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...

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Main Authors: Yousaf J. Bhatti, Alexandra J. Rice, Aleksander Kempny, Konstantinos Dimopoulos, Laura C. Price, Harpreet Ranu, Athol Wells, S. John Wort, Colm McCabe
Format: Article
Language:English
Published: Wiley 2019-04-01
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.
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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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