先天性主动脉瓣上狭窄的外科治疗(附35例报告)  

SURGICAL REPAIR OF CONGENITAL SUPRAVALVULAR AORTIC STENOSIS IN 35 CASES

作　　者：王水云[1] 胡盛寿[1] 吴清玉[1] 孙立忠[1] 许建屏[1] 刘迎龙[1] 郭岩[1] 

机构地区：[1]中国医学科学院中国协和医科大学心血管病研究所阜外心血管病医院外科,北京市100037

出　　处：《中国心血管病研究》2003年第2期96-98,共3页Chinese Journal of Cardiovascular Research

摘　　要：目的 总结35例先天性主动脉瓣上狭窄(SVAS)术前诊断和外科治疗中的经验、教训和疗效。方法 1996年10月-2003年1月收治SVAS患者35例,男25例,女10例,平均年龄(9.16±6.35)岁。局限性狭窄25例,弥漫性狭窄10例。Williams综合征7例。14例并发其他心脏畸形。术前超声心动图检查漏诊3例,均为并发主动脉瓣狭窄。单片法加宽21例,倒“Y”法11例,“三片法”2例,Ross手术1例。结果 手术死亡1例。肾功能衰竭1例。升主动脉补片处出血4例,其中2例采用纱布压迫、延迟关胸止血;另2例经二次开胸缝合止血。主动脉瓣上血流速度术前平均(4.72±1.08)m/s,压差平均(93.4±52.9)mmHg。术后早期分别为(1.89±0.61)m/s,(16.3±10.5)mmHg。31例随访1个月-5.5年,心功能Ⅰ级28例,Ⅱ级3例。结论 该病的手术治疗效果良好。局限性SVAS并发主动脉瓣狭窄时,术前超声心动图检查易于漏诊。对Williams综合征患者,术前应进行更为详细的检查。Objective To review the experience of diagnosis and surgical repair of congenital supravalvular aortic stenosis(SVAS) . Methods From October 1996 to January 2003,35 cases underwent surgical correction for congenital SVAS at Fu Wai Hospital. There were 25 males(71% )and 10 females (29%) ranging from 0.9-33(9.16±6.35) years old. Of the 35 cases, 25(71% ) were localized type SVAS and 10(29 % ) diffused type SVAS. And there were 7 cases with Williams syndrome, 14 cases with other cardiac defects, 3 cases with aortic valve stenosis misdiagnosed by echocardiography examination preoperatively. Operative technique included single, diamond-shaped patch aortoplasty in 21 cases, inverted bifurcated patch aortoplasty in 11 cases, three coronary sinuses patch aortoplasty in 2 cases, and Ross procedure in 1 case. Results 1 case with Williams syndrome died of severe pulmonary hypertension during the operation. Hemmorage in the aortic patch area was found in 4 cases. Renal failure occurred in 1 case postoperatively. The mean pressure gradient reduced from (93. 4 ± 52.9)mmHg to (16.3±10.5) mmHg in the early postoperative period. Of 31 cases followed up from 1 month to 5. 5 years, there were 28 class I heart function cases and 3 class Ⅱ cases. Conclusion Good surgical outcome of congenital SVAS can be achieved with the patch aortoplasty technique. The aortic valve stenosis in cases with localized type SVAS is easily misdiagnosed by echocardiography examination preoperatively. More detail examination is necessary in SVAS cases with Williams syndrome.

关 键 词：先天性主动脉瓣上狭窄 外科治疗 SVAS 手术 诊断 

分 类 号：R654.2[医药卫生—外科学]