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Case Reports
. 2024 Oct 2;29(19):102579.
doi: 10.1016/j.jaccas.2024.102579.

Granulomatosis With Polyangiitis Causing Severe Aortic Regurgitation

Katarina L Fabre  1 , Megan Ellis  1 , Katlin T Schmitz  1 , Richard C Daly  2 , Andrew Layman  3 , Joseph J Maleszewski  3 , Raghav Julakanti  4 , Jared G Bird  4
Affiliations
Case Reports

Granulomatosis With Polyangiitis Causing Severe Aortic Regurgitation

Katarina L Fabre et al. JACC Case Rep. .

Abstract

Cardiac granulomatosis with polyangiitis is a rare necrotizing vasculitis impacting small to medium vessels. We present a case of granulomatosis with polyangiitis causing acute aortic valve regurgitation. Imaging reveals valve thickening, inflammation without destruction. Treatment involves glucocorticoids and rituximab for remission, with consideration of surgery for severe valve disease.

Keywords: aortic regurgitation; granulomatosis with polyangiitis; vasculitis.

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Conflict of interest statement

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Figure 1
Figure 1
PET-CT Scan Positron emission tomography-computed tomography scan with corresponding coronal computed tomography. Changes consistent with vasculitis involving the aortic root and ascending aorta.
Figure 2
Figure 2
Chest Computed Tomography Angiography Significant infiltrative tissue surrounding the aortic root involving the aortic valve commissures and associated central diastolic noncoaptation of the cusps. L = left coronary cusp; N = noncoronary cusp; R = right coronary cusp.
Figure 3
Figure 3
Transthoracic Echocardiogram Moderately thickened aortic valve leaflets, retracted, with asymmetric leaflet thickening at the commissural insertion points. Abbreviations as in Figure 2.
Figure 4
Figure 4
Transthoracic Echocardiogram With Color Doppler Findings with aortic valve leaflet thickening and retraction with a large central coaptation defect resulting in severe aortic valve regurgitation.
Figure 5
Figure 5
Follow-Up Chest Computed Tomography Angiography Overall improvement in the thickness of the aortic root after immunosuppressive therapy (yellow arrows). However, persistent soft tissue thickening is present at the valve commissure insertion points and leaflets (red arrows) resulting in central noncoaptation of the cusps. Abbreviations as in Figure 2.
Figure 6
Figure 6
Surgically Resected Aortic Valve Cusps (A) The right (R) and posterior (P) cusps show uniform, marked thickening (compared with the left [L] cusp), with concurrent scar retraction, indicative of postinflammatory changes. Low-power view of (B) a thickened semilunar valve (×50 original magnification) with (C) thick-walled neovascularization (arrows; ×100 original magnification) and (D) chronic inflammation (asterisks; ×100 original magnification). B, C, and D show hematoxylin and eosin staining.
See this image and copyright information in PMC

References

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