手术治疗先天性主动脉瓣上狭窄的临床分析及远期结果

Clinical Analysis of Congenital Supravalvular Aortic Stenosis Treated by Surgery and Long-term Outcome

目的分析先天性主动脉瓣上狭窄的手术效果及远期疗效。方法收集阜外医院2008年9月至2014年9月先天性主动脉瓣上狭窄25例患者资料,进行回顾性分析,并长期随访。结果25例患者经超声心动图检查明确诊断为先天性主动脉瓣上狭窄,其中男性17例(68%),女性8例(32%)。年龄为24-582(106.88±127.60)月,体重为10.6-60(24.45±13.92)公斤。患者经手术治疗后,主动脉瓣上狭窄处得以加宽,狭窄处流速、压差及左室舒张末内径较术前发生显著性差异(P<0.05),术后均恢复良好并顺利出院。Doty法和McGoon法矫治先天性主动脉瓣上狭窄均可获得满意的治疗效果。术后平均随访时间56.59±30.38月,随访患者20例(80%),目前均生存,无心功能不全症状;生长发育迟缓,身高及体重低于同年龄人3例(15%);智力低下者5例(25%),主动脉狭窄及压差偏高者3例(15%)。结论主动脉瓣上狭窄经手术治疗后,狭窄处流速及压差明显降低,左室舒张末内径显著变小,手术效果明显,远期疗效满意。

Objective To analyze the effect and the long-term outcome of congenital supravalvular aortic stenosis(SAVS)by surgical treatment.Methods Data of 25 patients with SAVS in Fuwai hospital from September 2008 to September 2014 were collected for retrospective analysis and followed up to March 2019.Results 25 patients were diagnosed with SAVS by echocardiography,including 17 males(68%)and 8 females(32%).The age was 24-582(106.88±127.60)months,and the weight was 10.6-60(24.45±13.92)kg.Compared with preoperative,the stenosis was widened and there were significant differences in the flow rate,pressure gradient and the left ventricular end-diastolic dimension after surgery(P<0.05).All patients recovered well and were discharged successfully.Both Doty and McGoon procedure could get satisfactory clinic results.20 patients(80%)were followed up for 56.59±30.38 months,all of whom were alive without cardiac dysfunction.Growth retardation,lower height and weight than the same age in 3 cases(15%).Mental retardation in 5 cases(25%),aortic stenosis and high differential pressure in 3 cases(15%).Conclusions After surgical treatment of SAVS,the flow rate and pressure gradient at the stenosis site and the left ventricular end-diastolic diameter were significantly decreased.The surgical effect was obvious,and the long-term outcome was satisfactory.