Abstract Body (Do not enter title and authors here): BACKGROUND: Total anomalous pulmonary venous return (TAPVR) is a known risk factor for mortality in single ventricle (SV) heterotaxy patients with heterotaxy. Outside of infracardiac or obstructed TAPVR, no consensus exists regarding if/when to repair other subtypes. We sought to determine if delaying repair is associated with worse outcomes.
METHODS: All SV heterotaxy patients with TAPVR who had cardiac surgery from 2005-2023 were included. Demographics, anatomic details, and operative course were analyzed. Parametric risk hazard analysis was performed to model risk of death/heart transplant and identify risk factors. Type of TAPVR repair (upfront (at first operation, isolated or concomitant) or delayed (any subsequent operation, isolated or concomitant)) were analyzed as time-varying covariates.
RESULTS: Among 91 SV heterotaxy patients, 29 (32%) were female and 71 (78%) had right ventricular dominance. All had TAPVR: 38 (42%) supracardiac, 30 (33%) cardiac, 15 (17%) infracardiac, and 7 (8%) mixed. Eighteen (20%) presented with obstruction. Upfront TAPVR repair was done in 51 (56%) patients at median age 3 days (IQR 1-7)). Cardiac TAPVR most commonly underwent non-upfront repair vs. upfront repair (23 (77%) vs. 7 (23%), p<0.001). All infracardiac TAPVR patients received upfront repair (15 (100%)). There were 42 deaths and 1 transplant in the cohort. Of 51 upfront repairs, 28 (54.9%) died during the study period and 1 (2%) was transplanted. Of 40 patients without upfront repair, 6 (15%) eventually had vein repair, while 34 (85%) never had vein repair. Of the non-upfront repairs, 14 (35%) died (2 eventually repaired and 12 never repaired). Fontan was achieved in 41 (45%) patients (23/40 (58%) in non-upfront repair group, and 18/51 (35%) in upfront repair group). (Figure 1)
Multivariable analysis for death/transplant demonstrated that time-varying initial repair of TAPVR (parameter estimate (PE) 1.45±0.47, p=0.002) was an early-phase risk, while AVV repair/replacement (PE 2.62±0.75, p=0.0005) and delayed TAPVR repair (PE 2.0±0.96, p=0.04) were time-varying late-phase risks.
CONCLUSIONS:
The need for early repair of TAPVR portends risk for mortality for children with heterotaxy and single ventricle. If children survive neonatal repair, the diagnosis of TAPVR no longer independently predicts an ongoing risk for mortality. However, if TAPVR is left unrepaired in infancy, it remains an independent risk factor for late mortality whether repair is attempted or not.