Pathophysiology of exercise intolerance in pulmonary arterial hypertension

Derek L. Tran, Edmund M. T. Lau, David S. Celermajer, Glen M. Davis, Rachael Cordina

Research output: Contribution to journalReview articleResearchpeer-review

Abstract

Pulmonary arterial hypertension (PAH) is a chronic disease with poor prognosis and important exercise limitation despite the proliferation of treatment options in the last decade. Chronically increased right ventricular (RV) 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. RV 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.

LanguageEnglish
Pages148-159
Number of pages12
JournalRespirology
Volume23
Issue number2
DOIs
Publication statusPublished - 1 Feb 2018

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Respiratory Muscles
Pulmonary Hypertension
Uncertainty
Blood Vessels
Chronic Disease
Heart Failure
Quality of Life
Lung
Population

Keywords

  • exercise
  • heart failure
  • physiology
  • pulmonary hypertension
  • respiratory muscle

Cite this

Tran, D. L., Lau, E. M. T., Celermajer, D. S., Davis, G. M., & Cordina, R. (2018). Pathophysiology of exercise intolerance in pulmonary arterial hypertension. Respirology, 23(2), 148-159. https://doi.org/10.1111/resp.13141
Tran, Derek L. ; Lau, Edmund M. T. ; Celermajer, David S. ; Davis, Glen M. ; Cordina, Rachael. / Pathophysiology of exercise intolerance in pulmonary arterial hypertension. In: Respirology. 2018 ; Vol. 23, No. 2. pp. 148-159.
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Tran, DL, Lau, EMT, Celermajer, DS, Davis, GM & Cordina, R 2018, 'Pathophysiology of exercise intolerance in pulmonary arterial hypertension', Respirology, vol. 23, no. 2, pp. 148-159. https://doi.org/10.1111/resp.13141

Pathophysiology of exercise intolerance in pulmonary arterial hypertension. / Tran, Derek L.; Lau, Edmund M. T.; Celermajer, David S.; Davis, Glen M.; Cordina, Rachael.

In: Respirology, Vol. 23, No. 2, 01.02.2018, p. 148-159.

Research output: Contribution to journalReview articleResearchpeer-review

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Tran DL, Lau EMT, Celermajer DS, Davis GM, Cordina R. Pathophysiology of exercise intolerance in pulmonary arterial hypertension. Respirology. 2018 Feb 1;23(2):148-159. https://doi.org/10.1111/resp.13141