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(Circulation. 1999;100:II-157.)
© 1999 American Heart Association, Inc.

Surgery for Congenital Heart Disease

Repair of Tetralogy of Fallot in Neonates and Young Infants

Frank A. Pigula, MD; Philipe N. Khalil, MD; John E. Mayer, MD; Pedro J. del Nido, MD; Richard A. Jonas, MD

From the Department of Cardiac Surgery, Boston Children’s Hospital, Boston, Mass.

Background—The timing of repair of tetralogy of Fallot (TOF) remains controversial. Advantages to early complete repair include removal of right ventricular outflow tract obstruction, alleviation of systemic hypoxia, and avoidance 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. Univariate and multivariate analyses of patient characteristics, anatomic features, 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 year 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 freedom from reoperation than did those repaired for TOF/PS.

Conclusions—Early complete TOF repair can be accomplished with a low mortality. Children with TOF/PA repaired had a lower freedom from reoperation that did those with TOF/PS. Longer follow-up, with emphasis on arrhythmias and right ventricular function, is required to define the long-term benefits of early repair.

Key Words: tetralogy of Fallot • surgery • pediatrics