Background. Pulmonary hypertension (PH) is an often complication of severe cystic fibrosis (CF); however, data on the presence and impact of pulmonary vasculopathy in adult CF patients with milder disease, is very limited. Aim. To investigate, for the first time, the impact of systolic pulmonary arterial pressure (PASP) on maximal exercise capacity in adults with mild-to-moderate cystic fibrosis, without PH at rest. Methods. This is a Case Control study. Seventeen adults with mild-to-moderate CF, without PH at rest (cases) and 10 healthy, nonsmoking, age, and height matched controls were studied. All subjects underwent maximal cardiopulmonary exercise testing and echocardiography before and within 1 minute after stopping exercise. Results. Exercise ventilation parameters were similar in the two groups; however, cases, compared to controls, had higher postexercise PASP and decreased exercise capacity, established with lower peak work rate, peak O2 uptake, anaerobic threshold, and peak O2 pulse. Furthermore, the change in PASP values before and after exercise was strongly correlated to the parameters of exercise capacity among cases but not among controls. Conclusions. CF adults with mild-to-moderate disease should be screened for the presence of pulmonary vasculopathy, since the elevation of PASP during exercise might contribute to impaired exercise capacity. 1. Introduction Exercise impairment in cystic fibrosis (CF) is well established and a variety of determinants, such as pulmonary and nutritional factors, muscle dysfunction and deconditioning, have been studied in this direction [1–4]. It seems that the factors which are limiting exercise tend to vary across disease stages; ventilatory impairment is probably the major factor limiting exercise in severe disease, while nonpulmonary factors seem to be related to reduced exercise capacity in mild and moderate disease [4]. Pulmonary hypertension (PH), which is a common determinant of exercise capacity in patients with respiratory disorders [5], is an often complication of CF. PH is observed in 20–65% of adult CF patients with severe disease [6–10], and it has been associated with increased mortality [6, 11]. However, data on its frequency and impact among patients with milder disease are limited. Although adult CF patients with mild-to-moderate disease achieve maximum exercise without generally reaching ventilatory limitation [4], the potential effect of pulmonary vasculopathy on exercise capacity, in this patient population, has not yet been clarified. In this study we hypothesized that pulmonary
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