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Ann Thorac Surg 2007;83:622-630
© 2007 The Society of Thoracic Surgeons


Original Articles: Cardiovascular

Fontan Operation: A Comparison of Lateral Tunnel with Extracardiac Conduit

Andrew C. Fiore, MDa,b,*, Mark Turrentine, MDa,b, Mark Rodefeld, MDa,b, Palaniswamy Vijay, PhDa,b, Theresa L. Schwartz, MDa,b, Katherine S. Virgo, PhDa,b, Laurice K. Fischer, MDa,b, John W. Brown, MDa,b

a Divisions of Cardiothoracic Surgery, St. Louis University School of Medicine/Cardinal Glennon Children’s Hospital, St. Louis, Missouri
b Indiana University School of Medicine/James Whitcomb Riley Children’s Hospital, Indianapolis, Indiana

Accepted for publication September 19, 2006.

* Address correspondence to Dr Fiore, St. Louis University Health Sciences Center/Cardinal Glennon Children’s Hospital, 1465 S Grand Blvd, St. Louis, MO 63104 (Email: fiorem2{at}slu.edu).

Presented at the Forty-first Annual Meeting of The Society of Thoracic Surgeons, Tampa, FL, Jan 24–26, 2005.


    Abstract
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
BACKGROUND: The purpose of this report is to compare the outcome of the extracardiac (EC) with the lateral tunnel (LT) Fontan.

METHODS: From January 1990 to October 2004, the Fontan operation was performed in 162 patients, of which 49 were EC and 113 were LT. Cardiac morphology and ventricular dominance were similar, except EC patients were older and had a greater frequency of heterotaxy syndrome, and LT patients had a higher incidence of hypoplastic left heart syndrome. Preoperative transpulmonary gradient, ventricular end-diastolic pressure, McGoon index, room air saturation, and cardiac rhythm were similar. EC patients underwent superior caval pulmonary connection, and LT patients underwent hemi-Fontan. Cardiopulmonary bypass time was similar, but fewer EC patients needed aortic cross-clamping. Fenestration was more frequent in LT patients (EC, 16% versus LT, 73%; p < 0.01).

RESULTS: Overall operative mortality was 1.8% (EC, 1 versus LT, 2; p = NS). Postoperative transpulmonary gradient, readmission for chylous effusion, and change in ejection fraction relative to preoperative level did not differ between cohorts. Resource utilization was higher in the EC group. The loss of sinus rhythm and the frequency of all neurologic events did not differ. There were seven late deaths (EC 4 versus LT 3; p = NS). Actuarial survival at 5 years was not significantly different (EC, 90% versus LT, 95%; p = 0.08).

CONCLUSIONS: The EC and LT operation had comparable early and late mortality, readmission for chylous effusion, preservation of sinus rhythm, and frequency of all neurologic events. The more frequently fenestrated LT cohort used fewer resources.


    Introduction
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
Most patients born with a univentricular heart undergo a staged palliation towards a goal of total caval pulmonary connection. The superior caval pulmonary connection is usually performed first and consists of either a bidirectional superior caval pulmonary anastomosis or a hemi-Fontan procedure. The inferior caval pulmonary connection is subsequently performed within 1 to 3 years. At the present time, the two modifications currently used to direct inferior vena cava blood to the pulmonary arteries are the lateral tunnel or the extracardiac conduit operation.

The extracardiac conduit procedure has several potential advantages, including the preservation of normal atrial pressure, the absence of extensive intraatrial suture lines, the avoidance of cardiopulmonary bypass (CPB), assuming no intracardiac repairs are needed, and the feasibility of early or late fenestration. This may reduce the frequency of early and late arrhythmias and preserve pulmonary and cardiac function. However, this procedure has potential disadvantages related to using the extracardiac tube, including the lack of growth potential, conduit stenosis owing to intimal peel formation, an increased risk of thromboembolism and the need for reoperation [1].

The lateral tunnel procedure contains several intraatrial suture lines and requires either fibrillatory arrest or aortic cross-clamping, which may be associated with increased atrial arrhythmias and global single ventricular dysfunction [2].

