ABSTRACT Granulomatosis with polyangiitis, formerly Wegener's granulomatosis, is a rare antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis primarily affecting the respiratory tract and kidneys. Cardiovascular involvement, particularly valvular disease, is rare, especially in pediatric patients. We report a case of a 12‐year‐old boy newly diagnosed with granulomatosis with polyangiitis, who developed an aortic valve perforation. The patient presented with systemic inflammatory symptoms and was found to have aortic regurgitation secondary to aortic valve perforation. Laboratory findings confirmed markedly elevated c‐ANCA titers. Imaging revealed pulmonary involvement, and echocardiography confirmed a perforation in the noncoronary cusp of the aortic valve. Immunosuppressive therapy led to clinical improvement and stabilization of cardiac function, without the need for immediate surgical intervention. This case highlights the importance of considering vasculitis in pediatric patients with systemic inflammation and unexplained cardiac murmurs. Early recognition and prompt immunosuppressive treatment can prevent severe complications.
Svoboda et al. (2026) studied this question.