Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis

Johannes P Schwaiger, Christopher D Loder, David Dobarro, Thomas Kaier, Sally Reddecliffe, Benjamin E Schreiber, Clive Handler, Christopher P Denton, John G Coghlan, Johannes P Schwaiger, Christopher D Loder, David Dobarro, Thomas Kaier, Sally Reddecliffe, Benjamin E Schreiber, Clive Handler, Christopher P Denton, John G Coghlan

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 μm 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.

Conflict of interest statement

The authors declare no competing financial interests.

Figures

Figure 1. Small pulmonary artery side branches…
Figure 1. Small pulmonary artery side branches and the smallest pulmonary artery imaged.
Coloured tracings represent endoluminal contours. (a) 60 μm side branch (measurement “A”) (b) 330 μm side branch (measurement “C”) (c) diameter of vessel approaching diameter of the OCT catheter (470 μm). A small stitch artefact at 7 o’ clock.
Figure 2. Examples of IMT measurement in…
Figure 2. Examples of IMT measurement in patients with and without PAH.
Clearly defined structure at outer border. All three pictures demonstrate eccentric IMT thickening with difficult delineation towards adventitia. Coloured tracings represent endoluminal contours. (a) SSc, no PAH. IMT 0.10 mm (measurement “C”). PA mean 19 mmHg (b) SSc-PAH. IMT 0.27 mm (measurement “B”). mPAP 61 mmHg (c) SSc-PAH. IMT 0.15 mm (measurement “B”). mPAP 46 mmHg.
Figure 3. Examples of pulmonary arteries in…
Figure 3. Examples of pulmonary arteries in patients with PAH (limited and good haemodynamic response) and without PAH.
Coloured tracings represent endoluminal contours. (a) SSc-PAH. mPAP 51 mmHg. Treatment with Sildenafil. Limited haemodynamic response, 8% reduction in PVR. 0.2 side branches <300 μm per cm vessel (b) SSc-PAH. mPAP 29 mm Hg. Treatment with Bosentan. Good haemodynamic response, 51% reduction in PVR. 6.4 side branches <300 μm per cm vessel (c) SSc. mPAP 19 mmHg. 5.8 side branches <300 μm per cm vessel.
Figure 4. Graphical representation of the Pearson’s…
Figure 4. Graphical representation of the Pearson’s correlation between IMTA at 2 mm vessel level and mPAP at time of OCT (r = 0.60, p = 0.004).
Figure 5. Intravascular thrombus formation in patients…
Figure 5. Intravascular thrombus formation in patients with SSc-PAH.
Coloured tracings represent endoluminal contours. (a,b) SSc-PAH; wall-adherent thrombus (c) SSc-PAH; wall-adherent thrombus and web.
Figure 6. Swan-Ganz-Catheter in wedge position with…
Figure 6. Swan-Ganz-Catheter in wedge position with selective pulmonary angiography in lower right lobe.
OCT catheter in distal pulmonary artery.

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