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Chronically increased right ventricular (<jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content>) afterload results in right heart failure and without treatment, rapid clinical deterioration is common. Exercise intolerance is the cardinal feature of the disease impacting upon quality of life and clinical outcome. The pathophysiological mechanisms that lead to reduced exercise capacity in this population are complex with ventriculoarterial uncoupling likely to be the predominant feature. The relative contributions of additional factors that contribute to exercise limitation beyond ventriculoarterial uncoupling have not been characterized. This review addresses these factors with a focus on recent developments and uncertainties. <jats:styled-content style=\"fixed-case\">RV<\/jats:styled-content> maladaptation and the intricate interplay between the heart, abnormal pulmonary vascular bed and peripheral factors such as dysfunction of the respiratory and peripheral muscles are discussed in detail.<\/jats:p>","DOI":"10.1111\/resp.13141","type":"journal-article","created":{"date-parts":[[2017,8,28]],"date-time":"2017-08-28T03:06:38Z","timestamp":1503889598000},"page":"148-159","source":"Crossref","is-referenced-by-count":52,"title":["Pathophysiology of exercise intolerance in pulmonary arterial hypertension"],"prefix":"10.1111","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3274-363X","authenticated-orcid":false,"given":"Derek L.","family":"Tran","sequence":"first","affiliation":[{"name":"Faculty of Health Sciences The University of Sydney  Sydney NSW Australia"},{"name":"Department of Clinical Medicine, Faculty of Medicine and Health Sciences Macquarie University  Sydney NSW Australia"},{"name":"Pulmonary Hypertension Service Royal Prince Alfred Hospital  Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1473-0437","authenticated-orcid":false,"given":"Edmund M.T.","family":"Lau","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension Service Royal Prince Alfred Hospital  Sydney NSW Australia"},{"name":"Sydney Medical School The University of Sydney  Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David S.","family":"Celermajer","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension Service Royal Prince Alfred Hospital  Sydney NSW Australia"},{"name":"Sydney Medical School The University of Sydney  Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glen M.","family":"Davis","sequence":"additional","affiliation":[{"name":"Faculty of Health Sciences The University of Sydney  Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachael","family":"Cordina","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension Service Royal Prince Alfred Hospital  Sydney NSW Australia"},{"name":"Sydney Medical School The University of Sydney  Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,8,27]]},"reference":[{"key":"e_1_2_11_2_1","unstructured":"GalieN HumbertM VachieryJL GibbsS LangI TorbickiA SimonneauG PeacockA NoordegraafAV BeghettiMet al.;European Respiratory Society (ERS);European Society of Cardiology (ESC). 2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC) International Society for Heart and Lung Transplantation (ISHLT).Eur. 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