Perioperative management of tetralogy of Fallot with absent pulmonary valve

Background: Tetralogy of Fallot with absent pulmonary valve is a rare form of congenital heart disease. The records of patients with this lesion were reviewed over a 61/2 year period, and the perioperative management of 13 patients is described. Methods: We found that our patients could be divided into two groups by age. The younger group failed medical management and was referred for surgery at less than 1 year of age. This group of patients had considerable respiratory disease at the time of surgery, greater ventilatory problems during surgery and prolonged recovery. Most patients in this group have residual respiratory disease. Results: There were significant differences in postoperative outcome between younger versus older patients: days of ventilation 37 +/- 41 versus 1 +/- 0.6 days, length of ICU stay 28 +/- 31 versus 3 +/- 1 days and length of hospital stay 64 +/- 48 versus 9 +/- 5 days.