A 26-year experience with surgical management of tetralogy of Fallot: risk analysis for mortality or late reintervention

Abstract

Background: Over the past decade repair of tetralogy of Fallot (TOF) in infancy has gained favor. It is still uncertain what effect early complete repair will have on survival or late reoperation on the right ventricular outflow tract.

Methods: To assess these outcomes, we reviewed our experience (1971-1997) with 294 patients undergoing operation at one institution. Median follow-up was 10.6 years (range, 0.1 to 26 years), and was complete for 90.2% patients.

Results: Primary complete repair was done in 199 patients (68%), and a staged repair in 62 patients (21%). Thirty-three patients had only a palliative procedure. Sixty-eight patients (23.1%) had complex pathologic processes, including pulmonary atresia in 53. Hospital mortality for primary repair was 11.1% (22/199), for staged repair was 17.7% (11/62), and for palliative procedures was 15.5% (16/103 procedures). Since 1990 mortality has been 2.1%, 11.8%, and 0% respectively (p < 0.001), despite younger age at repair (0.6+/-0.1 versus 2.1+/-0.2 years; p < 0.001). Multivariate analysis identified longer period of hypothermic circulatory arrest, pulmonary artery patch angioplasty, earlier year of operation, and closure of the foramen ovale as risk factors for hospital death. For hospital survivors 20-year survival was 98%+/-3% for TOF with pulmonary stenosis and 88%+/-9% for TOF with pulmonary atresia (p=0.09). Reintervention on the right ventricular outflow tract was needed in 14.1% (37/261) patients. Freedom from reintervention on the right ventricular outflow tract at 20 years was 86%+/-4% for TOF with pulmonary stenosis and 43%+/-16% for TOF with pulmonary atresia (p=0.001). For the subgroup TOF with pulmonary stenosis, this was 85%+/-5% after primary repair and 91%+/-8% after staged repair (not significant). At 15-year follow-up, this was 78%+/-10% for patients not older than 1 year at operation compared with 88%+/-4% for older patients (not significant).

Conclusions: Early mortality after primary repair of TOF has significantly improved and late survival is excellent. Primary repair in infancy does not increase risk for reintervention on the right ventricular outflow tract.