We present the case of a 63-year-old man suffering from dyspnoea on exertion, NYHA III. On TOE, we found bicuspid aortic valve disease with severe stenosis - gradient 82/ 51 mmHg, AVA 0.6 cm2, AVAi 0.3 cm2/m2, aortic regurgitation 2-3/4, EF 60%.
Initially, the patient was referred for surgical aortic valve replacement, but the procedure was not performed because of the thickening and infiltration of aortic wall and pericardium. Further examination verified ANCA positive autoimmune inflammation of the aorta.
At the time of ongoing investigations, the patient got progressively worse with dyspnoea and had decrease of EF to 30%. His valve disease had to be treated immediately.