Repair of tetralogy of Fallot in neonates and young infants

Background-The timing of repair of tetralogy of Fallot (TOF) remains contro versial. Advantages to early complete repair include removal of right ventr icular outflow tract obstruction, alleviation of systemic hypoxia, and avoi dance of palliation with an arteriopulmonary shunt. Methods and Results-This is a retrospective review of 99 children with TOF pulmonary stenosis (TOF/PS) or TOF pulmonary atresia (TOF/PA) who were <90 days of age undergoing early complete repair. Fifty-nine were prostaglandin E dependent, and 91% of neonates were symptomatic at the time of repair. U nivariate and multivariate analyses of patient characteristics, anatomic fe atures, and operative management showed the diagnosis of TOF/PA and smaller body surface area to be the only independent risk factors for death. Early mortality was 3% (3 of 99), and actuarial survival rates were 94% at 1 yea r and 91.6% at 5 years. Freedom from catheterization was 86% at 1 year and 73% at 5 years. Patients repaired for TOF/PA had a significantly lower free dom from reoperation than did those repaired for TOF/PS. Conclusions-Early complete TOF repair can be accomplished with a low mortal ity. Children with TOF/PA repaired had a lower freedom from reoperation tha t did those with TOF/PS. Longer follow-up, with emphasis on arrhythmias and right ventricular function, is required to define the long-term benefits o f early repair.