Physical and mathematic models have confirmed the hemodynamic benefits of both procedures [3, 4]. This purpose of this retrospective study was to compare both surgical techniques to achieve total caval pulmonary connection with respect to early and intermediate outcomes. The particular emphasis of this comparison is directed towards postoperative hemodynamics, arrhythmias, the late development of chylous effusions, and neurologic events.


    Patients and Methods
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
The medical records of all patients undergoing a Fontan procedure from January 1, 1990, to December 31, 2004, at James Whitcomb Riley Children’s Hospital at Indiana University, Indianapolis, Indiana, and Cardinal Glennon Children’s Hospital at St. Louis University, St. Louis, Missouri, were reviewed. The Institutional Review Boards (IRBs) at St. Louis University and Indiana University approved this study, and both IRBs waived the need for patient consent.

The analysis excluded conversions of previous atrial pulmonary connections and patients aged older than 10 years. The study population includes 162 Fontan procedures of which 113 were lateral tunnel and 49 were extracardiac conduits. At Indiana University, 110 patients received the lateral tunnel (LT group) and 16 the extracardiac conduit (EC group), whereas at St. Louis University, 3 patients received the lateral tunnel and 33 the extracardiac conduit Fontan. This indicates an institutional bias for the lateral tunnel operation at Indiana University and the extracardiac conduit at St. Louis University.

Demographic information, cardiac anatomy, pre-Fontan hemodynamics, operative details, and postoperative outcomes were recorded from patient records. Transthoracic echocardiography was used to estimate ejection fraction and degree of atrioventricular (AV) valve insufficiency. Ejection fraction was calculated by using Simpson’s rule [5]. Mild AV valve regurgitation was defined as a narrow regurgitant jet reaching to the mid right atrium; moderate regurgitation was a wider jet with a narrow rim of proximal, isovolumic acceleration, and severe regurgitation was a longer and wider jet reaching to the posterior right atrial wall, with a wide rim of proximal isovolumic acceleration [6].

Postoperative cardiac rhythm was assessed on 12-lead electrocardiograms (ECG). Sinus node dysfunction was defined as a need for temporary or permanent atrial pacing, a heart rate greater than 2 standard deviations less than the age-adjusted mean, or junctional escape rhythm [7]. Supraventricular tachycardia was defined as nonsinus, narrow complex tachycardia, including junctional ectopic tachycardia and atrial flutter. Dysrhythmia was scored as any rhythm disturbance (as defined previously), or the need for an ablation procedure or a pacemaker at the time of follow-up.

Recurrent chylous effusions were defined in a patient who required readmission to the hospital any time after the Fontan operation with a pleural fluid collection demonstrated by chest roentgenogram or ultrasound evaluation that required thoracentesis or chest tube placement alone or in combination with total parenteral nutrition.

The definitive diagnosis of any postoperative neurologic event was made in consultation with a pediatric neurologist, including physical examination, electroencephalograph recording, and brain imaging studies (ultrasound, computed tomography or magnetic resonance imaging). A neurologic event was defined as a seizure, a transient ischemic attack, a subarachnoid or intracranial hemorrhage, or a complete stroke, and was scored at discharge after the Fontan operation and at the last follow-up.

Early mortality was defined as death in the hospital or within 30 days of discharge. All other mortality was considered late.

Patient’s families, cardiologists, and primary care physicians were contacted when necessary to acquire current follow-up information. Follow-up was achieved in 151 (95%) of 159 hospital survivors. Eight patients in the LT group were lost to follow-up. The lateral tunnel operation was initiated earlier in time and was associated with a longer mean follow-up of 5.0 ± 3.1 years compared with 3.0 ± 2.3 for the extracardiac conduit procedure (p < 0.01).

Operative Technique
The operative data are summarized in Table 1. The decision to use the lateral tunnel or extracardiac conduit procedure was based on the patient’s anatomy and the surgeon’s preference, without randomization.


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Table 1. Demographics
 
All Fontan operations were performed using median sternotomy, standard CPB, and mild hyperthermia (30°C to 32°C). The superior caval pulmonary connection and the inferior vena cava were routinely cannulated. If aortic cross-clamping was used, myocardial protection was by cold, intermittent, antegrade blood cardioplegia. Ventricular fibrillation was not used in any patient.

