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Bhatti YJ, Rice AJ, Kempny A, et al. Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing. Pulm Circ. 2019;9(2):2045894019845615doi: 10.1177/2045894019845615.
Bhatti, Y. J., Rice, A. J., Kempny, A., Dimopoulos, K., Price, L. C., Ranu, H., Wells, A., Wort, S. J., & McCabe, C. (2019). Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing. Pulmonary circulation, 9(2), 2045894019845615. https://doi.org/10.1177/2045894019845615
Bhatti, Yousaf J, et al. "Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing." Pulmonary circulation vol. 9,2 (2019): 2045894019845615. doi: https://doi.org/10.1177/2045894019845615
Bhatti YJ, Rice AJ, Kempny A, Dimopoulos K, Price LC, Ranu H, Wells A, Wort SJ, McCabe C. Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing. Pulm Circ. 2019 Apr-Jun;9(2):2045894019845615. doi: 10.1177/2045894019845615. PMID: 30945593; PMCID: PMC6469282.
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Pulm Circ. 2019 Apr-Jun;9(2):2045894019845615. doi: 10.1177/2045894019845615.

Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing.

Pulmonary circulation

Yousaf J Bhatti, Alexandra J Rice, Aleksander Kempny, Konstantinos Dimopoulos, Laura C Price, Harpreet Ranu, Athol Wells, S John Wort, Colm McCabe

Affiliations

  1. 1 Adult Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
  2. 2 Department of Histopathology, Royal Brompton Hospital, London, UK.
  3. 3 Imperial College, London, Royal Brompton Hospital, UK.
  4. 4 Department of Respiratory Medicine, Brighton and Sussex Hospitals NHS Trust, Brighton, UK.
  5. 5 Department of Respiratory Medicine, Royal Brompton Hospital, London, UK.

PMID: 30945593 PMCID: PMC6469282 DOI: 10.1177/2045894019845615

Abstract

Early diagnosis of pulmonary artery hypertension (PAH) is diagnostically challenging given the extent of pulmonary vascular remodeling required to bring about clinical signs and symptoms. Exercise testing can be invaluable in this setting, as stressing the cardiopulmonary system may unmask early disease. This report describes a young patient with a positive family history of PAH in whom contemporaneous invasive cardiopulmonary exercise testing and surgical lung biopsy reveal the novel association between exercise pulmonary hypertension (ePH) and early histological changes of PAH. Exercise PH currently carries no pathological correlates which means the hemodynamic effects of early pulmonary vascular remodeling remain unknown. Following the recent proceedings from the World Symposium in Pulmonary Hypertension 2018, which broaden the hemodynamic definition of PAH, this report suggests an important association between ePH and early pulmonary vascular remodeling supporting a role for exercise hemodynamic evaluation in patients at increased familial risk of PAH.

Keywords: cardiopulmonary physiology and pathophysiology; vascular remodeling

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