Short-term And Long-term Prognosis And Application Of Fenestration In Patients Undergoing Total Cavopulmonary Connection

OBJECTIVE:Many studies had reported the long-term outcomes of Fontan patients in foreign centers.However limited study in China had reported that.As a result,this study was conducted to evaluat retrospectively the early and long-term results of 424 patients who underwent total cavo-pulmonary connection(TCPC)in Fuwai Hospital from January 2008 to December 2015,to detect the risk factors of the early death and long-term Fontan failure.METHODS:Collected the clinical data of patients who performed the Fontan operation in our hospital retrospectively,and followed up long-term outcomes of those patients by telephone or outpatient.Univariate and multivariate logistic regression was used to explore the risk factors of the early death,and Univariate and multivariate Cox regression models were used to and long-term Fontan failure.Then comparing the early outcomes,complications and Fontan failure among the age groups,dominant ventricle of right and left,one-stage TCPC and staged TCPC.RESULTS:There were 258 males(60.8%)in the cohort.The mean age is 9.2±6.9 years,and 382 patients(90.1%)performed the external conduit TCPC.The duration of hospitalization and duration of chest drainage was short in the fenestration group than those in the non-fenestration group(hospital stay:17.0 days vs.15.0 days,P=0.022;chest drainage time:13.0 days vs.8.0 days,P=0.002).There was no significant difference in the incidence of early death(P=0.671)and Protein-losing enteropathy(P=0.982),arrhythmia(P=0.447),heart failure(P=0.992),Fontan failure(P=0.618)among the fenestration group and non-fenestration group.Risk factors of early death was detected by univariate logistic regression analysis,we found that total atrioventricular septal defect(HR=5.8,95%CI:1.2-27.3,P=0.026),post-operative pulmonary artery pressure more than 18mmHg(HR=2.6,95%CI:1.4-16.9,P=0.004),ascites(HR=7.3,95%CI:1.7-31.5,P=0.008),application of ECMO(HR=48.1,95%CI:12.0-134.7,P=0.001)were predictors for early death.Univariate and multivariate Cox regression model was used to detected the risk factors of Fontan failure.We found that pre-operative pulmonary artery pressure ≥18mmHg(HR=5.2,95%CI:1.1-24.5,P=0.035),total atrioventricular septal defect(2.6,95%CI:1.0-6.3,P=0.042),postoperative atrioventricular valve regurgitation(HR=6.3,95%CI:2.0-14.7,P=0.005),renal dysfunction(HR=6.7,95%CI:1.6-28.99,P=0.011),ascites(HR=7.3,95%CI:3.4-15.9,P=0.020),protein-losing enteropathy(HR=6.4,95%CI:1.3-33.0,P=0.025),PLE(HR=6.4,95%CI:1.3-33.0,P=0.025)were independent predictors of postoperative Fontan circulation failure.CONCLUSION:Multivariate regression analysis showed that total atrioventricular septal defect,ascites,application of ECMO and post-operative pulmonary artery pressure were risk factors for early death.total atrioventricular septal defect,pre-operative pulmonary artery pressure,ascites,protein-losing enteropathy and thromboembolic events were risk factors of long-term Fontan failure.5-year and 10 year survival rate were 95.4%and 91.0%.One-stage TCPC and older age TCPC also can achieve an optimal long-term outcomes.OBJECTIVE:To evaluate the long-term effects of fenestration on patients at different risk levels in those performed external conduit total cavo-pulmonary connection operation,and explore the function in older patients and one-stage TCPC patients.METHODS:This is a retrospective analysis enrolled 383 patients underwent external conduit total cavo-pulmonary connection in Fuwai hospital from January 2008 to December 2015.Based on the preoperative data and whether fenestration in the operation,the whole cohorts were divided into four subgroups:high risk group with fenestration(mean age:10.53±7.06 years,male:55 cases),high risk group with non-fenestration(mean age:9.30±7.83 years,male:43 cases),low risk group with fenestration(mean age:8.91±7.13 years,male:65 cases)and low risk group with non-fenestration(mean age:8.23±5.34 years,male:67 cases).Then we collected and analysis the perioperative data and long-term prognosis of this cohorts in different risk levels.RESULTS:In the high-risk group,the duration of chest drainage in fenestration group was significantly shorter than that of the non-fenestration