All patients underwent a primary superior caval pulmonary connection consisting of either a bidirectional superior caval pulmonary anastomosis (bidirectional Glenn) or a hemi-Fontan procedure. The latter was constructed by sewing the cranial end of the divided superior vena cava to the superior aspect of the right pulmonary artery and the cardiac end to the inferior aspect of the right pulmonary artery. The superior vena cava–right atrial orifice was closed within the right atrium by using a circular patch of a Gore-Tex polytetrafluoroethylene (PTFE) (W. L. Gore and Associates, Flagstaff, AZ). If a left superior vena cava was present, it was routinely anastomosed to the left pulmonary artery. All patients underwent modified ultrafiltration after separation from CPB.

Associated conditions such as pulmonary artery stenosis and moderate-to-severe AV valve regurgitation were repaired at the time of superior caval pulmonary connection to avoid the need for additional procedures at the time of the Fontan operation.

Lateral Tunnel
After removal of the circular Gore-Tex patch at the superior vena cava right atrial orifice, the inferior vena cava blood was routed along the lateral aspect of the right atrium to the superior caval pulmonary connection with a Gore-Tex patch sutured posteriorly to the edge of the atrial septal defect and anteriorly to the edge of the atrial wall. The atriotomy was closed by incorporating the patch within the suture line. A fenestration was created in the patch using an aortic punch and sized with Hegar dilators.

Extracardiac Conduit
This operation was performed in patients weighing 13 to 16 kg to allow for a larger conduit. A Gore-Tex tube 18 to 20 mm in diameter was sewn to the divided inferior vena cava and to the undersurface of the right and left pulmonary artery junction. A fenestration was placed by interposing a small Gore-Tex tube between the extracardiac conduit and the right atrium.

The indications for fenestration included (1) hypoplastic left heart syndrome (HLHS), (2) a post-Fontan transpulmonary gradient exceeding 12 to 15 mm Hg, and (3) any preoperative variable such as small pulmonary arteries that could potentially increase the risk of the total caval pulmonary connection.

All patients underwent modified ultrafiltration. At the time of sternal closure, tubes were inserted into both pleural spaces and in the mediastinum and were removed when the daily drainage was less than 2 mL/kg. Monitoring lines were removed within 24 to 36 hours. These included a superior or inferior vena caval catheter, usually inserted percutaneously after anesthetic induction, and a right atrial catheter placed intraoperatively.

Anticoagulation
All patients were managed with intravenous heparin postoperatively and placed on Coumadin (Bristol-Myers Squibb, Princeton, NJ) therapy at the time of discharge. Patients remained on Coumadin therapy as long as the fenestration was patent. Aspirin was initiated at the time of fenestration closure at a daily dose of 10 mg/kg. Nonfenestrated patients remained on Coumadin therapy for 1 year and then lifetime aspirin. The international normalized ratio (INR) was maintained at 1.5 to 2.0.

Statistical Analysis
Categoric data are presented as percentages accompanied by the number of cases. Continuous data are presented as medians with ranges and means ± standard deviations, as appropriate. Categoric characteristics and outcomes of patients having the lateral tunnel versus the extracardiac Fontan connection were compared using the Fisher exact test or {chi}2 test, as appropriate. Continuous characteristics and outcomes were compared using t tests. Time from surgery to death or last known follow-up visit was analyzed using the Kaplan-Meier method. A value of p < 0.05 was considered significant. All analyses were performed using SPSS (SPSS Inc, Chicago, IL) software.


    Results
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
Demographics and Hemodynamics
The demographic and morphologic characteristics of the study population are summarized in Table 1. The body surface area and size of the EC group were significantly higher to accommodate the largest possible extracardiac conduit. The ventricular dominance and variations in cardiac morphology were similar between cohorts, but the incidence of HLHS was significantly higher in the LT cohort. Heterotaxy syndrome and dextrocardia were significantly associated with the use of an extracardiac conduit to achieve total caval pulmonary connection.

The pre-Fontan hemodynamic variables are summarized in Table 2. The transpulmonary gradient and the single ventricular function, as judged by ventricular end-diastolic pressure and ejection fraction, as well as the McGoon index of pulmonary artery size, were not significantly different between cohorts. The degree of AV valve regurgitation was similar, and all patients were in normal sinus rhythm at the time of the Fontan operation.


