Echocardiographic and clinical characteristics of aortic regurgitation because of systemic vasculitis

Although systemic vasculitis has been described as one cause of aortic regurgitation (AR), clinical features of this disease entity have not been fully characterized.

The clinical data of 153 consecutive patients (103 male), who underwent open-heart operation to correct predominant AR from January 1990 to December 2000 were retrospectively analyzed.

A total of 16 patients (10%) were found to have AR associated with systemic vasculitis; there were 9 patients with Takayasu's arteritis (TA) and 7 with Behçet's disease (BD). All patients with TA, but only 3 with BD, were female (P <.05). Echocardiography showed redundant motion of the elongated aortic cusp with the frequent periaortic echo-free space in BD, and characteristic motion limitation of thickened cusp with dilated and thickened aorta in TA. All patients with TA, but only 1 patient with BD, were given a diagnosis before operation (P <.05); the others underwent emergency operation to control acute AR of unknown etiology, and were given a correct diagnosis after detection of paravalvular leakage. In TA, paravalvular leakage developed in 1 patient (11%) and another died suddenly with chest pain. In BD, paravalvular leakage developed in 6 patients (86%) who needed repeated operation, and 2 died even after the third operation. The 2-year survival without clinical events (paravalvular leakage, repeated operation, or death) was 76 +/- 15% in TA and 14 +/- 13% in BD (P <.05).

TA and BD are 2 important causes of AR, and show different clinical features. A high rate of paravalvular leakage, especially in BD, warrants cautious systemic evaluations for the cause of AR, and characteristic echocardiographic findings seem to be helpful in the differential diagnosis.