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

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, Ad J.J.C. Bogers, Paul H. Schoof, Tjark Ebels

*Corresponding author for this work

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Abstract

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 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. 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.

Original languageEnglish
Pages (from-to)1166-1175
Number of pages10
JournalJournal of Thoracic and Cardiovascular Surgery
Volume163
Issue number3
Early online date18 May 2021
DOIs
Publication statusPublished - 1 Mar 2022

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