TY  - JOUR
N2  - Our current understanding of the pathophysiology of pulmonary vascular disease is incomplete, since information about alterations of the pulmonary vasculature in pulmonary arterial hypertension (PAH) is primarily provided by autopsy or tissue specimens. The aim of this study was to compare the distal pulmonary vasculature of <2?mm in diameter in Systemic Sclerosis (SSc) patients with (n?=?17) and without (n?=?5) associated PAH using Optical Coherence Tomography during Right Heart catheterization. SSc-PAH patients showed significant thickening of Intima Media Thickening Area compared to patients without PAH (27 +/- 5.8% vs. 21 +/- 1.4%, p?=?0.024). A good haemodynamic response to previous targeted PAH treatment was associated with a significantly greater number of small pulmonary artery side branches <300??m per cm vessel (3.8 +/- 1.1 vs. 1.8 +/- 1.1; p?=?0.010) and not associated with Intima Media thickening Area (26 +/- 5.4% vs. 28 +/- 6.7%; p?=?0.6). Unexpected evidence of pulmonary artery thrombus formation was found in 19% of SSc-PAH patients. This is the first in-vivo study demonstrating a direct link between a structural abnormality of pulmonary arteries and a response to targeted treatment in PAH. Intravascular imaging may identify subgroups that may benefit from anticoagulation.
ID  - discovery1544974
UR  - http://dx.doi.org/10.1038/srep43304
SN  - 2045-2322
JF  - Scientific Reports
A1  - Schwaiger, JP
A1  - Loder, CD
A1  - Dobarro, D
A1  - Kaier, T
A1  - Reddecliffe, S
A1  - Schreiber, BE
A1  - Handler, C
A1  - Denton, CP
A1  - Coghlan, JG
KW  - Interventional cardiology; Systemic sclerosis; Thrombosis
TI  - Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis
AV  - public
Y1  - 2017/02/24/
VL  - 7
N1  - This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article?s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/
ER  -