大于6月龄室间隔完整型肺动脉闭锁患者的外科治疗策略和中远期随访分析

Surgical strategy and mid-and long-term follow-up analysis of the patients older than 6 months with pulmonary atresia and intact ventricular septum

目的本研究总结及分析近十年接受首次外科干预时年龄已超过6个月龄室间隔完整型肺动脉闭锁(PA/IVS)患者的外科治疗策略。方法回顾2007年1月至2018年12月,上海儿童医学中心共收治63例年龄超过6个月的PA/IVS患者,其首次干预时的年龄为6.2~79.6(20.1)个月,体重为4.6~17.5(9.5)kg,将其中51例已获终期治疗的患者分为3组,包括双心室组11例、一个半心室组18例和单心室组22例,并对各组右室发育程度、术后生存率、再次手术干预率及中远期心功能状况进行比较分析。结果术后随访时间为0.6~11.9(5.5)年,失访5例,死亡3例(5.9%,3/51)。其中,双心室组右室发育不良均为轻或中度(中度占45.5%),一个半心室组以中度为主(中度占94.4%),单心室组则为重度为主(中度占13.6%),各组三尖瓣Z值比较具有统计学差异(P<0.01)。双心室组、一个半心室组和单心室组大多数患者中远期随访结果良好,各组术后十年免再手术干预率分别为75.8%、85.2%和76.8%(P>0.05),心功能分级大多数患者均为Ⅰ或Ⅱ级,仅一个半心室1例和单心室组2例为Ⅲ级。结论大年龄PA/IVS患者由于右心室生长潜能受到限制而导致较大比例患者失去双心室矫治机会,但依据右室发育不良程度选择个性化的手术方案,最终都可获得较低死亡率,且中远期生存质量良好。

Objective To summarize and evaluate the surgical strategy of the patients older than 6 months with pulmonary atresia and intact interventricular septum(PA/IVS)at the time of the first surgical intervention in the past decade at our center.Methods From January 2007 to December 2018,63 patients with PA/IVS older than 6 months were treated.The median age and median weight at the first intervention were 20.1 months(range,6.2-79.6 months),and 9.5 kg(range,4.6-17.5 kg)respectively.51 cases received definitive repair were divided into three groups:biventricular repair group(n=11),one and a half ventricular repair group(n=18)and univentricular repair group(n=22).The development of right ventricular,postoperative survival rate,reintervention rate,as well as medium-and long-term cardiac function were compared and analyzed.Results Median follow-up time was 5.5 years(range,0.6-11.9 years)with 5 cases lost to follow-up and 3 cases died(5.9%,3/51).The development of right ventricular was mild or moderate in biventricular group(moderate 45.5%),mainly moderate in one and a half ventricular group(moderate 94.4%),and mainly severe in univentricular group(moderate 13.6%).There was significant difference in Z value of tricuspid valve among each group(P<0.01).Among biventricular group,one and a half ventricular group and univentricular group,10-year intervention-free rates were 75.8%,85.2%and 76.8%,respectively(P>0.05).The medium-and long-term follow-up results of most patients were good.The cardiac function grading of most cases were gradeⅠorⅡ,except that one case in one and a half ventricular group and two cases in univentricular group were gradeⅢ.Conclusion Due to the limitation of right ventricular growth potential,a large proportion of older PA/IVS patients lose the opportunity of biventricular repair.According to the degree of right ventricular dysplasia,individualized surgical strategy could achieve lower mortality and good quality of life in the medium and long term ultimately.