Extracardiac Conduit Total Cavopulmonary Connection Research Articles

Anesthetic management for withdrawal from a right ventricular assist device and Fontan procedure in a patient with an implantable left ventricular assist device for fulminant cardiomyopathy

BackgroundWe herein report the anesthetic management for extracardiac conduit–total cavopulmonary connection (EC-TCPC) for weaning from an extracorporeal right ventricular assist device (RVAD) in a patient with an implantable left ventricular assist device (LVAD) for fulminant cardiomyopathy.Case presentationA 24-year-old man developed fulminant cardiomyopathy and was placed on a biventricular assist device (BiVAD) comprising an implantable LVAD and an extracorporeal RVAD. The Fontan procedure was performed to wean the patient from the RVAD and allow him to be discharged home. Atrial septal defect creation, right ventricular suture, and tricuspid valve closure were then simultaneously performed to ensure sufficient left ventricular preload to drive the LVAD. Furthermore, to keep the central venous pressure lower, the inflow cannula of the LVAD was oriented in the correct direction.ConclusionThis is the first report of anesthetic management of the Fontan procedure in a patient with a BiVAD.

Commentary: Extracardiac conduit-total cavopulmonary connection for heterotaxy-Is going the "extra" mile worth it.

Extracardiac conduit-total cavopulmonary connection (EC-TCPC) represents the latest evolution in Fontan palliation for single ventricle physiology. Here, we review a manuscript by Pan et al. in the Journal of Cardiac Surgery detailing a retrospective, single-center cohort study of patients with and without heterotaxy syndrome undergoing EC-TCPC. Although lacking a comparison to other techniques for single ventricle palliation, this approach appears to offer excellent outcomes for this complex patient cohort.

Comparable long-term results of the extracardiac conduit total cavopulmonary connection in heterotaxy to non-heterotaxy patients.

Fontan operation in heterotaxy patients has been associated with high mortality. We studied whether adoption of the extracardiac conduit (EC) total cavopulmonary connection (TCPC)in heterotaxy demonstrated comparable results to non-heterotaxy population. A retrospective medical record review of 35 consecutive patients with heterotaxy and 70 consecutive patients without heterotaxy syndrome who underwent EC TCPC between 2000 and 2018 was performed. In the 35 heterotaxy patients, 30 were right and 5 were left atrial isomerism. Anomalies of venous return included bilateral superior vena cava in 20 (57.1%), separated hepatic vein in 8 (22.9%), interrupted inferior vena cava in 3 (8.6%), total anomalous pulmonary venous return in 7 (20%), and partial in 2 patients (5.7%). All patients underwent EC TCPC under beating-heart cardiopulmonary bypass except in four patients (11.4%) cardioplegic arrest was needed for cardiac repair. The surgical mortality rate was lower in heterotaxy patients (0% vs. 5.7%; p = 0.299) but statistically not significant. The follow-up ranged from 2 months to 17.8 years (mean 9.4 ± 5.6 years). At 15 years, there was no significant difference between the heterotaxy and non-heterotaxy patients regarding the long-term survival (70% vs. 78.6%; p = 0.443), freedom from reoperation (81.9% vs. 96.5%; p = 0.057), and postoperative arrhythmia (17.1% vs. 7.1%; p = 0.174). EC TCPC can be performed in heterotaxy patients with comparable early and late results to the non-heterotaxy population. However, the late morbidities regarding the Fontan circulation needs careful follow-up.

Intra-Extracardiac Total Cavopulmonary Connection for Patients With Anatomical Complexity

Currently, the extracardiac conduit total cavopulmonary connection (eTCPC) is the most widely used for Fontan modification worldwide. Nevertheless, there have been some cases that are difficult for performing eTCPC because of their anatomical complexity, such as apicocaval juxtaposition. For such cases, in 2002, we introduced the intra-extracardiac TCPC (ieTCPC). We reviewed our 20-year single-center experience with 316 TCPC patients to compare eTCPC (n= 277) and ieTCPC (n= 39) in terms of mortality and morbidity. ieTCPC was indicated for the cases in which there was concern that the TCPC conduit would be too curved for ordinary eTCPC. Early death occurred in 1 patient and late death occurred in 15 patients. The actuarial survival rate in the eTCPC and the ieTCPC groups at 10 years were 95.1% and 100.0%, respectively. There was no significant difference in actuarial survival between eTCPC and ieTCPC patients. In the multivariate analysis, preoperative superior vena cava pressure and preoperative oxygen saturation were found to be the independent predictor for postoperative mortality. There was also no significant difference in actuarial rate of freedom from late-occurring complications between eTCPC and ieTCPC groups. In the multivariate analysis, dominant right ventricle and preoperative SVC pressure were independent predictors for late-occurring complications. The clinical outcomes in patients who undergo eTCPC and ieTCPC appear to be excellent, with low mortality and morbidity rates in the midterm. ieTCPC may be a good option for TCPC cases with anatomical complexity such as apicocaval juxtaposition and separated hepatic vein drainage.

