功能性单心室合并肝静脉单独回流入心房的全腔静脉-肺动脉连接术

Surgical management of total cavopulmonary connection procedure in functional single ventricle with separate hepatic venous drainage

目的探讨合并肝静脉单独回流入心房(separate hepatic venous drainage,SHVD)的功能性单心室患者行全腔静脉-肺动脉连接术(total cavopulmonary connection,TCPC)时肝静脉的处理,并对手术方式进行评价。方法回顾性分析2005年8月—2017年1月于广东省人民医院行TCPC术32例合并SHVD的功能性单心室患者的临床资料,其中男25例、女7例,平均年龄2~19(8.0±5.0)岁,平均体重11~66(25.4±15.8)kg。结果32例患者中,20例合并内脏异位综合征,其它类型单心室12例。27例术前明确诊断肝静脉异常回流,其中20例采用心内-心外聚四氟乙烯(Gore-Tex,GOTX)管道连接,另外7例采用心外GOTX管道连接;5例漏诊肝静脉单独回流入心房,均在术后早期出现血氧饱和度严重下降,随即再次手术。术后血氧饱和度92.0%(90.0%,96.0%),中心静脉压10~23(15.5±3.5)mm Hg,机械辅助通气时间16.0(7.5,24.0)h,ICU停留时间3.0(2.0,5.5)d。围术期死亡3例,随访期因脑干梗塞死亡1例。结论心内-心外管道是功能性单心室合并肝静脉单独回流入心房患者有效可行的改良TCPC术式,需结合个体解剖结构制定具体的手术细节。术前须避免漏诊SHVD,否则TCPC术后会出现低循环压力的肝静脉大量窃血入心房,导致不可耐受的低氧血症。

Objective To investigate the management of hepatic vein(HV)in patients with functional single ventricle(FSV)and separate hepatic venous drainage(SHVD)undergoing total cavopulmonary connection(TCPC)and evaluate this kind of surgery.Methods The clinical data of 32 consecutive patients with SHVD who underwent modified TCPC operation from August 2005 to January 2017 in our center were retrospectively analyzed.There were 25 males and7 females with an average age of 2-19(8.0±5.0)years and body weight of 11-66(25.4±15.8)kg.Results There were 20patients with heterotaxy syndrome and 12 patients with other types of FSV.SHVD was diagnosed preoperatively in 27patients,among whom 20 patients were connected by intra-extracardiac Gore-Tex conduit,and the other 7 patients were connected by extracardiac Gore-Tex conduit.Because of the missed diagnosis of SHVD,the other 5 patients showed severe decrease of blood oxygen saturation in the early postoperative period and underwent re-operation soon.The postoperative blood oxygen saturation was 92.0%(90.0%,96.0%),central venous pressure was 10-23(15.5±3.5)mm Hg,mechanical ventilation assisted time was 16.0(7.5,24.0)h,and ICU stay time was 3.0(2.0,5.5)d.There were 3 early and 1late deaths.Conclusion Intra-extracardiac conduit is an effective and feasible modified TCPC operation for patients with FSV and SHVD,while the surgical details need to be formulated in combination with individual anatomical structure.Preoperative missed diagnosis of SHVD must be avoided.Otherwise,after TCPC,a large amount of stealing blood from HV with low circulation pressure into atrium would lead to unacceptable hypoxemia.