Aortic valve replacement due to granulomatosis with polyangiitis: a case series

Abstract

Background: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic inflammatory process predominantly affecting upper and lower respiratory tract and kidneys. Valvular heart disease is a rare manifestation of GPA.

Case summary: We report two cases of acute valvular heart disease mimicking acute endocarditis caused by GPA. Both patients were middle-aged females with acute aortic valve regurgitation suggestive of possible infective endocarditis. In their recent medical history, atypical otitis and sinusitis were noted. The first patient was admitted with heart failure and the second patient because of persisting fever. Echocardiogram revealed severe aortic regurgitation with an additional structure on two cusps, suggestive of infective endocarditis in both patients. Urgent surgical replacement was performed; however, intraoperative findings did not show infective endocarditis, but severe inflammatory changes of the valve and surrounding tissue. In both patients, the valve was replaced by a prosthetic valve. Microscopic examination of the valve/myocardial biopsy showed diffuse acute and chronic inflammation with necrosis and necrotizing granulomas, compatible with GPA after infectious causes were excluded. Disease remission was obtained in both patients, in one patient with Rituximab and in the other with Glucocorticoids and Cyclophosphamide. Both had an uneventful follow-up.

Discussion: Granulomatosis with polyangiitis can be a rare cause of acute aortic valve regurgitation mimicking infective endocarditis with the need for surgical valve replacement. Atypical ear, nose, and throat symptoms can be a first sign of GPA. Symptom recognition is important for early diagnosis and appropriate treatment to prevent further progression of the disease.

Keywords: Case series; Aortic valve replacement; Endocarditis; Granulomatosis with polyangiitis (Wegener’s granulomatosis).

Figure 1

Bilateral ground-glass opacities on computed tomography thorax in Patient 1 (blue arrows).

Figure 2

Transoesophageal echocardiography in Patient 1. (A) Mid-oesophageal right ventricular inflow–outflow view showing thickened aortic valve leaflets with an additional structure at level of the non- and right coronary cusp. (B) Mid-oesophaegeal aortic valve long-axis view showing thickening of the aortic sinus in continuity with the base of the anterior mitral leaflet. Ao, aorta; LA, left atrium; lc, left coronary sinus and cusp; LV, left ventricle; nc, non-coronary; rc, right coronary; RV, right ventricle.

Figure 3

Macroscopic view of thickened and inflamed aortic valve leaflet in Patient 1.

Figure 4

Histopathology of the aortic valve in Patient 1: geographic necrosis (arrows) (haematoxylin and eosin staining, original magnification ×200).

Figure 5

Histopathology of the aortic valve in Patient 1: small granuloma (arrows) (haematoxylin and eosin staining, original magnification ×400).

Figure 6

Transoesophageal echocardiography in Patient 2. (A) Mid-oesophageal right ventricular inflow–outflow view showing thickened aortic valve leaflets with an additional structure at level of the non- and right coronary cusp. (B) Mid-oesophaegeal aortic valve long-axis view showing thickening of two aortic valves and the aortic sinus in continuity with the base of the anterior mitral leaflet. Ao, aorta; LA, left atrium; lc, left coronary sinus and cusp; LV, left ventricle; nc, non-coronary; rc, right coronary; RV, right ventricle.

Figure 7

Microscopy of the aortic valve in Patient 2, demonstrating a necrotizing granuloma with surrounding acute and chronic inflammatory infiltrate (arrows) (haematoxylin and eosin staining, original magnification ×200).