Noninvasive Assessment of Pulmonary Artery Pressure in Patients with Extracardiac Conduit Total Cavopulmonary Connection.

To assess quantitative measurement of mean pulmonary artery pressure (PAP) in extracardiac total cavopulmonary connection (TCPC) patients by noninvasive echocardiographic inferior vena cava collapsibility index (IVC-CI) and also correlation between the peripheral vein pressure and mean PAP. In 19 TCPC patients with at least 1-year follow-up after completion of TCPC, complete echocardiography including IVC-CI was recorded. All patients underwent cardiac catheterization for mean PAP pressure, peripheral vein pressure (PVP) and contrast study. Different cutoff points of mean PAP were analyzed, and based on the highest cutoff point, patients were categorized into two groups: mean PAP<17mmHg (acceptable) and >=17mmHg (high) for comparison analysis. Between 2006 and 2015, from 43 patients who had undergone TCPC, nineteen patients with a mean age 12.9±6.6years and mean follow-up time of 3.4±1.9years were included in this study. Mean PAP was 14.6±3.97 (range 8-22mmHg). Thirteen (68%) patients had PAP<17mmHg. No significant statistical difference was detected with respect to age, sex, type of congenital heart disease and fenestration between these two groups. But, right ventricular dominancy was more prevalent in the high PAP group (50% vs. 7.7% P value 0.03). IVC-CI had a correlation with mean PAP (r 0.67, P<0.001). IVC-CI<21.8% can predict PAP>=17mmHg with 83% sensitivity and 100% specificity. Regression analysis proposed an equation for PAP measurement: PAP (mmHg)=20.2097-0.1796×(IVC-CI), (r2=0.56). Peripheral vein pressure measurement also showed a good correlation with mean PAP and may be used to estimate PAP with the following equation: PAP (mmHg)=0. 8675×PVP, (r 0.90, P<0.0001). In conclusion, IVC-CI as noninvasive and peripheral vein pressure measurement as a minimal invasive method may be useful for quantitative estimation of PAP in patients with extracardiac TCPC.

Results of extracardiac conduit total cavopulmonary connection in 500 patients.

This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included protein-losing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from late-occurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R(2) = 0.32) and elapsed time since operation (R(2) = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m(2) and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for ≥10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients. The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

Outcomes of One-Lung Fontan Operation: A Retrospective Multicenter Study in Japan

The Fontan operation for patients with one available lung is an extremely challenging situation. However, few reports are available on this procedure. The purpose of this study was to describe outcomes of one-lung Fontan operation. A retrospective multicenter study was performed. Twelve of 1,142 patients whose data were recorded here underwent one-lung Fontan operation between September 1989 and October 2009. Preoperative, operative, and postoperative data were reviewed. Median age at operation was 3.5 years (range, 1.0 to 22.8), the preoperative mean pulmonary pressure was 11.5±3.3 mm Hg (range, 7.0 to 18.0), the ventricular ejection fraction was 58%±13% (range, 39 to 76), and end-diastolic ventricular pressure was 7.5±3.5 mm Hg (range, 1.0 to 12.0). The available lung was right in 9 patients and left in 3 patients. Eleven patients underwent a two-staged Fontan completion. Extracardiac conduit total cavopulmonary connection, intraatrial extracardiac conduit total cavopulmonary connection, and atriopulmonary connection were performed in 10 patients, 1 patient, and 1 patient, respectively. The estimated actuarial survival was 83% at 1 year, 73% at 5 years, and 73% at 10 years. Impaired ventricular function was found to be a significant risk factor for mortality by univariate analysis (43.0%±9.5% versus 64.0%±9.5%, p<0.01), but not by multivariate analysis. One-lung Fontan operation can be performed with an acceptable midterm to long-term mortality rate in patients without impaired ventricular function. Thus, absence of one lung itself is not a contraindication to the Fontan operation.

