Intermediate outcomes after the Fontan procedure in the current era.

OBJECTIVE: This study was undertaken to investigate the intermediate outcomes after the Fontan operation in the current era.

METHODS: A cross-sectional analysis was performed by using data gathered between October 13, 2003, and February 22, 2005, on all 310 survivors of the Fontan procedure between January 1, 1992, and December 31, 1999. Medical records were reviewed, and a customized questionnaire was administered to caregivers. Outcome variables included failure of the Fontan operation (ie, death, takedown of Fontan, or transplantation), number of cardiac-related rehospitalizations, parental assessment of health, school performance, and cardiac functional status.

RESULTS: A total of 332 patients underwent the Fontan procedure during the study period. A lateral tunnel was constructed in 281 (85%), and an extracardiac Fontan procedure was performed in 51 (15%). A total of 210 (63%) children had undergone previous stage I reconstruction, and 162 (49%) had received an initial diagnosis of hypoplastic left heart syndrome. There were 310 hospital survivors of the Fontan procedure (93.4%), with an additional 16 deaths (5.2%) during follow-up and 1 living heart transplant patient before the cross-sectional period. For initial hospital survivors, Kaplan-Meier estimates of freedom from death or transplantation were 98.0% (95% confidence interval, 95.6%-99.1%) at 1 year after the Fontan procedure, 94.9% (91.6%-97.0%) at 5 years, and 93.9% (90.1%-96.2%) at 8 years. Questionnaires were completed for 240 (81.9%) of the 293 available patients. By parental recollection, a total of 130 (54.2%) children required cardiac-related rehospitalization at some point during the follow-up period. At a median follow-up of 8.6 years (range, 4.1-12.8 years), 94.6% of guardians described their child's overall health as excellent or good, and 5.4%, as fair or poor. School performance was described as above average in 30.2%, average in 39.9%, and below average in 29.8%. With regard to cardiac functional status, 34.2% responded that their child had no limitations to physical activity, 52.5% reported a slight limitation, 12.1% reported a significant limitation, and 1.2% reported a severe limitation.

CONCLUSIONS: Acceptable survival outcomes have been observed at intermediate follow-up of the Fontan operation in this cohort; a significant proportion of patients had hypoplastic left heart syndrome. Although cardiac-related rehospitalization is common, the parents and guardians believed that overall health, school performance, and cardiac functional status were good to excellent for most patients.