Long-term follow-up of atrioventricular valve function in Fontan patients : effect of atrioventricular valve surgery

Objective To evaluate the relationship between atrio-ventricular valve and ventricular function in Fontan survivors, including the effect of atrio-ventricular valve surgery. Methods Analysis focused on transplant-free survival and need for atrio-ventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed. Results Fontan completion was performed in 113 patients, having a right univentricular morphology in 33.6%, a left ventricle-morphology in 62.8% and ambiguous in 3.6%. Peri-operative mortality was 2.7%(n = 3). Within a median follow-up of 16.3y(IQR 10.6 - 23.6), transplant-free survival was 96.1±1.9% and 90.4±5.8% at 10-25y. Twenty AV-valve procedures were performed in 14(12.4%) children, respectively pre-Fontan(n = 10), per-Fontan(n = 8), and post-Fontan(n = 2), resulting in a cumulative incidence of AV-valve surgery is 5.7±2.2% and 12.3±3.2% at 1-5y Atrio-ventricular valve function deteriorated over time(HR 1.112, 95%CI 1.089-1.138, p < 0.001), without difference for valve morphology(p = 0.736) or ventricular dominance(p = 0.484). AV-valve dysfunction was greater in patients requiring AV-valve surgery(HR 20.383, 95%CI 6.223-36.762, p < 0.001), but showed a comparable evolution since repair to those without valve surgery(HR 1.070, 95%CI 0.987-1.160, p = 0.099). Progressive time-related ventricular dysfunction was observed(HR 1.141, 95%CI 1.097–1.182, p < 0.001), significantly less in left ventricle-dominance(HR 0.927, 95%CI 0.860-0.999, p = 0.047) but more after AV-valve surgery(HR 1.103, 95% CI 1.014–1.167, p = 0.022). Conclusion In a homogeneously treated Fontan population, 25-year transplant-free survival is encouraging. Atrio-ventricular valve surgery was necessary in 12.4%, resulting mostly in a durable valve function. However, a slow time-related decline of atrio-ventricular valve function as of ventricular function is worrisome, evoking a role for additional heart failure therapy.

Publication year:2023

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