肝静脉直接入心房的(功能)单心室患者的改良Fontan术式及早中期疗效

摘要

目的:总结肝静脉直接入心房(SHV)的(功能)单心室患者的改良Fontan术式及早中期临床结果.方法:回顾性分析2009-01至2015-12阜外医院对39例SHV的(功能)单心室患者施行改良Fontan术的临床资料.男26例(66.7%),平均年龄(6.98±3.62)岁,平均体重(20.79±9.66)kg,术前平均肺动脉压(mPAP)9.77±2.51(4~15)mmHg(1mmHg=0.133kPa).依据SHV患者是否合并下腔静脉与心尖同侧(ACJ),分为SHV组(n=29)和SHV+ACJ组(n=10).对术后早期(术后3个月)及中期(5年)进行临床结果分析.结果:两组患儿的临床资料比较差异无统计学意义(P>0.05).39例患者中,一期手术20例,二期手术19例(18例为Glenn术后,1例为B-T术后).选择的改良Fontan术式主要采取外管道—共同开口术式(30例,76.9%).19例患者建立体外循环并阻断主动脉,体外转流时间72~446 min,平均(189.74±84.96)min,主动脉阻断26~171min,平均(89.05±43.96)min;另19例患儿并行循环,体外转流时间60~209 min,平均(109.99±98.86)min.术后早期因并发症再次手术10例.术后院内死亡3例,死亡率7.7%.36例患儿平均随访时间3.90(0.83~7.17)年,35例心功能为Ⅰ级,1例为Ⅱ级,5年生存率100%.结论:SHV的(功能)单心室患者行改良Fontan术后早中期随访结果良好.而改良Fontan术式的选择应依据下腔静脉与肝静脉间的距离以及是否合并ACJ.%Objective: To summarize the early- and med-term outcomes of modified Fontan operation in patients with functional single ventricle and separate hepatic venous (SHV) drainage. Methods: A total of 39 patients with functional single ventricle and SHV drainage received modified Fontan operation in our hospital from 2009-01 to 2015-12 were retrospectively analyzed. There were 26 (66.7%) male, the average age was (6.98±3.62) years, body weight was (20.79±9.66) kg and pre-operative mean pulmonary artery pressure was (9.77±2.51) mmHg. Based on if SHV combining Apicocaval Juxtaposition (ACJ), the patients were divided into 2 groups: SHV group,n=29 and SHV+CAJ group,n=10. The early (3 months) - and med (5 years)-term post-operative outcomes were analyzed. Results: General clinical data was similar between 2 groups,P>0.05. There were 20/39 patients with one-stage operation, 19 with two-stage operation (18 after Glenn operation and 1 after B-T operation). Modified Fontan operation method was mainly used extracardiac conduit-common open technique (30 patients, 76.9%). 19 patients had CPB with aorta clamping, CPB time was 72-446 (189.74±84.96) min and aorta clamping time was 26-171 (89.05±43.96) min; the other 19 patients had CPB without aorta clamping, CPB time was 60-209 (109.99±98.86) min. 10 patients received re-surgery due to early post-operative complications. 3 patients died at early post-operation with the mortality at 7.7%. The average follow-up time was 3.9 (0.83-7.17) years, 1 patient had cardiac function at NYHA II and the others at NYHA I; the 5-year survival rate was 100%. Conclusion: The early- and med-term outcomes of modified Fontan operation were good in patients with functional single ventricle and SHV drainage. Selection of modified Fontan operation method should be based on the distance form inferior vena cava to SHV and presence of ACJ.