儿童先天性主动脉瓣狭窄的临床分类及转归分析

摘要

目的:探讨儿童先天性主动脉瓣狭窄的解剖特点、治疗方式及临床转归.方法:对2012年1月至2016年10月,于我科住院治疗的儿童先天性主动脉瓣狭窄(AS)患者82例(男女比1.16∶1,中位年龄48个月)按不同标准行临床分组研究:包括主动脉瓣解剖形态,合并的心内畸形及不同的干预手段等,通过随访超声心动图指标、临床症状及心功能,评估其转归及影响因素.结果:①本组病例单纯AS36例中,10例行经皮球囊主动脉瓣成形术(PBAV);11例选择外科手术9例行主动脉瓣成形术(AVP),2例行主动脉瓣置换(AVR),另15例随访观察;AS合并其他心血管畸形患儿46例中,14例选择外科手术同时矫治AS及其他合并畸形,合并动脉导管未闭或主动脉缩窄的部分病例因AS轻度未处理主动脉瓣,随访AS程度减轻.②瓣膜形态与疗效:40例三叶主动脉瓣(TAV)中,有13例行介入或外科手术治疗;41例二叶主动脉瓣(BAV)中,22例行介入或外科手术治疗.按BAV及TAV分组,本组提示同种干预方式时的即刻和中期压差变化差异无统计学意义(P＞0.05),且主动脉瓣反流均无明显加重.③干预方式与疗效:33例AS矫治成功的患者中,10例PBAV及23例AVP分组比较,发现在同种瓣膜形态时,即刻和中期压差下降,差异无统计学意义(P＞0.05),且随访主动脉瓣反流无明显加重.结论:PBAV或AVP治疗对BAV与TAV的AS患儿,早、中期疗效无明显差异.儿童AS需综合评估制定个体化随诊治疗方案.%Objective:To investigate the clinical characteristics,managements and prognosis of congenital aortic stenosis in children.Methods:82 patients of AS since 2012 were selected and assorted to different groups,according to their accompanied cardiovascular diseases,shapes of the aortic valve and managements.The ratio of male to female is 1.16∶ 1,and the median age is 48 months.Results:①Among the 36 cases of isolated AS,10 cases were treated by PBAV,11 cases underwent surgical operations (AVP in 9 cases,AVR in 2 cases),the others are on observation and followed-up.Among the 46 cases with other cardiovascular deformities,surgical operations are offered in 14 cases to corrected both AS and other malformations.Partial patients of mild AS accompanied by PDA or CoA were not treated but presented with decreasing pressure.②Aortic valve configuration and effect:There are 13 cases of 40 in TAV,and 22 cases of 41 in BAV were treated by intervention or surgical operations.Grouped by TAV and BAV,no significant statistical difference (P ＞ 0.05) was found in the immediate and midterm pressure reducement when offered the same treatment,so did the aortic valve regurgitation.③Treatment and effect:Among the successfully treated 33 cases grouped by PBAV (10 cases) and AVP (23 cases),no significant statistical difference (P ＞ 0.05) was discovered in the immediate and midterm pressure relief when came to the same valve deformation,so did the aortic valve regurgitation.Conclusion:PBAV and AVP can be selected as modality of treatment in congenital AS children,and they are of the comparable effects in the short-term and midterm in this study.Children of AS are recommended with individual managements in therapy and following-up only after a compositive evaluation is made.