先天性完全性房室传导阻滞临床特征及治疗转归

Clinical Feature and the Results of Therapy of Congenital Complete Atrioventricular Conduction Block

目的:分析先天性完全性房室传导阻滞患儿的临床特征及治疗转归,为早期诊断、治疗和改善预后提供临床依据。方法:回顾性总结分析2000年8月～2010年10月在我院住院并确诊为先天性完全性房室传导阻滞患儿共38例,男21例,女17例,分析母孕期情况、临床表现、心电图、Holter和超声心动图结果,随访时行心电图和超声心动图等检查。结果:在38例CCAVB患儿中发现宫内心动过缓者9例,其中出生后确诊为新生儿红斑狼疮2例;15例伴阿斯综合征发作,初发年龄为4月～3岁,平均(1.9±1.0)岁;14例合并先天性心脏病,分别为ASD 5例,PDA 7例,PS 1例,MS 1例;19例安装起搏器1,例为PDA手术时行心外膜起搏;超声心动图发现左心室增大20例,以学龄(前)期多见,较婴幼儿期有显著差异(P<0.05);心电图发现QTc延长者11例3,例合并尖端扭转室速;Holter显示婴幼儿期室性早搏多见,较学龄(前)期有显著差异(P<0.05);随访发现起搏器安装后心脏扩大伴二尖瓣反流者3例,心功能不全1例。结论:先天性完全性房室传导阻滞患儿临床以心动过缓和阿斯综合征发作为主要表现,部分可合并各种先天性心脏病。早期确诊患儿如出现心动过缓伴心脏扩大、心功能不全或阿斯发作需早期安装永久起搏器,可减少病死率;年长儿出现阿斯综合征发作或心功能不全也需安装起搏器。随访发现右室心尖部起搏可造成心脏扩大、二尖瓣反流和心功能不全,选择右室流出道起搏不良反应更少。

Objective：To analyze the clinical feature and the results of therapy for congenital complete atrioventricular conduction block,and to offer clinical evidence for early diagnosis,treatment,and improved prognosis.Methods：Thirty-eight patients were diagnosed with CCAVB in our hospital during Aug,2000 to Oct 2010,21 males and 17 females,the condition of pregnancy,clinical manifestations,EKG,Holter and echocardiography results were analyzed.All of them were examined with EKG and 2DE when follow-upped.Results：Nine patients were found with intrauterine bradycardia,2 of them were diagnosed with neonatal lupus erythematosus,15 with Adams-Stokes,the first time of diagnosis were 4-month to 3-years after birth,the average age was 1.9±1.0 year;14 combined with CHD,5 had ASD,7 PDA,1 PS and 1 MS respectively,19 were implanted with PPM,1 had pericardial pacing when taken for surgical procedure,the results of 2DE showed 20 with left cardiac dilatation which was common in（before） school age.There was significantly different（P0.05） compared to infancy.The results of EKG showed 11 with QTc prolongation,3 of them were confirmed with Tdp;the results of Holter showed premature ventricular contraction which was common in infancy.There was significant difference（P0.05） compared to（before） school age;3 patients were found with cardiac dilatation and mitral regurgitation,1 with heart failure when follow-upped after PPM implant.Conclusions： The clinical features of patients with CCAVB are bradycardia and Adams-Stokes.Some cases could be combined with a variety of congenital heart diseases.The indication of implanting PPM is bradycardia with cardiac dilatation or heart failure and Adams-Stokes when they are diagnosed in early age and the mortality will reduce;the older children should also be implanted PPM when they had Adams-Stokes and heart failure;the patients are found with MR and cardiac dilatation or heart failure after pacing at RV apex when follow-upped,RV outlet pacing is a better alternative selection.