Abstract 15412: Long-term Outcomes of the Truncal Valve in Truncus Arteriosus

Abstract

Objectives: Long-term survival in patients with truncus arteriosus (TA) is favorable, but there remains significant morbidity associated with need for ongoing re-interventions. The purpose of this study was to understand the long-term outcomes of the truncal valve (TV) and identify risk factors associated with the need for TV intervention. Methods: We retrospectively reviewed 170 patients who underwent initial TA repair at our institution from 1985-2015. Analysis of long-term outcomes was performed on the 148 patients who survived greater than 30 days post-operatively and to hospital discharge using multivariable competing risks Cox regression modeling. Results: Median follow up time was 12.6 years (IQR 5.0, 22.1 years) after full repair. Freedom from death or transplant at 1, 5, 10, and 20 years was 93.1 ± 2.1%, 88.0 ± 2.7%, 86.2 ± 3.0% and 78.3 ± 4.1%. Thirty patients (20%) underwent at least one intervention on the TV (22 repairs, 21 replacements). Freedom from any TV intervention at 1, 5, 10 and 20 years was 99 ± 1%, 94 ± 8%, 82 ± 9%, and 70 ± 5%. Of those with TV repair, 59% subsequently underwent TV replacement. Independent risk factors for need for TV intervention included ≥moderate TV regurgitation (TVR) (HR 4.77, p<0.001) or stenosis (HR 4.12, p<0.001) prior to full repair, and ≥moderate TVR at initial hospital discharge after full repair (HR 8.6, p<0.001). A single coronary ostium was also independently associated with need for TV intervention (HR 6.94, p=0.01). Quadricuspid valve morphology and TV repair at initial TA repair, risk factors in univariate analysis, were not independent predictors on multivariable analysis. Overall, 28% of patients progressed to ≥moderate TVR and to Z-scores of greater than 5 for valve dimensions. Conclusion: Long-term need for TV intervention remains significant. Moderate or worse initial TVR or stenosis, residual TVR after initial TA repair, and single coronary ostium are risk factors associated with need for subsequent TV intervention.