Extensive Cardiac Involvement in ANCA-Associated Vasculitis Presenting With Severe Aortic Regurgitation and Atrioventricular Block.

BACKGROUND

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis rarely presents with severe inflammatory cardiac involvement. Valvuloaortic disease with high-grade atrioventricular block is particularly uncommon.

CASE SUMMARY: A 53-year-old woman was referred with a presumptive diagnosis of non-ST-segment elevation myocardial infarction. Coronary angiography showed no obstructive epicardial coronary artery disease. Multimodality imaging demonstrated severe aortic regurgitation, inflammatory valvuloaortic thickening with cusp retraction, moderate pericardial effusion, nonischemic septal late gadolinium enhancement, and progressive high-grade atrioventricular block. Rheumatologic assessment supported myeloperoxidase-positive ANCA-associated vasculitis. She underwent mechanical aortic valve replacement and cardiac resynchronization therapy pacemaker implantation. Rituximab was subsequently initiated as immunosuppressive therapy. Histopathology showed active inflammatory valvulitis without detectable microorganisms.

DISCUSSION: This case highlights a rare but clinically important inflammatory cardiac phenotype and underscores the value of multimodality imaging and multidisciplinary evaluation.

TAKE-HOME MESSAGE: ANCA-associated vasculitis may rarely present as multicompartment cardiac involvement and should be considered in severe inflammatory valvuloaortic disease with conduction abnormalities.