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Effects of exercise training in patients with idiopathic pulmonary arterial hypertension

Depts of ¹ Pulmonology, and ² Physiology, Institute for Cardiovascular Research, and ⁷ Dept of Clinical Epidemiology and Biostatistics, The Institute for Research in Extramural Medicine, VU University Medical Center, Amsterdam, ³ Breda Rehabilitation Center Foundation, Breda, ⁴ Rijnlands Rehabilitation Center, Leiden, ⁵ Dept of Pulmonology, Leeuwarden Medical Center, Leeuwarden, and ⁶ Dept of Pulmonology, Isala Clinics, Zwolle, The Netherlands.

CORRESPONDENCE: A. Vonk Noordegraaf, Dept of Pulmonology, VU University Medical Center, Boelelaan 1117, 1081 HV Amsterdam, The Netherlands. E-mail: a.vonk{at}vumc.nl

Keywords: Biopsy, exercise training, pathophysiology, pulmonary hypertension, skeletal muscle dysfunction

Received: February 17, 2009
Accepted March 13, 2009

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.