Abstract
BackgroundAntineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides have a variety of presentations, but cardiac valvular involvement is rarely diagnosed and its management is not established.Case presentationWe report the case of a 44 year old man who presented with an ANCA-associated systemic vasculitis and aortic regurgitation of unusual mechanism. Transthoracic and transesophageal echocardiography disclosed septal hypertrophy preventing a complete closure of the aortic valve and thus responsible for a massive aortic regurgitation. After 4 months of immunosuppressive therapy, the valve lesion did not subside and the patient had to undergo aortic valve replacement. This report also reviews the 20 cases of systemic ANCA-associated vasculitis with endocardial valvular involvement previously reported in the English language medical literature.ConclusionsValvular involvement in ANCA-associated systemic vasculitides is rarely reported. Most of these lesions are due to Wegener's granulomatosis and half are present when the diagnosis of vasculitis is made. The valvular lesion is usually isolated, aortic regurgitation being the most frequent type, and often requires valve replacement in the months that follow it's discovery.
Highlights
Antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides have a variety of presentations, but cardiac valvular involvement is rarely diagnosed and its management is not established.Case presentation: We report the case of a 44 year old man who presented with an ANCA-associated systemic vasculitis and aortic regurgitation of unusual mechanism
In this report we describe the case of a patient with an ANCA associated systemic vasculitis who presented with aortic regurgitation of unusual mechanism requiring surgical replacement
Four months after the beginning of treatment there was an absence of echocardiographic improvement of the septal hypertrophy and aortic regurgitation and the patient had developed congestive heart failure, so the decision was made to perform an aortic BicarbonTMvalve replacement
Summary
Valvular involvement in ANCA-associated systemic vasculitides is rarely reported. Most of these lesions are due to Wegener’s granulomatosis and half are present when the diagnosis of vasculitis is made. The valvular lesion is usually isolated, aortic regurgitation being the most frequent type, and often requires valve replacement in the months that follow it’s discovery
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