Granulomatosis with Polyangiitis Presenting as Aortic Valve Incompetence: Case Report and a Brief Review of the Literature

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)–associated small-vessel vasculitis that predominantly affects the respiratory tract and kidneys, with cardiac involvement uncommon and often underrecognized. When present, cardiac manifestations are associated with increased morbidity and mortality and may include myocarditis, pericarditis, conduction abnormalities, coronary arteritis, and intracardiac masses. The nonspecific nature of cardiac symptoms often delays diagnosis, underscoring the importance of heightened clinical suspicion.

We report a case of GPA complicated by clinically significant cardiac involvement, presenting with progressive cardiopulmonary symptoms and electrocardiographic abnormalities. Laboratory evaluation demonstrated antineutrophil cytoplasmic antibody positivity consistent with GPA, and multimodality cardiac imaging revealed myocardial involvement. Definitive diagnosis was established through histopathologic confirmation of necrotizing granulomatous inflammation, supporting vasculitic cardiac involvement. Cardiac magnetic resonance imaging played a critical role in delineating the extent of myocardial disease, consistent with prior studies demonstrating its utility in detecting overt and subclinical cardiac manifestations of ANCA-associated vasculitis.

The patient was treated with immunosuppressive therapy in accordance with established vasculitis management strategies, resulting in clinical stabilization. This case highlights the diagnostic challenge posed by cardiac GPA and reinforces the importance of integrating advanced cardiac imaging with serologic and tissue-based diagnosis to guide timely therapy. Increased awareness of cardiac involvement in GPA is essential to facilitate early recognition, individualized treatment, and prevention of irreversible cardiac injury.