BACKGROUND: Although systemic @entity1333 has been described as one cause of @entity337 ( @entity337 ), clinical features of this disease entity have not been fully characterized. METHODS: The clinical data of 153 consecutive @entity1 (103 male), who underwent open-heart operation to correct predominant @entity337 from January 1990 to December 2000 were retrospectively analyzed. RESULTS: A total of 16 @entity1 (10%) were found to have @entity337 associated with systemic @entity1333 ; there were 9 @entity1 with @entity12040 ( @entity12040 ) and 7 with @entity195 ( @entity195 ). All @entity1 with @entity12040 , but only 3 with @entity195 , were female (P <.05). Echocardiography showed redundant motion of the elongated aortic cusp with the frequent periaortic echo-free space in @entity195 , and characteristic motion limitation of thickened cusp with dilated and thickened aorta in @entity12040 . All @entity1 with @entity12040 , but only 1 @entity1 with @entity195 , were given a diagnosis before operation (P <.05); the others underwent emergency operation to control acute @entity337 of unknown etiology, and were given a correct diagnosis after detection of @entity2680 . In @entity12040 , @entity2680 developed in 1 @entity1 (11%) and another died suddenly with @entity662 . In @entity195 , @entity2680 developed in 6 @entity1 (86%) who needed repeated operation, and 2 died even after the third operation. The 2-year survival without clinical events ( @entity2680 , repeated operation, or @entity204 ) was 76 +/- 15% in @entity12040 and 14 +/- 13% in @entity195 (P <.05). CONCLUSIONS: @entity12040 and @entity195 are 2 important causes of @entity337 , and show different clinical features. A high rate of @entity2680 , especially in @entity195 , warrants cautious systemic evaluations for the cause of @entity337 , and characteristic echocardiographic findings seem to be helpful in the differential diagnosis.

Echocardiographic and clinical characteristics of @entity337 because of systemic XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity662 :: ('MESH:D002637', 'Disease') :: ['chest pain'] @entity2680 :: ('MESH:D003763', 'Disease') :: ['paravalvular leakage'] @entity1333 :: ('MESH:D014657', 'Disease') :: ['vasculitis'] @entity12040 :: ('MESH:D013625', 'Disease') :: ["Takayasu's arteritis", 'TA'] @entity337 :: ('MESH:D001022', 'Disease') :: ['AR', 'aortic regurgitation'] @entity195 :: ('MESH:D000544', 'Disease') :: ["Beh  et's disease", 'BD'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity1333 :: (MESH:D014657,Disease) :: ['vasculitis']
