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

doi:10.1016/j.jtcvs.2021.05.015

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 language	English
Pages (from-to)	1166-1175
Number of pages	10
Journal	Journal of Thoracic and Cardiovascular Surgery
Volume	163
Issue number	3
Early online date	May 2021
DOIs	https://doi.org/10.1016/j.jtcvs.2021.05.015
Publication status	Published - Mar 2022

Keywords

Fontan
single ventricle

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Arrigoni, S. C., IJsselhof, R., Postmus, D., Vonk, J. M., François, K., Bové, T., Hazekamp, M. G., Rijnberg, F. M., Meyns, B., van Puyvelde, J., Poncelet, A. J., de Beco, G., van de Woestijne, P. C., Bogers, A. J. J. C., Schoof, P. H., & Ebels, T. (2022). Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study. Journal of Thoracic and Cardiovascular Surgery, 163(3), 1166-1175. https://doi.org/10.1016/j.jtcvs.2021.05.015

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title = "Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study",

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.",

keywords = "Fontan, single ventricle",

author = "Arrigoni, {Sara C.} and Rinske IJsselhof and Douwe Postmus and Vonk, {Judith M.} and Katrien Fran{\c c}ois and Thierry Bov{\'e} and Hazekamp, {Mark G.} and Rijnberg, {Friso M.} and Bart Meyns and {van Puyvelde}, Joeri and Poncelet, {Alain J.} and {de Beco}, Geoffroy and {van de Woestijne}, {Pieter C.} and Bogers, {Ad J.J.C.} and Schoof, {Paul H.} and Tjark Ebels",

note = "Publisher Copyright: {\textcopyright} 2021 The Authors",

year = "2022",

month = mar,

doi = "10.1016/j.jtcvs.2021.05.015",

language = "English",

volume = "163",

pages = "1166--1175",

journal = "Journal of Thoracic and Cardiovascular Surgery",

issn = "0022-5223",

publisher = "Mosby Inc.",

number = "3",

}

Arrigoni, SC, IJsselhof, R, Postmus, D, Vonk, JM, François, K, Bové, T, Hazekamp, MG, Rijnberg, FM, Meyns, B, van Puyvelde, J, Poncelet, AJ, de Beco, G, van de Woestijne, PC, Bogers, AJJC, Schoof, PH & Ebels, T 2022, 'Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study', Journal of Thoracic and Cardiovascular Surgery, vol. 163, no. 3, pp. 1166-1175. https://doi.org/10.1016/j.jtcvs.2021.05.015

TY - JOUR

T1 - Long-term outcomes of atrioventricular septal defect and single ventricle

T2 - A multicenter study

AU - Arrigoni, Sara C.

AU - IJsselhof, Rinske

AU - Postmus, Douwe

AU - Vonk, Judith M.

AU - François, Katrien

AU - Bové, Thierry

AU - Hazekamp, Mark G.

AU - Rijnberg, Friso M.

AU - Meyns, Bart

AU - van Puyvelde, Joeri

AU - Poncelet, Alain J.

AU - de Beco, Geoffroy

AU - van de Woestijne, Pieter C.

AU - Bogers, Ad J.J.C.

AU - Schoof, Paul H.

AU - Ebels, Tjark

PY - 2022/3

Y1 - 2022/3

N2 - 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.

AB - 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.

KW - Fontan

KW - single ventricle

UR - http://www.scopus.com/inward/record.url?scp=85107366609&partnerID=8YFLogxK

U2 - 10.1016/j.jtcvs.2021.05.015

DO - 10.1016/j.jtcvs.2021.05.015

M3 - Article

AN - SCOPUS:85107366609

SN - 0022-5223

VL - 163

SP - 1166

EP - 1175

JO - Journal of Thoracic and Cardiovascular Surgery

JF - Journal of Thoracic and Cardiovascular Surgery

IS - 3

ER -

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

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