Background. Over the past four decades, the surgical trend has been toward
early, complete repair of tetralogy of Fallot (TOF). Many centers currently
promote all neonates for total correction irrespective of anatomy and symp
toms, with some surgeons advocating hypothermic circulatory arrest for repa
ir in small infants. We believe this approach increases morbidity.
Methods. Based on approximately 40 years' experience in 2,175 patients, we
developed a management protocol focused on patient size, systemic arterial
saturations, and anatomy. Symptomatic patients (hypercyanotic spells, ducta
l dependent pulmonary circulation) weighing less than 4 kg undergo palliati
ve modified Blalock-Taussig shunt (BTS) followed by complete repair at 6 to
12 months. Asymptomatic patients, weighing less than 4 kg who have threate
ned pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. A
ll other patients undergo complete repair after 6 months.
Results. From July 1, 1995, to December 1, 1999, 144 patients underwent ope
ration for TOF (129 patients) or TOF with atrioventricular septal defect (T
OF/AVSD, 15 patients). Ninety-four patients underwent one stage complete re
pair (88 TOF, 6 TOF/AVSD). Thirty-nine patients underwent repair after init
ial BTS (32 TOF, 7 TOF/ AVSD). Ten patients are awaiting repair after BTS.
The mean age and weight at complete repair were 18 months and 9 kg. There w
ere no operative deaths. There have been 3 late deaths with complete follow
-up (mortality 3 of 144 [2.1%]). Four of 133 patients (3%) have required re
operation after total correction.
Conclusions. This management strategy optimizes outcomes by individualizing
the operation to the patient. Advantages include avoidance of circulatory
arrest, low morbidity and mortality, and low incidence of reoperation after
complete repair. (Ann Thorac Surg 2001;71:1556-63) (C) 2001 by The Society
of Thoracic Surgeons.