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Multicenter Study
. 2025 Mar 5;27(3):euaf040.
doi: 10.1093/europace/euaf040.

Late outcomes of congenital and childhood non-immune, isolated atrioventricular block: a French nationwide retrospective cohort study

Florence Mycinski  1   2 , Victor Waldmann  3   4   5 , Florence Kyndt  2   6 , Béatrice Guyomarch  7 , Alice Maltret  8 , Marie Wilkin  3   9 , Caroline Ovaert  9   10 , Guy Vaksmann  11 , Jean-Benoit Thambo  12   13   14 , Jean-Marc Sellal  15 , Paul Padovani  1   16 , Naychi Lwin  1   16 , Solène Prigent  1   16 , Oscar Werner  1   16 , Julien Barc  2   6 , Jean-Jacques Schott  2   6 , Damien Bonnet  3   6   17 , Vincent Probst  2   6   18 , Alban-Elouen Baruteau  1   2   6   16   19 ; Congenital AVB study group
Collaborators, Affiliations
Multicenter Study

Late outcomes of congenital and childhood non-immune, isolated atrioventricular block: a French nationwide retrospective cohort study

Florence Mycinski et al. Europace. .

Abstract

Aims: The natural history of congenital or childhood non-immune, isolated atrioventricular block (AVB) is poorly defined. We aimed at clarifying its long-term outcomes.

Methods and results: We retrospectively studied 385 children with isolated, non-immune AVB diagnosed from in utero or up to 18 years of age, at 29 French medical centres, between 1980 and 2022. Patients with structural heart disease, endomyocardial fibrosis, or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 314 (81.6%) and complete in 263 (68.3%) patients at the time of diagnosis. There was progression to complete AVB in 84/122 (68.8%) patients with incomplete AVB over 12 years (7-17). A total of 286/385 patients (74.3%) received a permanent pacemaker, implanted in the first year of life in 39 (14%) and before 10 years of age in 172 (60%) children. The pacing indication was prophylactic in 203 children (71%). Genetic screening was performed in 133/385 patients (34.5%), leading to the identification of a clinically actionable variant in 11 (8.3%) patients. After a median follow-up of 10 years (5-17), no patient died or developed endomyocardial fibrosis or dilated cardiomyopathy.

Conclusion: In this large nationwide study, the long-term outcome of congenital or childhood non-immune, isolated AVB was excellent. Most children required pacemaker implantation over time, albeit often as a prophylactic measure.

Keywords: Cardiac conduction disorder; Clinical outcome; Pacemaker; Paediatric electrocardiology.

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Conflict of interest statement

Conflict of interest: none declared.

Figures

Graphical Abstract
Graphical Abstract
Figure 1
Figure 1
Age at the time of diagnosis of heart block. In 268 patients (69.6%), the diagnosis was made before 5 years of age. AVB, atrioventricular block.
Figure 2
Figure 2
Risk of progression of incomplete AVB to complete AVB. (A) At 13 years, half of the children progressed from incomplete to complete AVB. (B) Curves according to age at diagnosis. AVB, atrioventricular block.
Figure 3
Figure 3
Rate of pacemaker implantation during long-term follow-up. (A) After a median follow-up of 10 years, 74.3% of the patients had received a permanent pacemaker, implanted in the first year of life in 7% and before 10 years of age in 77.6% of children. (B) Curves according to age at diagnosis.
Figure 4
Figure 4
Type of pacemaker implantation according to age of implantation and studied period—endocardial or epicardial leads.
Figure 5
Figure 5
Type of first pacemaker implantation by age and time of studied period—single- or dual-chamber device.
See this image and copyright information in PMC

Comment in

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