儿童肺动脉高压276例病因学分析

Analysis of the Etiology of Pulmonary Arterial Hypertension in 276 Children

目的探讨儿童肺动脉高压（PAH）的病因及相关因素,以提高儿童PAH的早期诊断和治疗。方法回顾性分析1995年5月-2007年5月北京大学第一医院儿科住院患儿中包含PAH诊断并经超声心动图检查证实为肺动脉压力升高的病例,记录其年龄、性别、临床主要诊断、PAH相关症状及超声心动图估测的肺动脉压力,按病因进行分组统计,对不同病因组间经多普勒超声心动图三尖瓣返流速度估测的肺动脉收缩压（sPAP）进行比较。不属于威尼斯会议PAH临床诊断分类中第一类的PAH未统计在内。结果共诊断PAH 276例。男168例,女108例;年龄1个月~17岁,中位年龄9个月。其中特发性PAH 9例（3.3%）,余267例（96.7%）均为相关性PAH,其中以先天性心脏病（先心病）相关性PAH为主,共245例（88.7%）,其中又以室间隔缺损等左向右分流先心病为主,共217例（88.6%）,复杂先心病相关PAH 28例（11.4%）。结缔组织病相关PAH 19例（6.9%）,PAH在SLE、幼年型类风湿关节炎及多发性大动脉炎中的发病率分别为10.3%（13/126）、8.7%（4/46）、15.4%（2/13）,余2例与门脉高压相关,1例与珠蛋白生成障碍性贫血相关。经三尖瓣返流速度估测的8例特发性PAH患儿sPAP为（74.6±23.9）mmHg（1mmHg=0.133 kPa）,显著高于33例先心病相关PAH[（58.0±19.7）mmHg]及12例结缔组织病相关PAH[（49.6±18.9）mmHg]（t=-2.052,-2.609 Pa〈0.05）。结论儿童PAH主要是由多种病因引起的相关性PAH,常见病因为先心病和结缔组织病,特发性PAH患儿确诊时病情已较严重。

Objective To investigate the etiology and clinical characteristics of pediatric pulmonary arterial hypertension （ PAH ） and improve its early diagnosis and treatment. Methods The clinical and echocardiogram data of all inpatients with PAH in Pediatric Department of Peking University First Hospital between Mayd995 and May 2007 were retrospectively analyzed for age,sex,etlology, symptoms and echocardiographic measurement of pulmonary artery pressure, Data were divided into groups according to different etiology and statistics. Pulmonary arterial systolic pressure （sPAP） values estimated from the tricuspid regurgitant velocity by Doppler echocardlography were compared among different groups. Cases who were not belonged to the first category of the Venice Clinical Classification of pulmonary hypertension were not in- cluded. Results Totally 276 cases,168 boys and 108 girls were diagnosed to have PAH. Age ranged fi＇om 1 month to 17 years,median age was 9 months. Most of pediatric PAH was associated -PAH （267 cases ,96.7% ）, while idiopathic PAH took a small part （9 cases ,3.3 % ）. Congenital heart disease- associated PAH（CHD- PAH） was predominant （245 cases,88.7% ） and left to right shunt was the main lesion （217 cases,88.6% ）,while complex lesion- associated PAH comprised 28 cases（ 11.4% ）. Connective tissue disease associated PAH（ CTD- PAH） was the second common among this group of pediatric PAH patients （ 19 cases,6.9 % ）. The incidence of PAH in systemic lupus ery- thematosus（SLE） ,juvenile rheumatoid arteritis and takayasu arteritis were 10.3 % （ 13/126 ） , 8.7% （4/46）, 15.4% （ 2/13 ）, respectively. The other 3 cases of PAH were associated with portal hypertension （2 cases） and thalassanemia （1 case）. The estimated sPAP from tricuspid regurgitant velocity in 8 cases with idiopathic PAH [ （74.6 ± 23.9 ） mmHg （ 1 mmHg = 0.133 kPa） ] was higher significantly compared with those of 33 cases of CHD-PAH [（58.0±19.7） mmHg ] and 12 cases of CTD-PAH [（49.6±18.9） mmHg] respectively（t= -2.052, - 2. 609 Pa 〈 0.05 ）. Conclusions Pediatric PAH is mainly associated - PAH related to various diseases, among which CHD - PAH and CTD - PAH are predominant. Idiopathic PAH is more severe when the diagnosis was made.