Lung function in pulmonary hypertension

Respir Med. 2015 Oct;109(10):1244-9. doi: 10.1016/j.rmed.2015.05.022. Epub 2015 May 27.


Breathlessness is a common symptom in pulmonary hypertension (PH) and an important cause of morbidity. Though this has been attributed to the well described pulmonary vascular abnormalities and subsequent cardiac remodelling, changes in the airways of these patients have also been reported and may contribute to symptoms. Our understanding of these airway abnormalities is poor with conflicting findings in many studies. The present review evaluates these studies for the major PH groups. In addition we describe the role of cardiopulmonary exercise testing in the assessment of pulmonary arterial hypertension (PAH) by evaluating cardiopulmonary interaction during exercise. As yet, the reasons for the abnormalities in lung function are unclear, but potential causes and the possible role of inflammation are discussed. Future research is required to provide a better understanding of this to help improve the management of these patients.

Keywords: Congenital heart disease; Idiopathic pulmonary arterial hypertension; Inflammation; Lung function tests; Pulmonary arterial hypertension; Pulmonary hypertension.

Publication types

  • Review

MeSH terms

  • Exercise Test
  • Humans
  • Hypertension, Pulmonary / metabolism
  • Hypertension, Pulmonary / pathology*
  • Hypertension, Pulmonary / physiopathology
  • Predictive Value of Tests
  • Pulmonary Diffusing Capacity
  • Respiratory Function Tests