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 symptoms, with some surgeons advocating hypothermic
circulatory arrest for repair in small infants. We believe this approach increases
morbidity.
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, ductal dependent pulmonary circulation) weighing less
than 4 kg undergo palliative modified Blalock-Taussig shunt (BTS) followed by complete
repair at 6 to 12 months. Asymptomatic patients, weighing less than 4 kg who have
threatened pulmonary artery isolation, undergo BTS and repair at 6 to 12 months. All
other patients undergo complete repair after 6 months.
From July 1, 1995, to December 1, 1999, 144 patients underwent operation for TOF (129
patients) or TOF with atrioventricular septal defect (TOF/AVSD, 15 patients). Ninety-four
patients underwent one stage complete repair (88 TOF, 6 TOF/AVSD). Thirty-nine patients
underwent repair after initial 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 were 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 reoperation after
total correction.
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.