右心室双出口102例外科治疗

目的分析总结右心室双出口(DORV)外科治疗效果。方法 1997年1月—2012年6月,外科手术治疗DORV共102例,按照STS-EACTS制定的先心病数据库命名计划将DORV分型:法洛四联症型50例(49.0%)、室间隔缺损型28例(27.5%)、大动脉转位型16例(15.7%)、远离两大动脉VSD型8例(7.8%)。手术方式:双心室矫治术76例(74.5%),单心室功能矫治术19例(18.6%),其他姑息手术7例(6.9%)。结果全组住院死亡13例(死亡率12.7%),主要死亡原因是术后低心排血量综合征7例,早期再手术4例(3.9%)。随访67例,随访时间3～167(中位数50,四分位间距73)个月,需再次手术的患者共15例(15/67,22.4%),心室流出道梗阻是再次手术的主要原因。结论根据STS-EACTS制定的标准对DORV进行分类,更有利于选择手术指征和分析结果。随访期间需要再次手术的患者应及时采取治疗措施。DORV的外科治疗可获得良好的早中期效果。

Surgical outcomes in 102 patients with double outlet right ventricle

Objective To review the surgical results of double outlet right ventricle(DORV).Methods Between January 1997 and June 2012,102 patients with DORV underwent surgical treatment,including biventricular repair in 76 cases(74.5%),single-ventricle repair in 19 ones(18.6%),and palliative procedures in 7 ones(6.9%).The patients were classified into the four types according to the STS-EACTS international nomenclature for congenital heart surgery: 50 cases(49.0%) were DORV-Fallot,28(27.5%) were DORV-ventricular septal defect(VSD),16(15.7%) were DORV-transposition of the great artery(TGA)(Taussig-Bing),and 8(7.8%) were DORV non-committed VSD.As for surgical approaches,76 patients(74.5%) underwent biventricular repair,19 cases(18.6%) underwent single ventricular repair,and 7 cases(6.9%) underwent palliative operation.Results There were 13 deaths in the whole patients studied(overall mortality = 12.7%),and the leading cause was postoperative low cardiac output syndrome.67 patients were followed up for 3-167 months(median = 50,interquartile range = 73).Reoperations were needed in 15 cases(15 /67,22.4%) with the majority indication for relief of ventricular outflow tract obstruction.Conclusion The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality.The need for reoperation remains a considerable challenge during the follow-up.Early and medium outcomes are satisfactory for patients with DORV.