Objectives: The natural history of tetralogy of Fallot (TOF) allows that a minority of patients reach adulthood without any treatment, representing mild forms of the disease. The aim of this study is the long-term evaluation of patients with TOF surgically treated in adulthood, in order to define its real benefit.
Methods: Between November 1982 and January 2001, 39 patients older than 18 years of age with tetralogy of Fallot underwent total correction. Mean age was 26.6 years (range 18-67) and 21 patients (53.8%) were females. A previous modified Blalock-Taussig shunt was performed in four patients (10.3%). Fifteen patients (38.5%) were in NYHA functional class III or IV. Mean hematocrit was 53.6+/-10% and the mean gradient across the right-ventricular outflow tract was 93.9+/-24.8 mmHg. The operation was performed via transatrial/transpulmonary approach in 16 patients (41%) and six patients (15.4%) required transannular patch. Pulmonary valvotomy was necessary in 13 patients (33.3%) and pulmonary valve replacement with bioprosthesis in 3 patients (7.7%).
Results: Hospital and late mortality were 5.1 and 7.7%, respectively. The mean follow-up was 45.1 months (range 1-194 months). Actuarial survival was 91.2+/-4.9%, 85.5+/-7.2% and 68.4+/-16.3% at 3, 7 and 15 years, respectively. In the latest follow-up, 27 (79.4%) of the survivals are presently in NYHA functional class I (P<0.001). Echocardiography has shown moderate/severe pulmonary insufficiency in 9 patients (26.5%), moderate pulmonary stenosis in 3 patients (8.8%) and residual ventricular septal defect in 4 patients (11.8%). Arrhythmias were identified in 38.9% of patients with symptoms suspicious of rhythm disturbances. There was impairment of right-ventricular function in 13 patients (38.2%). Three patients were reoperated on to close residual ventricular septal defects in two patients and for pulmonary valve replacement in one patient.
Conclusions: The overall survival of surgically treated adult patients with TOF is acceptable. The great benefit of the complete repair at this age is the functional improvement. On the other hand, late complications closely related to chronic hypoxia, such as arrhythmia and ventricular dysfunction might direct for a more careful follow-up after the surgical correction.