Modified Fontan's Early And Mid-term Outcomes In Patients With (functional) Single Ventricle With Abnormal Inferior Vena Cava Drainage

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.%.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.Objective:Modifications of the Fontan operation are applied for patidents with functional single ventricle(FSV),we summed up the pathways and the clinical results in these FSV patients with apicocaval juxtaposition(ACJ)or separated hepatic venous drainage(SHV).Methods:Of the 78 patients with FSV and anomalous inferior vena cava(IVC)who underwent modified Fontan operation between January 2009 and December 2015,39 patients with ACJ(group 1)and the other 39 patients with SHV(group 2)were included in this retrospective study.And the group 2 included 10 cases with ACJ(25.6%).In group 1,there were three kinds of modified Fontan operation methods:29 cases of extracardiac conduit-total cavopulmonary connection(EC-TCPC),9 cases of intracardiac conduit-total cavopulmonary connection(IC-TCPC),one of lateral tunnel-total cavopulmonary connection(LT-TCPC);the concomitant procedures included atrioventricular valve plasty in 4 cases,ligation of PDA in 3 cases,closure of aortopulmonary collateral arteries in one,pulmonary arterioplasty in one.In group 2,there were four kinds of modified Fontan operation methods:30 cases of EC-TCPC-common open technique,2 cases of intra-extracardiac conduit-total cavopulmonary connection(IEC-TCPC),4 cases of LT-TCPC,3 cases of IC-TCPC;the concomitant procedures included atrioventricular valve plasty in 7 cases,ligation of PDA in 2 cases,repair of TAPVC in one,ASD enlargement in one.Results:There were one patient in group 1 and 11 patients in group 2 required re-operation at the time of hospitalization(P<0.01),other clinical date between the two groups had no significant difference.The early-term survival rate was 100%in group 1 and 92.3%in the group 2.The mean follow-up period was 4.05 years(0.5-7.5 years).The cardiac function of NYHA I was 100%in group 1 and 97.1%in group 2 during follow-up period.The survival rate at 5 years was 100%in group 1 and 97.2%in group 2.Postoperative mean,pulmonary artery pressure(mPAP)>15mmHg emerged as a predictor for early reoperation(P<0.01)and early death(P<0.001)in univariate analysis.Conclusions:Modified Fontan operation can be performed in patients with ACJ or SHV with good early and mid-term results.In addition to the cri ter ions of routine Fontan operation,the distance between the IVC and the vertebrae,the apex for the patients with ACJ and the distance between the IVC and the SHV,the ACJ for the patients with SHV should be considered when choosing a surgical technique.And the technique should be altered with the postoperative mPAP greater than 15mmHg.