Shone综合征患儿手术治疗的早期结果分析

Short-term Post-operative Outcome of Infants and Children With Shone’s Syndrome

目的:总结分析Shone综合征患儿手术治疗的早期结果。方法:回顾性纳入中国医学科学院阜外医院从2002年1月至2019年12月行手术治疗的Shone综合征患儿共77例。统计分析病例的解剖畸形、手术方式、围术期结果和早期随访结果。结果:77例患儿中男性46例,年龄3.3(0.8,5.3)岁,体重13.0(8.5,18.0)kg。最常见的左心室流入道畸形是二尖瓣瓣上隔膜,最常见的流出道梗阻是主动脉缩窄。绝大部分手术使用正中开胸,体外循环时间103.0(86.0,126.0)min,主动脉阻断时间68.0(48.0,85.0)min,另有26例行深低温停循环,平均时间(23.46±7.53)min。重症监护病房停留时间2.0(1.0,4.0)d。术后早期死亡1例(1.3%)。术后早期左心室各水平压差较术前均显著降低(P均<0.01)。中位随访时间21.50(6.75,76.75)个月。随访期间无死亡。有5例(6.5%)再次手术,均有二尖瓣瓣上隔膜复发。5年免除再手术生存率为89.8%。结论:Shone综合征的临床表现多样,手术早期效果良好。

Objectives:To summarize and analyze the short-term post-operative outcome of children with Shone’s syndrome.Methods:A total of 77 infants and children,who were diagnosed with Shone’s syndrome and underwent surgery from January 2002 to December 2019 in Fuwai Hospital,were retrospectively included in this study.Anatomic malformations,operation methods,perioperative results and early outcomes were analyzed.Results:Among the 77 patients,46 were males.The median age was 3.3(0.8,5.3)years and body weight was 13.0(8.5,18.0)kg.The most common left ventricular inflow tract malformation was supravalve mitral membrane(n=61,79.2%),while the most common left ventricular outflow tract obstruction was aortic stenosis(n=42,54.5%).In most cases,median sternotomy was performed.The median cardiopulmonary bypass time was 103.0(86.0,126.0)min,and aortic clamp time was 68.0(48.0,85.0)min.Deep hypothermic circulatory arrest was performed in 26 cases,with an average time of(23.46±7.53)min.Median duration of ICU stay was 2.0(1.0,4.0)days.One patient(1.3%)died early post operation.The pressure gradients at all left ventricular stenotic levels were significantly decreased after surgery(all P<0.01).There was no further death during a median follow-up of 21.50(6.75,76.75)months.There were 5 reoperations(6.5%)due to recurrent supravalve mitral membrane.Freedom from reoperation survival was 89.8%at 5 years post operation.Conclusions:The clinical manifestations of Shone’s syndrome are diverse and heterogeneous.Short-term outcome postsurgery is satisfactory in these patients.