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

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Title: Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing
Authors: Bhatti, Y
Rice, AJ
Kempny, A
Dimopoulos, K
Price, L
Ranu, H
Wells, A
Wort, SJ
McCabe, C
Item Type: Journal Article
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.
Issue Date: 1-Apr-2019
Date of Acceptance: 1-Apr-2019
ISSN: 2045-8940
Publisher: SAGE Publications (UK and US)
Start Page: 1
End Page: 4
Journal / Book Title: Pulmonary Circulation
Volume: 9
Issue: 2
Copyright Statement: ┬ęThe Author(s) 2019. Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the CreativeCommons Attribution-NonCommercial 4.0 License ( whichpermits non-commercial use, reproduction and distribution of the work without further permission provided the originalwork is attributed as specified on the SAGE and Open Access pages (
Keywords: Science & Technology
Life Sciences & Biomedicine
Cardiac & Cardiovascular Systems
Respiratory System
Cardiovascular System & Cardiology
cardiopulmonary physiology and pathophysiology
vascular remodeling
cardiopulmonary physiology and pathophysiology
vascular remodeling
Publication Status: Published
Conference Place: United States
Open Access location:
Online Publication Date: 2019-04-04
Appears in Collections:National Heart and Lung Institute

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