Outcomes following repair of truncus arteriosus type A3.

OBJECTIVES

The study objectives were to describe the outcomes after repair of truncus arteriosus Van Praagh type A3, the rarest subtype of truncus arteriosus.

METHODS

This was a single-center, retrospective cohort study of patients with truncus arteriosus type A3 undergoing repair between 1984 and 2023.

RESULTS

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.

CONCLUSIONS

Repair of truncus arteriosus type A3 can be performed with acceptable operative mortality, although reinterventions, especially on the pulmonary arteries, are frequent.