Outcomes following repair of truncus arteriosus with interrupted aortic arch.

OBJECTIVE

To describe the long-term outcomes and burden of reintervention in patients undergoing neonatal repair of truncus arteriosus with interrupted aortic arch, a rare lesion associated with a high risk of mortality.

METHODS

Single-center retrospective cohort study of 33 patients with truncus arteriosus with interrupted aortic arch who underwent repair between 1987 and 2023. Survival was estimated using the Kaplan-Meier method. Freedom from reintervention was assessed using the Fine-Gray method, with death as a competing risk.

RESULTS

Thirty-one patients (94%) underwent single-stage repair. Twenty-five patients (76%) underwent neonatal repair. The median age at repair was 6 days (interquartile range [IQR], 4-10 days). Moderate or greater truncal valve insufficiency was present in 10 patients (30%). Aortic arch reconstruction was performed with patch augmentation in 16 patients (48%), direct end-to-side anastomosis in 11 patients (33%), and reverse subclavian flap in 5 patients (15%). Concomitant truncal valve surgery was performed in 8 patients (25%). Two patients (6%) died prior to discharge. The median duration of follow-up was 10.8 years (IQR, 3.2-17.8 years). Freedom from death or transplantation at 20 years was 87% (95% confidence interval [CI], 70-95%). On competing risks analysis, the 10-year risk of reoperation was 58% (95% CI, 41-76%) for the right ventricle (RV)-pulmonary artery (PA) conduit, 15% (95% CI, 3-28%) for the truncal valve, and 9% (95% CI, 0-21%) for the aortic arch.

CONCLUSIONS

Single-stage neonatal repair of truncus arteriosus with interrupted aortic arch can be performed with acceptable risk and excellent long-term prognosis. Unlike reoperation for RV-PA conduit stenosis, reoperation for recurrent arch obstruction and truncal valve insufficiency are relatively uncommon.