"\u00C9lectrocardiographie" . "Le bloc auriculo-ventriculaire (BAV) isol\u00E9 non immun cong\u00E9nital et de l'enfance est diagnostiqu\u00E9 de in utero jusqu'\u00E0 l'\u00E2ge de 18 ans. L'objectif de l'\u00E9tude \u00E9tait d'\u00E9tudier le pronostic \u00E0 long terme des enfants ayant ce type de BAV. Nous avons \u00E9tudi\u00E9 r\u00E9trospectivement les enfants ayant un BAV isol\u00E9 non immun n\u00E9s entre 1980 et 2022 avec un diagnostic avant 18 ans en France et avons \u00E9tudi\u00E9 les complications. Nous avons inclus 385 enfants atteints de BAV isol\u00E9 non immun. Le BAV \u00E9tait asymptomatique chez 311/385 (81%) et complet chez 263 (68.3%) patients au moment du diagnostic. Il y a eu une progression des BAV incomplets vers un BAV complet chez 70% des patients. L'implantation de pacemaker a \u00E9t\u00E9 n\u00E9cessaire chez 286 enfants (74%). Au cours du suivi, aucun patient n'est d\u00E9c\u00E9d\u00E9 ou n'a d\u00E9velopp\u00E9 de fibrose endomyocardique ou n'a d\u00E9velopp\u00E9 de cardiomyopathie dilat\u00E9e. A travers cette cohorte, nous pouvons confirmer l'excellent pronostic de ce type de BAV \u00E0 travers la plus grande cohorte existante sur le sujet." . . . "Bloc auriculoventriculaire" . . . "Devenir des blocs atrioventriculaires isol\u00E9s non immuns diagnostiqu\u00E9s in utero ou dans l'enfance, une \u00E9tude r\u00E9trospective" . "Th\u00E8ses et \u00E9crits acad\u00E9miques" . . . . "Devenir des blocs atrioventriculaires isol\u00E9s non immuns diagnostiqu\u00E9s in utero ou dans l'enfance, une \u00E9tude r\u00E9trospective" . . . . . . . . . "P\u00E9diatrie" . "2023" . "Background and aim: Congenital and childhood idiopathic atrioventricular block (AVB) are diagnosed in utero or up to 18 years old. Their natural history has been poorly studied but a first study including 141 children from 1980 to 2009 has shown a good prognosis with very few complications. Our aim was to complete this cohort and study the prognosis of these children with congenital or childhood idiopathic AVB. Methods: We retrospectively studied children with idiopathic AVB born between 1980 and 2022 with a diagnosis from in utero to the age of 18 years in 29 French medical centers and studied the clinical, rhythmic and echogenic complications during follow-up. Results: We included 385 children with idiopathic AVB: 34 were congenital, 351 had a childhood AVB. Children with structural heart disease, infective or immunologic (maternal antibodies) induced AVB were excluded. Atrioventricular block was asymptomatic in 311 (81%) and complete in 263 (68.3%) patients at diagnosis. There was a progression to complete AVB in 70% of patients with incomplete AVB. The median age at pacemaker implantation was 5 years [2.0;10.0] for the whole series; it was 1.2 month [1.2;12] in case of congenital AVB and 6 years [3;11] in case of childhood AVB. Indication for pacemaker implantation was prophylactic in 203 children (71%). During this follow-up, it was noted that cardiac stimulation methods have evolved with many more dual-chamber and epicardial pacemakers being implanted than in 1980. 3 patients have a regression of their conduction disorder and normalization of cardiac conduction. During the median follow up of 10 [5;17] years, no patient died, developed endomyocardic fibrosis or developed dilated cardiomyopathy. Conclusion: In this large French national retrospective study, long-term outcome of congenital or childhood AVB was excellent and confirms the very good prognosis of this type of AVB on a larger cohort, with a longer follow-up. Indeed, no patient died, developed endomyocardic fibrosis or dilated cardiomyopathy with or without pacemaker implantation. However, the majority of children required pacemaker implantation during their follow-up, albeit often as a prophylactic measure." . "Enfants -- Maladies -- Diagnostic" . . . . "Text" .