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Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis.

ABSTRACT: 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.

SUBMITTER: Schwaiger JP

PROVIDER: S-EPMC5324107 | biostudies-literature | 2017 Feb

REPOSITORIES: biostudies-literature

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Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis.

Schwaiger Johannes P JP Loder Christopher D CD Dobarro David D Kaier Thomas T Reddecliffe Sally S Schreiber Benjamin E BE Handler Clive C Denton Christopher P CP Coghlan John G JG

Scientific reports 20170224

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 ...[more]

PMID: 28233825

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Project description:ImportanceInvestigating the quantitative 3-dimensional (3-D) anatomy of polypoidal complex is important for a better understanding of the pathogenesis of polypoidal choroidal vasculopathy (PCV).ObjectiveTo quantitatively evaluate the 3-D characteristics of polypoidal structures, branching vascular networks (BVNs), and origin of PCV using optical coherence tomography angiography (OCTA) and multiple image systems.Design, setting, and participantsA prospective, observational study was conducted in 47 consecutive Taiwanese patients (47 eyes) from May 21, 2015, to April 30, 2017. All participants were scanned with the Optovue-RTVue-XR-Avanti OCTA system. Patients in whom PCV was identified on OCTA were examined to define characteristics and structures of the original spouting vessels (stalks) from the choroid, polypoidal structures, and BVNs on OCTA.Main outcomes and measuresQuantitative analysis of 3-D structures of the polypoidal complex.ResultsAmong the 47 patients, the mean (SD) patient age was 68.9 (8.0) years, and 28 (59.6%) men were included. Clear images of polypoidal structures could be detected in 17 eyes (36.2%, 22 polypoidal structures), BVNs in 26 eyes (55.3%, 26 tufts of BVNs), and stalks of origin from the choroid in 26 eyes (55.3%, 26 stalks) on the en face plane on OCTA. All polypoidal structures were found at a mean (SD) height of 45.3 (36.1) μm above the retinal pigment epithelium (RPE) reference plane that was preset by the machine, while the BVNs were found at a mean (SD) depth of 28.6 (14.2) μm below the RPE reference plane and the choroidal stalks at 80.4 (24.4) μm below RPE reference plane. The mean (SD) thickness of polypoidal structures was 38.4 (15.5) μm and of BVNs, 60.2 (25.0) μm. The polypoidal structures were all above the Bruch membrane within the dome of the RPE detachment, the choroidal stalks were all in the choroid layer. The BVNs could be either above (up to 18 μm), within, or below (up to 28 μm) the Bruch membrane and were in proximity to the double layers of flattened RPE detachment.Conclusions and relevanceThese results demonstrate a 3-D architecture of PCV that may be helpful for a better understanding of the anatomy, pathophysiology, and pathogenesis of PCV.

Dataset Information

Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis.

Publications

Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis.

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