endurance capacity improved significantly after training, demonstrated by a shift of the anaerobic threshold to a higher workload (from 32±5 to 46±6 W; p = 0.003) together with an increase in exercise endurance time (p<0.001). Moreover, exercise training increased quadriceps strength by 13% (p = 0.005) and quadriceps endurance by 34% (p = 0.001). Training enhanced aerobic capacity of the quadriceps, by increasing capillarisation (1.36±0.10 to 1.78±0.13 capillaries per muscle fibre; p<0.001) and oxidative enzyme activity, especially of the type-I (slow) muscle fibres. No changes were found in cross-sectional area and fibre type distribution. Exercise training in iPAH improves exercise endurance and quadriceps muscle function, which is also reflected by structural changes of the quadriceps.