Outcomes of truncus arteriosus repair: 40 years of experience at a single institution

Objectives: . To investigate the long-term outcomes of truncus arteriosus repair, focusing on the impact of associated truncal valve insufficiency and arch obstruction. Methods: . Sixty-five patients underwent surgery at our institution between 1981 and 2021. Twenty patients had truncal valve insufficiency of at least moderate severity and 22 had arch obstruction. Thirty patients underwent primary repair and 35 underwent staged repair. The median follow-up duration was 10.1 years (range, 0–33.5). Perioperative and follow-up data were analyzed retrospectively. Results: . There were six deaths before definitive repair, four of which were associated with extracardiac syndromes. There were seven deaths after definitive repair, with a 10-year overall survival rate of 81%. When patients with extracardiac syndromes other than 22q11 deletion were excluded, the 10-year survival rate improved from 20% in 1981–2000 to 92% in 2001–2021 in patients with moderate or severe truncal valve insufficiency ( p = .002) and from 50% to 100% in those with arch obstruction ( p = .004). The 10-year survival rate in patients with moderate or severe truncal valve insufficiency and/or arch obstruction was higher after staged repair than after primary repair (88% vs 57%, p = .057). The 10-year freedom from reoperation rate other than for the right ventricular outflow tract was 63%. Conclusions: . Truncus arteriosus can now be treated with a low mortality risk. The presence of truncal valve insufficiency or arch obstruction was associated with increased mortality in the early years but the mortality rate improved in later years. Institutional Review Board Approval: The study was approved by our institutional review board (25 November 2022; reference number 2022-110).