Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing
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 dis...
Main Authors: | , , , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Wiley
2019-04-01
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Series: | Pulmonary Circulation |
Online Access: | https://doi.org/10.1177/2045894019845615 |
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author | Yousaf J. Bhatti Alexandra J. Rice Aleksander Kempny Konstantinos Dimopoulos Laura C. Price Harpreet Ranu Athol Wells S. John Wort Colm McCabe |
author_facet | Yousaf J. Bhatti Alexandra J. Rice Aleksander Kempny Konstantinos Dimopoulos Laura C. Price Harpreet Ranu Athol Wells S. John Wort Colm McCabe |
author_sort | Yousaf J. Bhatti |
collection | DOAJ |
description | 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. |
first_indexed | 2024-04-12T09:56:19Z |
format | Article |
id | doaj.art-637d18bdefe0404280e9d8556908ab32 |
institution | Directory Open Access Journal |
issn | 2045-8940 |
language | English |
last_indexed | 2024-04-12T09:56:19Z |
publishDate | 2019-04-01 |
publisher | Wiley |
record_format | Article |
series | Pulmonary Circulation |
spelling | doaj.art-637d18bdefe0404280e9d8556908ab322022-12-22T03:37:40ZengWileyPulmonary Circulation2045-89402019-04-01910.1177/2045894019845615Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testingYousaf J. Bhatti0Alexandra J. Rice1Aleksander Kempny2Konstantinos Dimopoulos3Laura C. Price4Harpreet Ranu5Athol Wells6S. John Wort7Colm McCabe8Adult Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UKDepartment of Histopathology, Royal Brompton Hospital, London, UKImperial College, London, Royal Brompton Hospital, UKImperial College, London, Royal Brompton Hospital, UKAdult Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UKDepartment of Respiratory Medicine, Brighton and Sussex Hospitals NHS Trust, Brighton, UKDepartment of Respiratory Medicine, Royal Brompton Hospital, London, UKImperial College, London, Royal Brompton Hospital, UKImperial College, London, Royal Brompton Hospital, UKEarly 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.https://doi.org/10.1177/2045894019845615 |
spellingShingle | Yousaf J. Bhatti Alexandra J. Rice Aleksander Kempny Konstantinos Dimopoulos Laura C. Price Harpreet Ranu Athol Wells S. John Wort Colm McCabe Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing Pulmonary Circulation |
title | Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing |
title_full | Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing |
title_fullStr | Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing |
title_full_unstemmed | Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing |
title_short | Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing |
title_sort | early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing |
url | https://doi.org/10.1177/2045894019845615 |
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