Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve

Objective: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20–60%). We aim to report our results and identify factors associated with survival and prolonged ventilation. Methods: We performed a retrospective review of 62 consecutive patients following repair of TOF/APV (1982–2006). Median age at repair was 1.4 years (1 day–35 years). Twenty patients required preoperative intubation. Results: Sixty-one patients underwent complete repair. Thirty-three patients underwent pulmonary artery plication (n = 15) or reduction (n = 18). The right ventricular outflow tract (RVOT) was reconstructed with valved conduit (n = 31), bioprosthetic valve (n = 18), monocusp (n = 8), or transannular patch (n = 4). There were three perioperative and five late deaths. All perioperative deaths were in neonates and before 1995. Five- and ten-year survival was 93 ± 4% and 87 ± 5%. Mean ventilatory requirements for neonates, infants, and children ≥1 year were 36 ± 35, 8 ± 8, and 2.6 ± 2.4 days (p ≪ 0.0001). On multivariable analysis, significant factors associated with prolonged ventilation were neonates (p ≪ 0.0001) and preoperative mechanical ventilation (p = 0.088). Eight airway reinterventions were needed in seven infants with persistent postoperative airway compromise, pulmonary artery suspension (n = 4), innominate artery suspension (n = 2), and lobectomy (n = 2). Freedom from RVOT reoperation was 89 ± 5% and 59 ± 9% at 5 and 10 years. There were no significant risk factors for time-related survival or RVOT reoperation on multivariable analysis. Conclusions: In contrast to children and adults with TOF/APV, neonates and small infants presenting with respiratory symptoms require prolonged ventilation and additional reinterventions for airway compression. Our current surgical approach which includes reduction and suspension of pulmonary arteries, reconstruction of a competent RVOT, and aggressive postoperative ventilatory management to relieve airway obstruction offers satisfactory outcomes.