Long-term outcomes of atrioventricular septal defect and single ventricle: a multicenter study

Arrigoni, S.C.; IJsselhof, R.; Postmus, D.; Vonk, J.M.; Francois, K.; Bove, T.; ... ; Ebels, T. (2022)

Article / Letter to editor

Objective: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. Methods: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. Results: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. Conclusions: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

• Fontan
• single ventricle

• All authors: Arrigoni, S.C.; IJsselhof, R.; Postmus, D.; Vonk, J.M.; Francois, K.; Bove, T.; Hazekamp, M.G.; Rijnberg, F.M.; Meyns, B.; Puyvelde, J. van; Poncelet, A.J.; Beco, G. de; Woestijne, P.C. van de; Bogers, A.J.J.C.; Schoof, P.H.; Ebels, T.
• Date: 2022-03-01
• Journal: The Journal of Thoracic and Cardiovascular Surgery
• Volume: 163
• Issue: 3
• Pages: 1166 - 1175
• DOI: doi:10.1016/j.jtcvs.2021.05.015