Short-to-long-term outcomes following truncus arteriosus repair: reintervention burden and risk factor analysis from a tertiary center

Abstract

Background
Truncus arteriosus (TA) is a rare congenital heart defect requiring early surgical repair. Most patients require subsequent catheter-based or surgical reintervention during follow-up. This study aimed to describe short- to long-term outcomes following primary TA repair, characterize the burden of reintervention, and identify risk factors associated with surgical and catheter-based reintervention. A single-center retrospective review was conducted of all patients who underwent TA repair between 2010 and 2023. Kaplan–Meier analysis, Firth-penalized Cox regression, Fine–Gray competing risk analysis, and Andersen–Gill modeling for recurrent events were performed.


Results

Thirty-four patients were included (median age 4.0 weeks; median weight 3.0 kg). Type 1 TA was the most common subtype (73.5%). Thirty-day mortality was 11.8%, and overall mortality was 17.6%. Sixteen patients (47.1%) underwent surgical reintervention, predominantly right ventricle-pulmonary artery (RV-PA) conduit replacement (87.5%). Freedom from surgical reintervention was 50.4% at 5 years. Truncal valve stenosis (HR 4.91,
p
 = 0.022), concomitant pulmonary artery plasty (HR 4.52,
p
 = 0.005), and concomitant truncal valve replacement (HR 4.85,
p
 = 0.024) were significantly associated with surgical reintervention. Seventeen patients (50.0%) required catheter-based reintervention, with 10 requiring repeated procedures (total: 33 procedures). Confluent hypoplastic Pulmonary Artery (PA) anatomy was associated with catheter-based reintervention (HR 3.61,
p
 = 0.021). Competing risk analysis yielded slightly lower cumulative incidence estimates for reintervention when death was treated as a competing event.



Conclusions
TA repair is feasible with acceptable early mortality; however, reinterventions are frequent, principally related to RV-PA conduit dysfunction and branch pulmonary artery stenosis. Truncal valve stenosis and the need for concomitant procedures at index repair identify patients at higher risk for earlier surgical reintervention. Long-term surveillance and a low threshold for catheter-based intervention are essential in this population.