• Sara C Arrigoni, Rinske IJsselhof, Douwe Postmus, Judith M Vonk, Katrien François, Thierry Bové, Mark G Hazekamp, Friso M Rijnberg, Bart Meyns, Joeri van Puyvelde, Alain J Poncelet, Geoffroy de Beco, Pieter C van de Woestijne, BogersAd J J CAJJCDepartment of Cardiothoracic Surgery, University Medical Center Rotterdam, Rotterdam, The Netherlands., Paul H Schoof, and Tjark Ebels.
• Department of Cardiothoracic Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. Electronic address: s.c.arrigoni@umcg.nl.
• J. Thorac. Cardiovasc. Surg. 2022 Mar 1; 163 (3): 1166-1175.

ObjectiveThe study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect.MethodsData 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.ResultsMedian 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 15-year 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.ConclusionsThe 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.Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

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