The long-term result of total repair for tetralogy of Fallot.

OBJECTIVE The objective of this study was to evaluate the long-term outcome of total repair for tetralogy of Fallot. METHODS Between April 1986 and December 2007, a total of 734 patients underwent total repair for tetralogy of Fallot. There were 444 males and 290 females. The median age and weight were 17.2 months (0.4-329.6 months) and 9.5kg (2.6-53.5kg). The median follow-up duration was 150.2 months (1.9-356.2 months). RESULTS There were 27 early deaths (3.7%) and 13 late deaths. A longer cardiopulmonary bypass time and the use of total circulatory arrest were risk factors for early death. The overall survival rate was 94.8%, 92.8% and 92.8% at 10, 20 and 25 years, respectively. The presence of pulmonary atresia was a risk factor for long-term survival. Re-operation or re-intervention was required in 224 patients (31.7%). The most common causes of re-operation or re-intervention were pulmonary regurgitation in 109 patients and branch pulmonary artery stenosis in 127 patients. Freedom from re-operation or re-intervention rate was 81.5%, 68.9% and 46.6% at 5, 10 and 20 years, respectively. Reconstruction of the right ventricular outflow tract with other than non-trans-annular repair and branch pulmonary arterioplasty at the time of total repair were the risk factors for late re-operation or re-intervention. The use of a monocusp patch was not associated with early mortality or re-operation. At the latest follow-up, most patients were in the New York Heart Association functional class 1 or 2. CONCLUSIONS The long-term outcome of total repair for tetralogy of Fallot was satisfactory. A longer cardiopulmonary bypass time and the use of deep hypothermic circulatory arrest were associated with early mortality. The patients with pulmonary atresia have poorer late survival. Preservation of the pulmonary annulus can reduce the re-operation rate. A small pulmonary artery that requires augmentation may increase the risk of re-operation. The use of a monocusp in patients who underwent trans-annular repair has no benefit for early survival, the postoperative recovery and avoidance of re-operation. Age was not a risk factor for early mortality and re-operation.