Abstract 20492: Long Term Outcomes After Surgical Repair of Tetralogy of Fallot (TOF): A Study From the Pediatric Cardiac Care Consortium (PCCC)
Introduction: TOF can exist either in simple (without association with other lesions) or in complex form when associated with absent pulmonary valve (TOF-APV), complete atrioventricular canal (TOF-CCAVC) or pulmonary atresia (TOF-PA). Surgical repair is feasible in all forms, but risks for long term outcomes can be different depending on the substrate of the TOF. We examined the long term vital status and risk of transplantation of patients registered in PCCC with repaired TOF.
Methods: We identified 3,841 and 1,878 patients surviving after simple and complex TOF repair between 1982 and 2003. Of these 3,302 and 1,619 respectively had direct identifiers sufficient for submission to the National Death Index (NDI) and United Network for Shared Organs (UNOS) datasets to determine vital and transplant status through 2014. Kaplan Meier (KM) transplant-free survival curves and hazard of mortality were calculated for comparisons between groups.
Results: 275 deaths (8.3%) occurred in simple TOF and 199 (19.0%) in complex TOF during a median follow-up period of 19.4 years (IQR: 14.9-24.4 years). There were 11 heart transplants in simple TOF and 11 transplants (7 heart, 3 heart-lung and 1 lung) for complex TOF. Each complex cohort had a higher hazard of mortality than simple TOF: TOF-APV 3.27 (95% CI: 2.39-4.49, p<0.001), TOF-CCAVC 4.21 (95% CI: 3.14-5.63, p<0.001) and TOF-PA 2.8 (95% CI: 2.37-3.32, p<0.001). Hazard of mortality for those with a genetic defect was 3.13 (95% CI: 2.38-4.12, p<0.001) and 1.82 (95% CI: 1.47-2.25, p<0.001) times higher than for those without identifiable genetic condition for simple and complex TOF respectively.
Conclusions: 20 year transplant-free survival for patients with repaired simple and complex TOF reaches 91.7% and 81% respectively. These results provide a conservative estimate of expected outcomes for these groups of patients. In depth analysis is underway for risk factors and specific diagnoses affecting mortality.
Author Disclosures: C. Smith: None. M. Kelleman: None. C. McCracken: None. A. Kalogeropoulos: None. C. Sung: None. M. Oster: None. L. Spector: None. J. Moller: None. L. Kochilas: Consultant/Advisory Board; Modest; Novartis.
- © 2016 by American Heart Association, Inc.