不完全型Shone综合征6例临床分析及文献复习  

Six case report and literature review Incomplete shone syndrome

作　　者：曹跃丰[1,2] 李晓锋[1,2] 苏俊武[1,2] 刘迎龙[1,2] 

机构地区：[1]首都医科大学附属北京安贞医院 [2]北京市心肺血管疾病研究所小儿心脏中心,100029

出　　处：《心肺血管病杂志》2015年第7期559-561,共3页Journal of Cardiovascular and Pulmonary Diseases

摘　　要：目的:总结不完全型Shone综合征的外科治疗经验和策略。方法:回顾性分析2012年10月至2014年9月,收治的Shone综合征6例。总结Shone综合征的诊断及外科治疗经验,讨论外科治疗策略与技术关键。结果:5例行一期矫治,1例分2期矫治,无手术死亡。左心室流入道和左心室流出道梗阻解除满意,其中二尖瓣病变术前平均压差(18.7±6.91)mm Hg(1mm Hg=0.133k Pa),出院平均压差(8.7±2.73)mm Hg。结论:Shone综合征是一类罕见的左心室流入道和左心室流出道不同程度梗阻的疾病,一经诊断需手术干预,流入道梗阻是术后远期随访重点。Objective： Shone syndrome is an unusual and complicated multiple cardiac abnormalities.We retrospectively evaluate the surgical experience and strategy in patients with incomplete shone syndrome.Methods： Between October 2012 and September 2014,there were 6 patients diagnosed with Shone syndrome in our institution. The diagnostic experience and operative strategy in our group were introduced and the surgical outcomes were also analyzed in detail. Results： One-staged repair was performed in 5（ 83. 3%） patients,while two-staged surgery was performed in 1（ 17. 7%）. No surgical mortality occurred in these patients. The relief of obstruction with left ventricular inflow and outflow track was very effective. Early results were excellent with a significant decrease in pressure gradient from 18. 7 mm Hg（ 11- 30） mm Hg to 8. 7 mm Hg（ 7- 13） mm Hg.Conclusions： Shone syndrome defined as a kind of unusual congenital heart disease with obstruction in both left ventricular inflow and outflow track was always reported with poor prognosis. We suggest early intervention once diagnosed. And the left ventricular inflow track should be one of the most important aspects in the follow-up period.

关 键 词：Shone综合征 主动脉缩窄 二尖瓣瓣上环 降落伞样二尖瓣 

分 类 号：R54[医药卫生—心血管疾病]