Background Long-term survival in patients with truncus arteriosus is favorable, but there remains significant morbidity associated with ongoing reinterventions. We aimed to study the long-term outcomes of the truncal valve and identify risk factors associated with truncal valve intervention. Methods and Results We retrospectively reviewed patients who underwent initial truncus arteriosus repair at our institution from 1985 to 2016. Analysis was performed on the 148 patients who were discharged from the hospital and survived ≥30 days postoperatively using multivariable competing risks Cox regression modeling. Median follow-up time was 12.6 years (interquartile range, 5.0-22.1 years) after discharge from full repair. Thirty patients (20%) underwent at least one intervention on the truncal valve during follow-up. Survival at 1, 10, and 20 years was 93.1%, 87.0%, and 80.9%, respectively. The cumulative incidence of any truncal valve intervention by 20 years was 25.6%. Independent risk factors for truncal valve intervention included moderate or greater truncal valve regurgitation (hazard ratio [HR], 4.77; P<0.001) or stenosis (HR, 4.12; P<0.001) before full truncus arteriosus repair and moderate or greater truncal valve regurgitation at discharge after full repair (HR, 8.60; P<0.001). During follow-up, 33 of 134 patients (25%) progressed to moderate or greater truncal valve regurgitation. A larger truncal valve root z-score before truncus arteriosus full repair and during follow-up was associated with worsening truncal valve regurgitation. Conclusions Long-term rates of truncal valve intervention are significant. At least moderate initial truncal valve stenosis and initial or residual regurgitation are independent risk factors associated with truncal valve intervention. Larger truncal valve root z-score is associated with significant truncal valve regurgitation and may identify a subset of patients at risk for truncal valve dysfunction over time.
Keywords: congenital heart disease; truncal valve; truncus arteriosus.