Long-term results after early primary repair of tetralogy of Fallot

Objective: Early primary repair of tetralogy of Fallot has been routinely p erformed. at Children's Hospital, Boston, since 1972. We evaluated the long -term outcome of this treatment strategy including the influence of a trans annular patch. Methods: Fifty-seven patients less than 24 months of age (median 8 months) underwent primary repair of tetralogy of Fallot between January 1972 and De cember 1977. Thirty-one patients had a transannular patch. Survival and fre edom from reintervention were determined by the Kaplan-Meier method with 95 % confidence intervals. Results: There were 8 early deaths, and 1 patient died 24 years after initi al repair. Recent follow-up was obtained for 45 of the 49 long-term survivo rs (92%). Median follow-up was 23.5 years. Ten patients underwent reinterve ntion, 8 of whom underwent relief of right ventricular outflow tract obstru ction. Right ventricular outflow tract obstruction occurred in 6 patients w ithout a transannular patch and 2 with a transannular patch (33% vs 6%, P = .04). One pulmonary valve replacement was performed at another institution 20 years after the repair. Forty-one long-term survivors were in New York Heart Association class I and 4 were in class II. Actuarial survival was 86 % at 20 years (95% confidence intervals = 80%-92%). Freedom from reinterven tion was 93% at 5 years (95% confidence intervals = 87%-99%)and 79% at 20 y ears (95% confidence intervals = 70%-86%). No significant differences were found between patients with and without a transannular patch (survival, P = .34; freedom from reintervention, P = .09, log-rank tests). Conclusions: Long-term survival is excellent and the freedom from,reinterve ntion is satisfactory after early primary repair of tetralogy of Fallot in the 1970s. Use of a transannular patch does not reduce late survival and is associated with a lower incidence of right ventricular outflow tract obstr uction.