Outcomes of XXXX in the Fontan era.

@entity1145 @entity1 @entity4265 @entity6127
BACKGROUND: Whereas indications expand and results improve with increasing refinements to the Fontan procedure the overall impact on outcomes related to @entity1145 remains suboptimally defined. METHODS: We reviewed 225 consecutive @entity1 presenting between 1971 and 1999. All @entity1 had classic @entity1145 with absent right @entity1145 and with D-transposition of the great arteries in 21%, pulmonary outflow obstruction in 75%, and aortic outflow obstruction in 11%. RESULTS: Ten @entity1 died before any intervention and 3 @entity1 were lost to follow-up. Palliative procedures (includes 151 with systemic shunt, 27 pulmonary artery banding, 60 venous shunt) were performed in 203 @entity1 , with 44 deaths, 8 @entity1 awaiting Fontan, 12 @entity1 Fontan contraindicated, and 11 @entity1 lost-to-follow-up. A total of 137 @entity1 had the Fontan procedure (9 @entity1 without previous procedures) with 7 early deaths, 11 late deaths, and 3 @entity1 progressing to heart transplantation. Total survival for the cohort was 90% at the age of 1 month, 81% at 1 year, 70% at 10 years, and 60% at 20 years with no significant change over the time period. Independent factors associated with ineligibility or death without Fontan (n = 68, 30%) included earlier birth date, lower birth weight, presence of @entity6127 and greater @entity4265 , and no palliative procedure. There were no significant changes in mortality with Fontan over the study time period with survival of 95% at 1 month, 93% at 1year, and 82% at 10 years. CONCLUSIONS: Improvements in outcomes with @entity1145 will require attention to management and risk factors before Fontan.
