儿童特发性肺动脉高压的临床特点及预后的影响因素

目的：调查儿童特发性肺动脉高压( idiopathic pulmonary arterial hypertension,IPAH)的临床特点及预后的影响因素,为临床诊治及预后评估提供参考。方法回顾性分析2006年1月至2015年12月于我科诊断为IPAH患儿的临床资料,根据预后分为存活组和死亡组,对相关指标应用Logistic回归分析筛选出影响IPAH患儿预后的危险因素。结果共计26例患儿纳入研究,存活组17例,死亡组9例。儿童IPAH以学龄期儿童多见,平均年龄(6.6±3.6)岁,男女比例1.17∶1。所有患儿均有活动耐力下降和乏力症状,晕厥及呼吸困难、水肿的发生率分别为34.6％及23.1％,肺动脉瓣听诊区第2心音亢进为最常见体征(92.3％),50.0％患儿在诊断时心功能为Ⅲ~Ⅳ级。超声心动图检查示肺动脉收缩压(PASP)平均(79.0±20.3) mmHg(1 mmHg＝0.133 kPa),16例患儿右心室重度扩大。心电图以ST-T改变及右心室高电压最为常见,血浆脑钠肽(BNP)平均浓度为(870.4±720.9) pg/ml。单因素分析显示晕厥(OR＝26.25,95％CI 3.04~226.60,P＝0.003),心功能Ⅲ~Ⅳ级(OR＝19.199,95％CI 1.88~196.53,P＝0.0128),PASP≥70 mmHg(OR＝9.936,95％CI 1.81~∞,P＝0.005),BNP≥850 pg/ml(OR＝59.991,95％CI 4.69~767.62,P＝0.002)是预后不良的危险因素。结论儿童IPAH临床少见,预后不良。晕厥、心功能分级、PASP程度及BNP浓度是儿童IPAH预后的重要影响因素。 BNP作为IPAH的标志物在病情程度评估以及预后预测中具有一定的参考价值。

Clinical features and prognostic factors in children with idiopathic pulmonary arterial hypertension

Objective To investigate the clinical features and prognostic factors in children with idi-opathic pulmonary arterial hypertension(IPAH). Methods The data of children with IPAH diagnosed in Beijing Children′s Hospital from January 2006 to December 2015 were collected. The cases were divided into survival group and death group according to the prognosis. The risk factors influencing prognosis in children with IPAH were identified by Logistic regression analysis. Results Total 26 patients were enrolled in this study,and 17 of them survived and 9 of them were dead. IPAH was found to be more common in school-age children. The average age of the patients was(6. 6 ± 3. 6)years and the male to female ratio was 1. 17∶1. All of the children had the symptoms with decreased activity tolerance and fatigue. The incidence of syncope and dyspnea and edema of lower extremity were 34. 6%,23. 1%. Accentuated pulmonic second sound(P2) was detected in 92. 3% of patients during physical examination, which was also the most common sign. About 50. 0% patients were functional class Ⅲ-Ⅳ. Echocardiography showed that all childrens′ mean pulmonary artery systolic pressure(PASP) was(79. 0 ± 20. 3) mmHg(1 mmHg=0. 133 kPa) and 16 of them with right ventricular dilatation. Electrocardiogram with ST-T changes and right ventricular high voltage were common. The average plasma level of brain natriuretic peptide(BNP) in all cases was(870. 4 ± 720. 9) pg/ml. The single factor analysis illustrated that syncope(OR=26. 25,95%CI 3. 04 -226. 60,P=0. 003),heart func-tional class Ⅲ-Ⅳ(OR=19. 199,95%CI 1. 88 -196. 53, P=0. 0128), PASP≥70 mmHg(OR =9. 936, 95%CI 1. 81-∞,P=0. 005),BNP≥850 pg/ml(OR=59. 991,95%CI 4. 69-767. 62,P=0. 002) indica-ted the worse outcome. Conclusion IPAH in children is rare and the prognosis is poor. Syncope, cardiac function grade,PASP and the levels of BNP are the main important factors affecting the prognosis of children with IPAH. BNP has important clinical significance for the evaluation of IPAH and the prognosis of the disease.