INSERM, U915, l'Institut du Thorax, Nantes F-44000, France.
Eur Heart J. 2012 Mar;33(5):622-9. doi: 10.1093/eurheartj/ehr347. Epub 2011 Sep 14.
The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined.
We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1-155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0-300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1-32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%).
In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patient's age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few.
先天性或儿童期非免疫性孤立性房室（AV）阻滞的自然史尚未明确。
我们回顾性研究了 1980 年至 2009 年期间，13 家法国医疗中心诊断的 141 例孤立性、非免疫性 AV 阻滞的儿童患者，这些患者为宫内或 15 岁以下患者，且排除了结构性心脏病或母体抗体患者。119 例（84.4%）无症状，100 例（70.9%）完全性 AV 阻滞。29/41 例不完全性 AV 阻滞患者在 2.8±3.4 年内（1-155 个月）进展为完全性 AV 阻滞，但不完全性 AV 阻滞患者并非全部纳入研究。先天性 AV 阻滞 26 例中 18 例（69.2%）和儿童期 AV 阻滞 115 例中 106 例（92.2%）存在窄 QRS 波群。112 例（79.4%）儿童患者植入了起搏器，18 例（16.1%）在 1 岁以内，90 例（80.4%）在 10 岁以内植入。AV 阻滞诊断与起搏器植入之间的平均间隔为 2.6±3.9 年（0-300 个月）。70 例（62.5%）为预防性起搏指征。平均随访 11.6±6.7 年（1-32 年）后，无患者死亡或发生扩张型心肌病（DCM）。127 例（90.1%）患者长期随访无并发症。
在这项大型多中心研究中，先天性或儿童期非免疫性孤立性 AV 阻滞的长期预后良好，与患者诊断时的年龄无关。无患者死亡或发生 DCM，起搏器相关并发症较少。
