目的 探讨皮罗氏序列征伴先天性心脏病的临床特点。方法 我院2011年1月—2015年12月共收治141例皮罗氏序列征的患儿,将患儿分成单纯皮罗氏序列征组、皮罗氏序列征伴腭裂组及皮罗氏序列征伴听力损伤组,对3组患儿均行心电图及彩色多普勒超声心动图检查,对检查结果进行分析。结果 141例皮罗氏序列征患儿中共检出先天性心脏病19例,总发生率为13.47%,其中单纯皮罗氏序列征组患儿先天性心脏病的发生率为12.82%,伴腭裂组和伴听力损伤组患儿先天性心脏病的发生率分别为13.56%、25%。主要的先天性心脏病为:房间隔缺损(ASD)、室间隔缺损(VSD)、动脉导管未闭(PDA)、肺动脉瓣狭窄(PS)。通过统计学分析,3组患儿先天性心脏病的发生率差异无显著性。结论 先天性心脏病在皮罗氏序列征患儿中发生率较高,心电图及彩色多普勒超声心动图检查能清楚准确地诊断各类型的先天性心脏病,可用于皮罗氏序列征患儿常规检查,做出早期诊断、治疗,可以改善患儿的预后。

Objective To explore the clinical characteristics of congenital heart diseases in patients with Pierre-Robin sequence. Methods From January 2011 to December 2015, Guangzhou Women and Children's Medical Center has treated 141 patients with Pierre-Robin sequence. They were divided into 3 groups: Pierre-Robin sequence group, Pierre-Robin sequence and cleft palate group, and Pierre-Robin sequence and hearing impairment group. Electrocardiogram and colour Doppler echocardiogram was performed in each group and the results were analysed with statistics. Results 19 patients were found to have congenital heart diseases in 141 patients, the occurrence rate was 13.47%. The occurrence rate of congenital heart diseases in the group with Pierre-Robin sequence was 12.82%. The occurrence rate of congenital heart diseases in the group with Pierre-Robin sequence and cleft palate was 13.56%. The occurrence rate of congenital heart diseases in the group with Pierre-Robin sequence and hearing impairment was 25%. Atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA),and pulmonary valve stenosis (PS) were the most common lesions. Through statistical analysis, the occurrence rate of congenital heart diseases has no significant difference among the three groups of patients. Conclusion The occurrence rate of congenital heart diseases in patients with Pierre-Robin sequence is high. Electrocardiogram and colour Doppler echocardiogram are the useful and reliable tool in diagnosing congenital heart diseases in patients with Pierre-Robin sequence.