LONG-TERM SURVIVAL IN PATIENTS WITH REPAIR OF TETRALOGY OF FALLOT - 36-YEAR FOLLOW-UP OF 490 SURVIVORS OF THE FIRST YEAR AFTER SURGICAL REPAIR

Objectives. We sought to analyze risk factors for long-term survival ( up to 36 years) after surgical repair of tetralogy of Fallot (TOF). Ba ckground. Survival after repair is excellent, but data >20 years are r are, Methods. From 1955 to 1977, 658 patients underwent correction of TOF at our institution and were analyzed for survival. Of this patient group (age 12.2 +/- 5.6 gears [mean +/- SD], range 2 to 67), 39.7% ha d a previous palliation, Operative (n = 139) and 1-year (n = 29) death s were excluded for long-term calculations, resulting in a study group of 490 patients. Results. Actuarial 10-, 20, 30- and 36-year survival rates were 97%, 94%, 89% and 85%, respectively, Mortality increased 2 5 sears postoperatively from 0.24%/year to 0.94%/year (p = 0.003), The most common cause of death was sudden death (n = 13), followed by con gestive heart failure (n = 6), Multivariate correlates of impaired lon g-term survival were date of operation (before 1970, p = 0.0104), preo perative polycythemia (p = 0.0487) and use of a right ventricular (RV) outflow patch (p = 0.0079), Postoperative systolic RV/left ventricula r pressure ratio and age showed no influence, Patients without preoper ative polycythemia and an RV outflow patch (n = 164) had a 36-year act uarial survival rate of 96% and normal life expectancy. Conclusions. C yanosis, operative experience of the surgeon and an RV outflow tract p atch influence long-term outcome after repair of TOF in older children , Early repair by experienced surgeons to avoid polycythemia and exces sive RV hypertrophy is supported by this study, However, mortality ris k increases 25 years postoperatively, and thus heart monitoring should be intensified. (C) 1997 by the American College of Cardiology.