group(12.39±12.03 vs 23.30±15.36 days,p=0.001).The incidence of delayed chest drainage in the fenestration group was lower than that in the non-fenestration group(25%vs 47.1%,p=0.002).In addition,the length of hospital stay was shorter than that of the non-fenestration group(18.91±12.79 vs 29.68±37.77 days,p=0.004),with significant statistical difference.In the low risk group,there were 3(2.7%)and 2(1.6%)deaths at the follow-up in the non-fenestration and fenestration groups respectively,with p-value=0.761.And 1 case(1.3%),1 cases(1.4%)died in the fenestration and non-fenestration group,with p-value=0.593 in high risk group.However,there was no statistically significant difference among the fenestration and non-fenestration groups in terms of long-term intestinal protein loss syndrome and arrhythmia in different risk level groups.For younger patient with age<7yaers,fenestration could reduce the duration with mean 4 days(β=-4.3,95%CI:-8.5,-0.1P=0.045).Meanwhile,fenestration was functional in patients with pre-operative pulmonary more than 18 mmHg and McGoon ratio less than 1.5.CONCLUSION:Fenestration could reduce the incidence of early complications and hospital stay,effectively,especially for the patient with pre-operative pulmonary artery pressure ≥18mmHg or McGoon<1.5.Meanwhile,fenestration had no effects on early results and Fontan failure.At the same time,fenestration was recommended for patients with one-stage TCPC or older patients combine higher pulmonary artery pressure.BACKGROUND:Fenestration intervention was recommended after the Fontan palliation in some centers to avoid the potential suboptimal outcomes.Whereas,there are limited studies on the spontaneous fenestration closure.This study focus on the incidence of spontaneous fenestration closure and factors influencing the fate of fenestration.METHODS:A total of 202 patients performed fenestrated extracardiac conduit total cavo-pulmonary connection at the Fuwai Hospital from January 2008 and December 2015.According to the fate of fenestration at follow-up,192 fenestration patients was divided into spontaneous closure group(72 patients)and persistent patency group(120 patients).Multivariate Cox proportional-hazards analyses was used to explore the relationship between the spontaneous fenestration closure and variables at admission.Then Kaplan-Meier analysis was used to compare the Fontan failure at follow-up among two group.RESULTS:There were 202 patients performed extra-cardiac conduit fenestration total cavo-pulmonary connection in our center.10 patients were excluded from this study including 6 patients who performed fenestration closure and 4 patients with early death.The median age of patients received TCPC is 6.6 years and 6.5 years between two groups with P=0.599.72 patients(37.5%)occurred the fenestration closure spontaneously at the follow-up.The mean interval between the TCPC operation and fenestration closure was 3.81 years(Interquartile range:0.863 years to 5.228 years).Mean pulmonary artery pressure between fenestration patency group and fenestration closure group was 11.8±3.6mmHg and 12.2±3.4mmHg with P value=0.473.Among two group Nakata index and McGoon ratio was similar(Nakata Index:229.6 vs.216.0,P=0.694;McGoon ratio:1.8±0.6 vs.1.9±0.5,P=0.543).There was no significant difference on late mortality and Fontan failure between fenestration patency group and fenestration closure group by Kaplan-Meier analysis.Based on the multivariable Cox proportional-hazards analyses,the fenestration would prone to be patent in patients with postoperative pulmonary artery pressure more than 18 mmHg(HR=0.93 95%CI:0.07-1.00,P=0.049).Better dominant cardiac ejection fraction and prior Glenn operation would facilitate the fenestration closed spontaneously(1.05,95%CI:1.00-1.09,P=0.033HR=1.05,95%CI:1.00-1.09,P=0.003;prior Glenn operation:HR:2.00,95%CI:1.23-3.27,P=0.005).CONCLUSION:The incidence of spontaneous fenestration closure is 37.5%in this study.Prior Glenn operation and better’ cardiac ejection fraction are predictor of spontaneous fenestration closure.Fenestration would be patency for the patient with postoperative pulmonary artery pressure more than 18 mmHg.For the long-term outcomes as mortality and Fontan failure were similar among the fenestration closure group and fenestration patency group.