Outcomes following repair of truncus arteriosus type A3

<h2>Abstract</h2><h3>Objectives</h3> The study objectives were to describe the outcomes after repair of truncus arteriosus Van Praagh type A3, the rarest subtype of truncus arteriosus. <h3>Methods</h3> This was a single-center, retrospective cohort study of patients with truncus arteriosus type A3 undergoing repair between 1984 and 2023. <h3>Results</h3> Of 19 patients, the contralateral lung (ie, the lung not supplied by the branch pulmonary artery arising from the truncus) was supplied by the patent ductus arteriosus in 6 patients and by at least 1 major aortopulmonary collateral arising from the descending thoracic aorta in 13 patients. Eighteen patients underwent single-stage complete repair, and 12 patients underwent neonatal repair. There was 1 operative mortality from sepsis. There were 3 late deaths: 1 due to cardiac arrest and 2 due to pulmonary vascular occlusive disease. Median duration of follow-up was 4.8 years. Overall survival at 10 years was 76% (95% CI, 48-90). Reinterventions were required in 14 patients. This included catheter-based reintervention on the right ventricle-pulmonary artery conduit (N = 7) or pulmonary arteries (N = 12) and reoperation for right ventricle-pulmonary artery conduit stenosis (N = 9) or truncal valve regurgitation (N = 3). On latest lung perfusion scan, median perfusion to the contralateral lung was 38% (interquartile range, 26-47). On latest cardiac catheterization, median right ventricle to aortic pressure ratio was 0.84 (interquartile range, 0.66-1.06). All patients had New York Heart Association functional class I/II at the latest clinic follow-up. <h3>Conclusions</h3> Repair of truncus arteriosus type A3 can be performed with acceptable operative mortality, although reinterventions, especially on the pulmonary arteries, are frequent.