Genetics, Pregnancy, and XXXX .

@entity172 @entity1 @entity2362 @entity1580 @entity615 @entity2334 @entity439 @entity1498 @entity74 @entity2333 @entity5400 @entity528
UNASSIGNED: We present a case of @entity2334 and dissection (FTAAD) in a pregnant female. FTAAD is an @entity74 resulting from several genetic mutations critical to aortic wall integrity have been identified. One such mutation is the myosin heavy chain gene ( @entity5400 ) which is responsible for 1-2% of all FTAAD cases. This mutation results in aortic @entity528 , loss of @entity2362 , and reticulin fiber fragmentation predisposing to TAAD. @entity2333 is more aggressive during pregnancy as a result of increased wall stress from hyperdynamic cardiovascular changes and @entity172 -induced aortic media degeneration. Our @entity1 was a 29-year-old G2P1 @entity1 at 26  weeks gestation presenting with @entity615 . Work-up revealed a 6.4-cm ascending aortic @entity439 with a type A dissection extending into all arch vessels, @entity1498 at the isthmus, and a separate focal type B @entity1580 with visceral involvement. Surgical management included concomitant cesarean section with delivery of a live premature @entity1 , tubal ligation, ascending aortic replacement with reconstruction of the arch vessels, and aortic valve resuspension. The type B dissection was managed medically without complication. This is the first reported case of @entity1580 in a @entity1 with FTAAD/ @entity5400 mutation and pregnancy. This case highlights that FTAAD and pregnancy cause @entity528 via distinct mechanisms and that hyperdynamics of pregnancy increase aortic wall stress. Management of pregnancy associated with aortopathy requires early transfer to a tertiary center, careful investigation to identify familial aortopathy, fetal monitoring, and a multidisciplinary team approach.
