Granulomatosis polyangiitis of the heart—an unusual presentation: a case report and review of literature

Granulomatosis with polyangiitis (GPA) is a multi-system disorder associated with ocular, renal, cardiac, pulmonary involvement. However cardiac involvement is very rare compared to other systems. We present a case of an unusual presentation of GPA associated with ocular involvement super imposed on pre-existing rheumatic valvular disease. The cardiac involvement manifested as a thick fibro elastic membrane encasing the aortic and mitral valve annuli making the valve replacements a real technical challenge. The patient needed permanent pacemaker implantation due to intermittent complete heart block that was present even preoperatively. The patient was started on steroids and is doing well until the last follow-up. This case highlights the importance of recognition of GPA in cardiac surgery and to be aware of such an entity when one encounters a thick whitish avascular fibro elastic membrane encasing cardiac valves and endocardium, obliterating the anatomical delineation of structures during surgery.