Tetralogy of Fallot: surgical management individualized to the patient

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.