Late outcomes in adults with XXXX .

@entity1 @entity449 @entity101 @entity1498 @entity439 @entity299 @entity204
AIMS: Previous cohort studies of @entity1 with @entity1498 ( @entity1498 ) demonstrate reduced long-term survival. Improved surgical outcomes in @entity1 and evolution of @entity299 ( @entity299 ) services have resulted in improved survival in @entity1 with other CHDs. We hypothesise that for young adult @entity1 with @entity1498 long-term outcomes have improved in the contemporary era. METHODS: 151 @entity1 (58% @entity1 ) with simple @entity1498 followed up at a tertiary @entity299 service in Sydney, Australia, from 1993 to 2013 were included. We documented mortality and major morbidity such as the need for re-intervention for re- @entity1498 or @entity439 . RESULTS: 140 @entity1 (mean age 35  15   years) underwent @entity1498 repair at median age of 5 (IQR 0-10) years. Initial surgical strategy included end-to-end repair in 43, subclavian flap aortoplasty in 28 and patch aortoplasty in 31 @entity1 (and was not documented in 28 cases). 6 @entity1 had endovascular repair, 4 had interposition tube grafts and 11 were unrepaired. There were a total of seven @entity204 at a median age of 60   years. Actuarial survival was 98% at 40, 98% at 50 and 89% at 60   years of age. @entity1498 occurred in 34% and descending @entity449 were noted in 18%. @entity1 with end-to-end repair had lower rates of significant re- @entity1498 or descending @entity449 (p=0.026 and <0.001, respectively). 66% had bicuspid aortic valve and 44% were @entity101 . CONCLUSIONS: @entity1 with @entity1498 who reach adolescence demonstrate very good long-term survival up to age 60   years. Long-term morbidity is common, however, related largely to aortic complications and late @entity101 .
