Idiopathic pulmonary arterial hypertension (IPAH) is a pulmonary vasculopathy of unknown aetiology. Dyspnoea, peripheral airway obstruction and inefficient ventilation are common in IPAH. Data on respiratory muscle function are lacking.This prospective single-centre study included 26 female and 11 male patients with IPAH in World Health Organization functional classes II-IV. Mean¡SD pulmonary artery pressure was 48.6¡16.9 in females and 53.1¡22.9 mmHg in males; cardiac output was 3.7¡1.3 and 4.2¡1.7 L?min -1 .Maximal inspiratory pressure (PI,max) was lower in the female patients than in 20 controls (5.3¡2.0 versus 8.2¡2.0 kPa). In the male patients, PI,max was lower than in 25 controls (6.8¡2.2 versus 10.5¡3.7 kPa). Maximal expiratory pressure (PE,max) was lower in the female patients than in controls (6.2¡2.6 versus 9.5¡2.1 kPa), and in male patients as compared to controls (7.1¡1.6 versus 10.3¡3.9 kPa). There was no correlation between PI,max or PE,max and parameters of pulmonary haemodynamics or exercise testing. The ratio of mouth occlusion pressure within the first 0.1 s of inspiration and PI,max was higher in IPAH than in controls (females 0.067¡0.066 versus 0.021¡0.008; males 0.047¡0.061 versus 0.023¡0.016).In conclusion, this study provides the first evidence of inspiratory and expiratory muscle weakness in idiopathic pulmonary arterial hypertension. The pathomechanisms and the prognostic significance should be further investigated.KEYWORDS: Exercise test, pulmonary hypertension, respiratory function test, respiratory muscles I diopathic pulmonary arterial hypertension (IPAH) is defined as a pulmonary vasculopathy of unknown aetiology, predominantly affecting females [1]. Minute ventilation (V9E) is increased at rest and during exercise [2]. The ventilatory response to exercise in IPAH patients, i.e. the ratio of V9E to carbon dioxide production (V9CO 2 ) during exercise (V9E/V9CO 2 slope), is also increased and is a predictor of poor prognosis [2,3]. The increased V9E/V9CO 2 slope indicates inefficient ventilation in IPAH, which can partly be attributed to pulmonary-perfusion abnormalities [2]. It has been shown by the current authors' group and others that, in congestive left ventricular failure, inefficient ventilation is associated with reduced inspiratory muscle strength [4][5][6]. Moreover, inspiratory muscle function has recently been identified as a prognostic predictor in congestive left ventricular failure [7]. Risk stratification of patients with congestive left ventricular failure is improved by using the combination of maximal inspiratory pressure (PI,max) and peak oxygen consumption (V9O 2 ) [7].Inspiratory and expiratory muscle strength have not been studied in IPAH. Therefore, this prospective study investigated respiratory muscle function and the mouth occlusion pressures in 37 patients with IPAH. PI,max and maximal expiratory pressure (PE,max) were prospectively assessed in IPAH patients with varying degrees of disease and exercise limitation. Moreover, invasive (right heart c...