儿童肺动脉高压64例临床分析

摘要

目的 分析儿童肺动脉高压(pulmonary arterial hypertension,PAH)的临床资料,以提高对该病的认识.方法 对64例包含PAH诊断患儿的临床表现、辅助检查、诊疗过程进行回顾性分析,并复习相关文献.结果 ①64例患儿中57例(89.06%)为先天性心脏病相关PAH.左向右分流型先天性心脏病49例,其中11例经内科治疗,肺部感染、心力衰竭难以控制,肺动脉压力较另38例高[分别为(9.15±2.87)、(6.68±2.49)kPa,t=2.800 1,P<0.05],49例中除2例死亡外,余47例术后随访6个月~1年,肺动脉压力均降至正常;复杂型先天性心脏病相关PAH 8例,术后肺动脉压力较术前下降[分别为(5.36±1.53)、(8.77±2.61)kPa,t=-3.564 3,P<0.05].②2例(3.13%)为腺样体肥大引起PAH,肺动脉压力分别为16.08、16.91kPa,予以腺样体切除后,1例肺动脉压力降至正常,1例肺动脉压力较术前有所下降(10.67kPa),但仍较正常高.③2例(3.13%)为结缔组织病合并PAH,其中1例为系统性红斑狼疮,1例为幼年特发性关节炎,肺动脉压力分别为16.8、14.4kPa,2例均死亡.④3例(4.68%)为特发性PAH,肺动脉压力分别为14.67、8.88、10.67kPa,自动出院.结论 儿童PAH可由多种原因引起,需积极查找病因,及时治疗,可改善预后.%Objective To analyze the clinical data of pulmonary arterial hypertension( PAH )in children in order to improve the recognition of this disease. Methods The clinical manifestation,auxiliary examination, diagnosis and treatment of 64 pediatric inpatients with PAH were analyzed retrospectively,and the relative literatures were reviewed. Results ① In 64 pediatric inpatients with PAH,57( 57/64,89.06％ )cases were congenital heart disease- associated PAH( CHD- PAH ), left to right shunt CHD was 49 cases. Among 49 cases, 11 cases 'pulmonary inffection, cardiac failure were hard to control permedical treatment, their pulmonary arterial pressure( PAP )were higher than the other 38 cases'[ ( 9.15±2.87 )kPa vs( 6.68 ±2.49 )kPa,t =2. 800 1 ,P ＜0.05 ]. Except that 2 cases died,the other 47 cases 'PAP were normal in 6 months to 1 year follow up after the operation. Complex CHDPAH comprised 8 cases, their postoperative PAP were lower than preoperative PAP[ ( 5.36± 1.53 )kPa vs ( 8.77 ±2.61 )kPa,t = -3. 564 3 ,P ＜0.05 ]. ②Two( 2/64,3.13％ )cases were adenoid hypertrophyassociated PAH, PAP was respectively 16.08kPa and 16.91kPa. After adenoidectomy, 1 case's PAP was normal, the other's PAP was lower than preoperative PAP( 10.67kPa ), but higher than normal. (③)Two( 2/64,3.13％ )cases were connective tissue disease- associated PAH( CTD -PAH ), 1 case was systemic lupus erythematosus, the other was juvenile rheumatoid arthritis, PAP was respectively 16. 8kPa and 14.4kPa,2 cases all died. (④Three( 3/64,4. 68％ )cases were idiopathic PAH( IPAH ), PAP was respectively 14.67 kPa,8.88 kPa and 10.67 kPa, they left hospital voluntarily. Conclusion Pediatric PAH can be caused by various diseases, etiopathogenisis must be found out. If treating earlier, the prognosis can be improved.