Mycinski Florence, Waldmann Victor, Kyndt Florence, Guyomarch Béatrice, Maltret Alice, Wilkin Marie, Ovaert Caroline, Vaksmann Guy, Thambo Jean-Benoit, Sellal Jean-Marc, Padovani Paul, Lwin Naychi, Prigent Solène, Werner Oscar, Barc Julien, Schott Jean-Jacques, Bonnet Damien, Probst Vincent, Baruteau Alban-Elouen
Department of Pediatric Cardiology and Pediatric Cardiac Surgery, Nantes Université, CHU Nantes, FHU PreciCare, F-44000 Nantes, France.
Nantes Université, CHU Nantes, CNRS, INSERM, l'Institut du Thorax, F-44000 Nantes, France.
Europace. 2025 Mar 5;27(3). doi: 10.1093/europace/euaf040.
The natural history of congenital or childhood non-immune, isolated atrioventricular block (AVB) is poorly defined. We aimed at clarifying its long-term outcomes.
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.
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.
先天性或儿童期非免疫性孤立性房室传导阻滞（AVB）的自然病程尚不明确。我们旨在阐明其长期预后。
我们对1980年至2022年间法国29个医学中心诊断为孤立性、非免疫性AVB的385例儿童进行了回顾性研究。排除患有结构性心脏病、心内膜纤维化或母体抗体的患者。诊断时，314例（81.6%）患者的房室传导阻滞无症状，263例（68.3%）患者为完全性房室传导阻滞。122例不完全性房室传导阻滞患者中有84例（68.8%）在12年（7 - 17岁）内进展为完全性房室传导阻滞。共有286/385例患者（74.3%）接受了永久性起搏器植入，39例（14%）在出生后第一年植入，172例（60%）在10岁前植入。203例儿童（71%）的起搏指征为预防性。133/385例患者（34.5%）进行了基因筛查，11例（8.3%）患者鉴定出具有临床可操作性的变异。中位随访10年（5 - 17年）后，无患者死亡、发生心内膜纤维化或扩张型心肌病。
在这项全国性的大型研究中，先天性或儿童期非免疫性孤立性AVB的长期预后良好。大多数儿童最终需要植入起搏器，尽管通常是作为预防性措施。
