Unifocalization and pulmonary artery reconstruction in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery before referral

Objective: The study objective was to characterize and analyze outcomes in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who had undergone surgery elsewhere before referral (prereferral surgery).

Methods: Patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery between 2001 and 2019 at our center were reviewed. Prereferral surgery and unoperated patients were compared, as were subsets of prereferral surgery patients who had undergone different types of prior procedures. Primary outcomes included complete repair with survival to 6 months, death, and perioperative metrics.

Results: Of 576 patients studied, 200 (35%) had undergone a wide range and number of prior operations elsewhere, including 92 who had pulmonary blood supply through a shunt and 108 who had a right ventricle pulmonary artery connection. Patients who underwent prereferral surgery with an existing right ventricle pulmonary artery connection had undergone more prereferral surgery procedures than those with a shunt and were more likely to have a right ventricle outflow tract pseudoaneurysm or pulmonary artery stent (all P < .001) at the time of referral. The cumulative incidences of complete repair and death were similar regardless of prereferral surgery status, but the cumulative incidence of complete repair with 6-month survival was higher (P = .002) and of death lower (P = .18) in patients who had prior right ventricle pulmonary artery connection compared with those who had received a prior shunt only.

Conclusions: Our comprehensive management strategy for tetralogy of Fallot and major aortopulmonary collateral arteries can be applied with excellent procedural results in both unoperated patients and those who have undergone multiple and varied procedures elsewhere.