The impact of extracardiac conduit-total cavopulmonary connection on apicocaval juxtaposition☆

Modifications of the Fontan procedure are applied to a wide range of complex congenital heart defects with single ventricle physiology. We examined the pathway and the clinical results of extracardiac conduit-total cavopulmonary connection (EC-TCPC) for a malpositioned heart with apicocaval juxtaposition. Of the 365 patients who underwent EC-TCPC since 1994, 56 patients with a malpositioned heart with apicocaval juxtaposition were included in this retrospective study (group 1). The pathway for the EC was selected after careful consideration of the results of preoperative angiography and computed tomography, as well as intra-operative findings. A concurrent group of 299 patients undergoing EC-TCPC without apicocaval juxtaposition was used as a control group (group 2). The mean follow-up periods for groups 1 and 2 were 5.5 years (range: 0-12 years) and 5.5 years (range: 0-14 years), respectively. The mean age at operation was 4.2±3.2 years and the median size of the conduit was 18 mm (range: 16-20mm). In 30 patients, the conduit was placed between the inferior vena cava (IVC) and the opposite side of the pulmonary artery crossing the vertebra. In another 25 patients, the conduit was positioned behind the ventricle between the IVC and on the same side as the pulmonary artery (PA). There was one patient who had a Y-style conduit placed between the IVC and right and left PAs behind the ventricle. In group 1, there were no early deaths; three patients died in the intermediate term due to gastric bleeding in one, haemoptysis in another and sudden death in yet another. None of the patients developed conduit stenosis or pulmonary venous obstruction, and no patient required re-operation. Three patients developed late complications, including arrhythmias requiring medication in two and subdural haematoma in one. The incidence of death or late complications did not differ among the pathways of conduits. In group 2, there were eight late deaths, and 27 patients developed late complications. The Kaplan-Meier survival rate was 93.5% at 5 and 10 years in group 1, and 97.3% at 5 years and 96.1% at 10 years in group 2 (log-rank test, P=0.29). The haemodynamics in groups 1 and 2 during the intermediate term were identical with respect to IVC pressure (8.9 ± 2.5 in group 1 and 9.6 ± 2.6mmHg in group 2), left ventricular end-diastolic pressure (4.4 and 4.3 mmHg, respectively), cardiac index (3.3 and 3.4 l min(-1)m(-2), respectively) and arterial oxygen saturation (94 and 94.2%, respectively). No patient in either group had a pressure gradient >2 mmHg between the IVC and central PA. Postoperative catheterisation data showed no significant differences in haemodynamics between the conduit pathways in group 1. EC-TCPC can be performed in children with apicocaval juxtaposition with excellent mid-term outcomes compared with other Fontan candidates. Based on individual cardiac anatomy, the pathway of the EC behind the ventricle or crossing the vertebra can be used without conduit stenosis or pulmonary venous obstruction.

Excellent Midterm Outcome of Extracardiac Conduit Total Cavopulmonary Connection: Results of 126 Cases

Extracardiac conduit total cavopulmonary connection has shown good early results; however, its long-term outcome has yet to be reported. Of 282 patients who underwent extracardiac conduit total cavopulmonary connection since 1994, 126 patients who have been followed up for more than 5 years were included in this retrospective study. Actuarial survival rate, incidence of late complications, hemodynamic variables, and results of exercise tolerance test were reviewed. Follow-up time was 96.4 +/- 23.0 months. There was 1 operative death and 6 late deaths. Actuarial survival rate was 95.2% and 93.6% at 5 and 10 years, respectively. Seven patients had late complications including new-onset supraventricular arrhythmia in 3, protein-losing enteropathy in 2, thromboembolism in 1, and bleeding complication in 1. Seven patients underwent reoperation not related to the conduit. Freedom from Fontan-related events was 88.8% at 5 years and 84.3% at 10 years. Late cardiac catheterization in 119 survivors showed central venous pressure of 9.9 +/- 2.9 mm Hg, cardiac index of 3.6 +/- 0.8 L x min(-1) x m(-2) and arterial oxygen saturation of 94.5 +/- 2.3%. No patient showed conduit stenosis. Plasma concentration of atrial and brain natriuretic peptide (pg/mL) were 28.9 +/- 20.0 and 25.8 +/- 44.5. Exercise test performed in 101 patients showed endurance time of 75.7 +/- 12.9% of normal value, peak heart rate of 92.3 +/- 14.4% of normal, and peak oxygen consumption of 90.0 +/- 20.0% of normal. The latest echocardiogram showed ejection fraction of 60.4 +/- 11.7%. Three patients had pacemaker rhythm, 1 had junctional rhythm, and 115 patients had sinus rhythm. Midterm outcome of extracardiac conduit total cavopulmonary connection was satisfactory with low incidence of late mortality and morbidity, and excellent hemodynamic state.

