The Fate of the Truncal Valve: Multi-Institutional Insights Into Reintervention After Truncus Arteriosus Repair

As survival improves for patients undergoing truncus arteriosus (TA) repair, there remains a limited understanding of long-term outcomes and the need for truncal valve reintervention. This study aimed to elucidate the incidence and outcomes of truncal valve interventions after TA repair.

A retrospective review was performed of the Pediatric Health Information System (PHIS) database from January, 2004 to December, 2023. All patients with a diagnosis of TA who underwent complete TA repair and were admitted within their first week of life were included.

A total of 1079 patients underwent complete TA repair, with 538 (50%) female, 561 (52%) Non-Hispanic White, 184 (17%) premature, 361 (33%) with DiGeorge syndrome, and with a median age of 7.0 days (interquartile range, 5.0-11.0 days) at TA repair. After TA repair, 98 (9%) required truncal valve reintervention, with 61 (6%) patients undergoing repair and 37 (3%) undergoing replacements. Median follow-up was 3.7 years (interquartile range, 0.5-9.3 years). Freedom from any truncal valve reintervention was 98.3% (95% CI, 97.4%-99.2%) at 1 year and 76.3% (95% CI, 70.9%-82.1%) at 15 years. In multivariable Cox regression analysis, older age at TA repair was associated with a decreased risk of any reintervention (hazard ratio, 0.96; 95% CI, 0.93-0.99; P = .017), and a diagnosis of DiGeorge syndrome was protective (hazard ratio, 0.50; 95% CI, 0.30-0.81; P = .004). Freedom from truncal valve replacement was 99.0% (95% CI, 98.3%-99.7%) at 1 year and 89.9% (95% CI, 85.6%-94.5%) at 15 years.

Truncal valve reintervention after TA repair is not uncommon. Subsequent interventions for the truncal valve often follow initial reinterventions, thus highlighting the chronicity of the condition. These findings can help counsel patients and their families and help guide surveillance protocols and long-term management in this population.