不同手术方法治疗先天性主动脉瓣上狭窄的临床研究  

The treatment of congenital supravalvular aortic stenosis by different surgical technique

作　　者：朱海龙 魏东明 彭岚刚 顾春虎 韩跃虎 段乐 殷强 孙国成 ZHU Hai-long;WEI Dong-ming;PENG Lan-gang;GU Chun-hu;HAN Yue-hu;DUAN Le;YIN Qiang;SUN Guo-cheng(Department of Cardiac Surgery,Xijing Hospital,Air Force Medical University,Xi'an 710032,China)

机构地区：[1]空军军医大学西京医院心血管外科研究所,陕西省西安市710032

出　　处：《中国心血管病研究》2021年第1期22-25,共4页Chinese Journal of Cardiovascular Research

基　　金：科技部国家重点研发重大慢性非传染性疾病防控研究重点专项课题(2017YFC1308100)。

摘　　要：目的:探讨先天性主动脉瓣上狭窄外科纠治的手术适应证选择、不同手术方法的近、中期疗效。方法:回顾性分析西京医院2005年2月至2020年2月先天性主动脉瓣上狭窄外科治疗病例27例,年龄1.4~13.5y(4.1±3.2),合并染色体异常(Williams综合征)19例(70.4%)。术前主动脉瓣上峰值压差45~98(72.3±13.4)mmHg,其中。其中局限型24例(88.9%),弥漫型3例(11.1%)。主动脉瓣不同程度增厚24例,瓣窦发育不均衡9例,合并少量或微量主动脉瓣反流5例,无主动脉瓣狭窄。合并左、右肺动脉分支狭窄9例。手术均在中低温体外循环下实施,采用单片(McGoon)法7例(25.9%),双片(Doty)法11例(40.7%),三片法9例(33.3%),其中Brom法4例,Myers法5例。肺动脉狭窄同期补片加宽纠治。结果:全组无住院死亡。术后主动脉瓣上峰值压差14~54(21.6±8.4)mmHg,其中单片组20~54(30.6±11.9)mmHg,双片组14~23(19.3±2.9)mmHg,三片组14~22(17.6±2.5)mmHg,单片组与其他两组差异明显(p?0.01),双片组与三片组无明显差异(p>0.05)。主动脉瓣少量反流2例,微量6例。随访21例,失访6例,随访3月~11年(平均3.7年),主动脉瓣上峰值压差17~46(24.1±7.4)mmHg,其中单片组26~46(33.4±10.5)mmHg,双片组19~31(22.3±2.7)mmHg,三片组17~26(19.1±2.1)mmHg,单片组与其他两组差异明显(p>0.01),双片组与三片组无明显差异(p>0.05)。无远期死亡和再次手术干预。主动脉瓣少量反流4例,微量7例。结论:手术纠治先天性主动脉瓣上狭窄是安全的,近、远期疗效满意。双片和三片法手术疗效无明显差异,但均优于单片法,应作为首选手术方法。Objective To investigate the surgical indications for surgical correction of congenital supravalvular aortic stenosis(SVAS)and the short-term and medium-term effects of different surgical technique.Methods Retrospective analysis was performed of 27 cases of surgical treatment of congenital SVAS in Xijing Hospital from February 2005 to February 2020,ageat operation was 1.4-13.5 years,average(4.1±3.2)years and 19 cases(70.4%)with chromosomal abnormalities(Williams syndrome).Preoperative peak pressure gradient of supravalvular aortic was 45-98 mm Hg,average(72.3±13.4)mm Hg;discrete SVAS was present in 24 patients(88.9%)while in 3 patients(11.1%)SVAS morphology was diffuse.There were 24 patients with aortic valve thickening,9 patients with uneven aortic valve sinus,5 patients with mild aortic valve regurgitation and no aortic valve stenosis.There were 9 patients with left and right pulmonary artery branch stenosis.All the surgeries were performed under the moderate hypothermic cardiopulmonary bypass.The single-patch extending coronary sinus technique was performed in 7 patients(25.9%)(single-patch group),two-patch extending technique in 11 patients(40.7%)(two-patch group)and three-patch extending technique in 9 patients(33.3%)(including Brom technique in 4 patients and Myers technique in 5 patients)(three-patch group).Pulmonary artery largement was the concomitant procedures.Results None of the patients died in hospital.Postoperative peak pressure gradient of supravalvular aortic was 14-54 mm Hg,average(21.6±8.4)mm Hg,with 20-54 mm Hg,average(30.6±11.9)mm Hg in the single-patch,14-23 mm Hg,average(19.3±2.9)mm Hg in the two-patch and 14-22 mm Hg,average(17.6±2.5)mm Hg in the three-patch group.There was a higher of residual peak pressure gradient in the single-patch compared with the two-patch and the three-patch group(P<0.01),but no statistical different between the two-patch and the three-patch group(P>0.05).Mildaortic valve regurgitation in 8 patients.Follow-up 3 month to 11 years(average 3.7 years)in 21 patie

关 键 词：先天性心脏病 主动脉瓣上狭窄 WILLIAMS综合征 

分 类 号：R654.2[医药卫生—外科学]