PT - JOURNAL ARTICLE AU - Farina, Stefania AU - Correale, Michele AU - Bruno, Noemi AU - Paolillo, Stefania AU - Salvioni, Elisabetta AU - Badagliacca, Roberto AU - Agostoni, Piergiuseppe ED - , TI - The role of cardiopulmonary exercise tests in pulmonary arterial hypertension AID - 10.1183/16000617.0134-2017 DP - 2018 Jun 30 TA - European Respiratory Review PG - 170134 VI - 27 IP - 148 4099 - http://err.ersjournals.com/content/27/148/170134.short 4100 - http://err.ersjournals.com/content/27/148/170134.full SO - EUROPEAN RESPIRATORY REVIEW2018 Jun 30; 27 AB - Despite recent advances in the therapeutic management of patients affected by pulmonary arterial hypertension (PAH), survival remains poor. Prompt identification of the disease, especially in subjects at increased risk of developing PAH, and prognostic stratification of patients are a necessary target of clinical practice but remain challenging. Cardiopulmonary exercise test (CPET) parameters, particularly peak oxygen uptake, end-tidal carbon dioxide tension and the minute ventilation/carbon dioxide production relationship, emerged as new prognostic tools for PAH patients. Moreover, CPET provides a comprehensive pathophysiological evaluation of patients' exercise limitation and dyspnoea, which are the main and early symptoms of the disease. This review focuses on the role of CPET in the management of PAH patients, reporting guideline recommendations for CPET and discussing the pathophysiology of exercise limitation and the most recent use of CPET in the diagnosis, prognosis and therapeutic targeting of PAH.CPET provides valuable information on diagnosis, prognosis and therapy in PAH but it should be performed by experts http://ow.ly/q5O230j6hta