Surgical treatment of XXXX enrolled in the national registry of genetically triggered thoracic aortic conditions.

@entity1 @entity1614 @entity2334 @entity439 @entity74 @entity340 @entity65
BACKGROUND: @entity74 are an important cause of @entity2334 ( @entity2334 ) in young @entity1 . Despite advances in the treatment of genetically triggered @entity2334 , the optimal syndrome-specific treatment approach remains undefined. We used data from the National Institutes of Health-funded, multicenter National Registry of Genetically Triggered @entity2334 and @entity65 (GenTAC) to characterize the contemporary surgical treatment of @entity1 with genetically triggered @entity2334 . METHODS: GenTAC's aim is to collect longitudinal clinical data and banked biospecimens from 2800 @entity1 with genetically triggered @entity2334 . We analyzed data from the 606 @entity1 (mean age, 37.5 years) enrolled in GenTAC to date whose clinical data were available. RESULTS: The @entity1 ' primary diagnoses included @entity1614 (35.8%), bicuspid aortic valve with @entity439 (29.2%), and familial @entity2334 and dissections (10.7%). Of these, 56.4% had undergone at least one operation; the most common indications were @entity439 (85.7%), @entity340 (65.8%), and dissection (25.4%). Surgical procedures included replacement of the aortic root (50.6%), ascending aorta (64.8%), aortic arch (27.9%), and descending or thoracoabdominal aorta (12.4%). Syndrome-specific differences in age, indications for operation, and procedure type were identified. CONCLUSIONS: @entity1 with genetically transmitted @entity2334 evaluated in tertiary care centers frequently undergo surgical repair. @entity439 repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like @entity2334 themselves, complications of @entity2334 , including dissection and aortic @entity340 , are important indications for intervention. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of @entity1 with @entity2334 .
