Complete Heart-Block And Severe Aortic Incompetence In Relapsing Polychondritis - Clinicopathological Findings

We describe a patient with relapsing polychondritis who developed fatal cardiac involvement comprising complete heart block, acute aortic incompetence, and cardiovascular collapse. Pathologic studies showed fibrosis of the cardiac conducting system and necrotizing inflammation of the aortic valve, features not previously described in relapsing polychondritis, as well as evidence of coronary artery vasculitis.