Determinants of an elevated pulmonary arterial pressure in patients with pulmonary arterial hypertension

Authors

Seiichiro Sakao, Chiba University
Norbert F. Voelkel, Virginia Commonwealth UniversityFollow
Nobuhiro Tanabe, Chiba University
Koichiro Tatsumi, Chiba University

Document Type

Article

Original Publication Date

2015

Journal/Book/Conference Title

Respiratory Research

Volume

16

DOI of Original Publication

10.1186/s12931-015-0246-y

Comments

Originally published at http://dx.doi.org/10.1186/s12931-015-0246-y

Date of Submission

December 2015

Abstract

Given the difficulty of diagnosing early-stage pulmonary arterial hypertension (PAH) due to the lack of signs and symptoms, and the risk of an open lung biopsy, the precise pathological features of presymptomatic stage lung tissue remain unknown. It has been suggested that the maximum elevation of the mean pulmonary arterial pressure (P_pa) is achieved during the early symptomatic stage, indicating that the elevation of the mean P_pa is primarily driven by the pulmonary vascular tone and/or some degree of pulmonary vascular remodeling completed during this stage. Recently, the examination of a rat model of severe PAH suggested that the severe PAH may be primarily determined by the presence of intimal lesions and/or the vascular tone in the early stage. Human data seem to indicate that intimal lesions are essential for the severely increased pulmonary arterial blood pressure in the late stage of the disease.

However, many questions remain. For instance, how does the pulmonary hemodynamics change during the course of the disease, and what drives the development of severe PAH? Although it is generally acknowledged that both pulmonary vascular remodeling and the vascular tone are important determinants of an elevated pulmonary arterial pressure, which is the root cause of the time-dependent progression of the disease? Here we review the recent histopathological concepts of PAH with respect to the progression of the lung vascular disease.

Rights

Copyright © Sakao et al. 2015 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Is Part Of

VCU Internal Medicine Publications