Key result
Aortic valve replacement improves LVEF by ~20 percentage points in ANCA-associated vasculitis with severe AR.
Why the study?
ANCA-associated vasculitides predominantly affect the myocardium when involving the heart, while valvular involvement is rare and only described anecdotally.
Case Report (n=1)
No
In patients with ANCA-associated vasculitis presenting with worsening dyspnea, severe valvular inflammation and regurgitation should be considered alongside pulmonary exacerbation and evaluated via a multidisciplinary approach.
Alerts clinicians to evaluate for severe AR in dyspneic ANCA vasculitis patients; extends sparse case-level evidence without altering practice.
BACKGROUND Granulomatosis with polyangiitis (GPA)/Wegener's granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss' syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. CASE REPORT We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30-35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps. CONCLUSIONS Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered.
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Guterbaum et al. (2019) conducted a case report in ANCA-associated vasculitis with severe aortic regurgitation and heart failure (n=1). Aortic valve replacement (implantation of an aortic valve bioprosthesis via thoracotomy) was evaluated on Symptomatic relief of dyspnea and left ventricular ejection fraction recovery. Aortic valve replacement in a 56-year-old male with ANCA-associated vasculitis and severe aortic regurgitation improved dyspnea from NYHA III to II and increased LVEF from 30-35% to 50-55% at 1 year.
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