Once considered a uniformly fatal condition, the outlook for newborns with @entity1699 has been dramatically altered with staged reconstructive procedures. Refinements in operative technique and perioperative management have been largely responsible for this improved outlook. At the University of Michigan, 253 @entity1 underwent the Norwood operation for classic @entity1699 between January 1990 and November 1997. Hospital survival was 76%. Among @entity1 considered at standard risk, survival was significantly higher (86%) than that for those @entity1 with important risk factors (42%, p = 0.0001). Adverse survival was most strongly associated with significant associated noncardiac congenital conditions (p = 0.008) and severe @entity3885 (p = 0.03). Survival following second-stage reconstruction with a hemi-Fontan or bidirectional Glenn procedure was 97%. The Fontan procedure has been completed in 94 of these @entity1 with a hospital survival rate of 88%. Survival after the Fontan procedure improved significantly when the second stage of the reconstruction was completed with a hemi-Fontan procedure compared to a bidirectional Glenn (98% vs 81%, p = 0.05). Among the @entity1 considered at standard risk, actuarial survival was 70% at 5 years. The largest decrease in survival occurred in the first month of life and late deaths affected primarily those @entity1 in the high-risk group. Neurodevelopmental outcome studies demonstrated normal verbal and performance scores in the majority of @entity1 . Staged reconstruction has significantly improved the intermediate-term outlook for @entity1 with @entity1699 . Factors addressing improvements in early first-stage survival would be expected to add significantly to an overall improved late outcome.

Current status of staged reconstruction for XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity1699 :: ('MESH:D018636', 'Disease') :: ['hypoplastic left heart syndrome'] @entity3885 :: ('MESH:D008173', 'Disease') :: ['preoperative obstruction to pulmonary venous return']
@entity1699 :: (MESH:D018636,Disease) :: ['hypoplastic left heart syndrome']
