Aortic valvulitis complicating Wegener's granulomatosis

In a case of Wegener's granulomatosis aortic valve replacement was performed for worsening congestive cardiac failure secondary to aortic incompetence. Two paravalvular lesions and an isolated intraleaflet deficiency of the non-coronary cusp were identified at operation. Histological changes were consistent with a connective tissue disease.