Surgical treatment of complex congenital heart diseases with extracardiac conduit total cavopulmonary connection

To report the experience of extracardiac conduit total cavopulmonary connection (ECTCPC) in surgical treatment of complex congenital heart diseases. From 1998 to 2006, 68 patients underwent ECTCPC for complex congenital heart diseases. Among them, 45 had functional univentricle with transposition of the great artery (TGA) and pulmonary artery valve stenosis, 19 had tricuspid atresia with hypoplasia of right ventricle, 4 had Ebstein's anomaly with hypoplasia of right ventricle. Six had left superior vena cava, 18 had received Bidirectional Glenn operation; Fifty-seven cases were performed under cardiopulmonary bypass with general anesthesia and hypothermia, 11 cases were performed without cardiopulmonary bypass. There were two death, the mortality was 2.9%. All patients were followed up from 1 to 8 years with no clinical symptoms and have been doing well. The arterial oxygen saturation was 90% - 96%, the cardiac function were in NYHA class I - II. The extra cardiac conduit TCPC is a simple procedure and superior to other type of Fontan procedure in most patients.

Study of cardiopulmonary adaptation during exercise in patients after extracardiac conduit total cavopulmonary connection

To study cardiopulmonary physiology during exercise in patients after extracardiac total cavopulmonary connection (ECTCPC). Twenty-six patients were studied after ECTCPC by exercise testing with bicycle treadmill protocol. Heart rate (HR), blood pressure (BP), respiratory frequency (RF) and pulse oxygen saturation (SpO(2)) were measured continuously; twenty-six patients suffered from Fallot 4 underwent biventricular repair were also studied as control group. In ECTCPC group, HR, BP, SpO(2) and RF all increased with exercise below 3 grade; when exceed 4 grade, BP, SpO(2) decreased and RF kept increasing. Compared with control group, HR, RF were higher (t = 2.13, P < 0.05; t = 2.31, P < 0.05), SpO(2) was lower (t = 2.46, P < 0.05) under the quiescent condition; When exceed 3 grade, HR, BP, SpO(2) decreased more significantly, but RF increased continuously. In fenestration group after ECTCPC, HR reached the top at 5 grades, but in group without fenestration it reached the top at 3 grades; In the whole process of exercising, RF kept higher and SpO(2) kept lower in fenestration group. The ECTCPC patients showed obviously exercise limitation. Totally bypass of sinoatrial node in this operation may have some adverse effects on the integer regulation of HR.

Midterm surgical results of total cavopulmonary connection: clinical advantages of the extracardiac conduit method

Objective We evaluated the midterm surgical outcomes of intra-atrial lateral tunnel and extracardiac conduit total cavopulmonary connection to clarify the clinical superiority. Methods Patients (n = 167) underwent total cavopulmonary connection (88 with lateral tunnel and 79 with extracardiac conduit) from November 1991 to March 1999. Survival, incidence of reoperation and late complications, exercise tolerance, hemodynamic variables, and plasma concentration of natriuretic peptide type A were compared. In the lateral tunnel group, time-related change in lateral tunnel size was investigated for its relationship to postoperative arrhythmias. Results The 8-year survival was 93.2% in the lateral tunnel group and 94.9% in the extracardiac conduit group. Seven reoperations were performed in the lateral tunnel group but none in the extracardiac conduit group. Supraventricular arrhythmias developed in 14 patients (15.9%) in the lateral tunnel group and in 4 patients (5.1%) in the extracardiac conduit group ( P = .003). Freedom from cardiac-related events was 72.5% in the lateral tunnel group and 89.8% in the extracardiac conduit group at 8 years ( P = .0098). Hemodynamic variables and exercise tolerance were similar in both groups but plasma natriuretic peptide type A concentration, a parameter of atrial wall tension, was higher in the lateral tunnel group. In the lateral tunnel group, intra-atrial tunnel size increased by 19.4% during the 44.2-month interval and the percent increase in tunnel size was an independent predictor of supraventricular arrhythmias. Conclusions The midterm survival, hemodynamic variables, and exercise tolerance were similar and satisfactory in both lateral tunnel and extracardiac conduit groups; however, the incidence of cardiac-related events was significantly less frequent in the extracardiac conduit group. In the lateral tunnel group, careful observation is required to monitor the relationship of the dilating tendency of the intra-atrial tunnel and the development of late complications.