Unexpected late-onset aortic valvulitis and moderate regurgitation during longitudinal evaluation of atypical infantile Kawasaki disease: The heart beyond coronaries!

Kawasaki disease (KD) is the most common pediatric vasculitis with coronary involvement feared as the most serious complication. The reported case describes a child presenting initially with atypical KD and coronary artery aneurysms. He was treated with intravenous immunoglobulin and aspirin. In spite of adequate compliance and no clinical recurrence, serial echocardiography revealed nonregression of aneurysm and new-onset moderate aortic regurgitation (AR) in the subacute phase produced by prolapse of noncoronary cusp of the aortic valve. AR without aortic root dilatation from persistent inflammation of the valve leaflets in KD is a rare phenomenon. This case demonstrates unusual cardiac manifestations of KD and reoriented our protocol for long-term surveillance in infantile KD.