Effects of exercise training in patients with idiopathic pulmonary arterial hypertension

Abstract

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; p50.003) together with an increase in exercise endurance time (p,0.001). Moreover, exercise training increased quadriceps strength by 13% (p50.005) and quadriceps endurance by 34% (p50.001).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.

Safety and efficacy of exercise training in various forms of pulmonary hypertension

“…However, training studies cannot be performed in a blinded design and a selection bias towards highly motivated patients cannot be excluded [1,4]. Other studies that applied a less intense outpatient training programme in small cohorts of pulmonary arterial hypertension (PAH) patients did reveal a less pronounced [3], or even no significant [2,5], improvement in 6MWD or peak oxygen consumption, but an improvement in exercise endurance [2,5] and muscle capillarisation [2]. The effect of training in patients with other aetiologies of pulmonary hypertension (PH), such as associated PAH (APAH) or outof-proportion PH with left heart failure or respiratory diseases, has not yet been evaluated.…”

“…P revious studies in rather small cohorts of patients with idiopathic pulmonary arterial hypertension (IPAH) and inoperable chronic thromboembolic pulmonary hypertension (CTEPH) have shown beneficial effects of exercise training as add-on to disease-targeted medical therapy, by increasing exercise capacity [1][2][3] and quality of life [1]. Exercise training also improved peak oxygen consumption and World Health Organization (WHO) functional class [1], and possibly the clinical outcome, with 1-and 2-yr survival rates of 100 and 95%, respectively [4].…”

Vascular Remodeling Process in Pulmonary Arterial Hypertension, with Focus on miR‐204 and miR‐126 (2013 Grover Conference Series)

“…However, increasing data in the literature describe peripheral vascular damage in PAH, suggesting a more systemic impairment in this disease. 74,75 Potus et al 76 showed a decrease of capillary density in peripheral skeletal muscles of PAH patients, suggesting a peripheral angiogenesis defect in PAH. Furthermore, they identified the molecular pathway implicated and showed the major role of microRNA in this pathological process.…”

The Role of Exercise Testing in the Modern Management of Pulmonary Arterial Hypertension

“…Despite this reduced ventilatory capacity and increased demand, ventilatory limitation is said not to occur in PAH unless there is concomitant lung disease. Peripheral muscle dysfunction has been shown in PAH in the form of reduced capillary density and oxidative enzymes in muscle biopsies of these patients [10]. This abnormality can lead to impairment of extraction of oxygen from muscle capillaries and should be reflected in abnormally high values of S v O 2 at end-exercise [11].…”