Risk factors and outcomes of tricuspid valve surgery in hypoplastic left heart syndrome patients with tricuspid regurgitation

Objective The management of HLHS patients with tricuspid regurgitation (TR) remains controversial. We evaluate risk factors for mortality/transplant/Fontan takedown in HLHS patients with TR and the effect of tricuspid valve (TV) surgery on clinical trajectories. Methods Retrospective chart review was performed for all HLHS primary surgical Norwood recipients at our center who had moderate-or-greater TR (2006-2023). Freedom from a primary outcome of combined mortality/transplant/Fontan takedown was compared between those who underwent TV surgery (N=30, 67%) versus those who did not (N=15, 33%; Cox regression, Log-Rank). Among those who developed TR anytime post-Norwood (N=37), TV repair efficacy was assessed (Wilcoxon signed-rank, McNemar's test). Cumulative incidence of recurrent TR and RV dysfunction was estimated (Aalen-Johansen estimator, death/transplant as competing risks). Results Patients with pre-Norwood TR experienced a mortality-dominated trajectory (5-year freedom from mortality 37.5%), while post-Norwood/pre-Glenn TR patients experienced a transplant-dominated trajectory (5-year freedom from mortality 88.5%, composite outcome 55.9%). On univariable Cox regression, pre-Norwood RV dysfunction (HR=9.75, p<0.001) was associated with the primary outcome; pre-Norwood TR tended toward association (HR=2.40, p=0.10). On multivariable analysis, pre-Norwood RV dysfunction (HR=12.79 [3.16, 51.80], p<0.001) and pre-Norwood TR (HR=3.10 [1.03, 9.31], p=0.04) were both independently associated with the primary outcome. Among those with post-Norwood TR who underwent TV surgery, TR severity decreased, with 43% achieving preserved freedom from moderate-or-greater TR. Conclusions Pre-Norwood RV dysfunction and pre-Norwood TR were found to be associated with the primary outcome. In those with post-Norwood TR, TV repair reduced TR but was not associated with improved survival.