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Table 2. Pre-Fontan Hemodynamics
 
The pre-Fontan staging procedure was designed according to the type of total caval pulmonary connection to be used (Table 3). Patients in the EC cohort underwent a superior caval–pulmonary artery connection (Glenn), whereas those patients in the LT group had the hemi-Fontan procedure. A significant number of patients having the modified Norwood operation for HLHS later underwent a lateral tunnel Fontan. The frequency of systemic-to-pulmonary artery shunt and the use of pulmonary artery banding trended higher in the EC cohort, but the difference was not significant.


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Table 3. Previous Operative Procedures
 
Operative Variables
The operative data are summarized in Table 4. The time on the extracorporeal circuit was similar in both groups, but the frequency of aortic cross-clamping and baffle fenestration was significantly higher in LT patients. Although one third of the patients who underwent the extracardiac conduit procedure were fenestrated, the size of the fenestration was significantly larger in this cohort.


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Table 4. Operative Data
 
The use of resources and postoperative hemodynamics are summarized in Table 5. The transpulmonary gradient assessed at the time of patient arrival in the intensive care unit and 12 hours later was not significantly different in either cohort. Patients receiving the lateral tunnel operation were fenestrated more frequently, and this was associated with a significantly shorter hospital stay and a trend towards earlier extubation.


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Table 5. Postoperative Data
 
The estimate of the ventricular ejection fraction (EF) was 0.62 in EC patients versus 0.60 in LT patients (p = NS) and the change in ejection fraction ({Delta}EF) relative to the preoperative ventricular function was not significantly different in either cohort: 0.03 ± 0.012 in EC patients versus 0.031 ± 0.011 in LT patients (p = NS).

The readmission for the treatment of recurrent chylous effusions occurred with similar frequency in both cohorts. The mean interval from postoperative discharge to readmission was 8 days in the EC group and 15 days in the LT cohort.

Early Mortality
There were three early deaths, 1 (2%) of 49 in the EC group and 2 (2%) of 113 in the LT group (p = NS). One patient with dextrocardia, atrial situs solitus, double outlet right ventricle, and pulmonary stenosis underwent a bidirectional Glenn anastomosis with banding of the main pulmonary artery at 4 months, followed by an extracardiac conduit Fontan at 3 years of age. Twenty-four hours postoperatively, low cardiac output developed which required takedown of the conduit. He was extubated on postoperative day 2 but experienced a sudden cardiac arrest while being treated for generalized seizures 4 days postoperatively. A second patient, with Down syndrome and an unbalanced AV canal, underwent a pulmonary artery banding at birth. At 6 months, a 3.5-mm modified Blalock shunt was performed, followed by a hemi-Fontan at 1.5 years of age and a lateral tunnel Fontan at age 4. Two weeks postoperatively, he died after takedown of the lateral tunnel secondary to low cardiac output and multisystem organ failure. The remaining death in the LT group occurred in a Down syndrome patient with a double outlet right ventricle, complete AV canal, and pulmonary atresia. She underwent a modified Blalock shunt at birth, a hemi-Fontan at 6 months, and lateral tunnel Fontan at age 2. She died 6 days postoperatively from low cardiac output resulting in multisystem organ failure.

Late Mortality
There were seven late deaths (EC, 4/48, 8%; LT, 3/111, 3%; p = NS). Two cardiac-related deaths in the EC group occurred. One was at 17 months secondary to sepsis after transplantation for protein-loosing enteropathy, and one was at 6 years postoperatively from chronic cyanosis with congestive heart failure in the setting of heterotaxy syndrome. A third patient with heterotaxy syndrome experienced a sudden unexplained death at home 4 years after an EC Fontan. One noncardiac related late death in the EC cohort occurred at 4 months post-Fontan secondary to an accident.

Three late deaths occurred in the LT group. One patient with HLHS experienced a cardiac arrest home 3.5 years postoperatively, and a second patient, with tricuspid atresia, a history of atrial flutter, and a middle cerebral artery stroke, died during physical therapy 9 years postoperatively. The remaining late death in the LT cohort occurred 6 years post-Fontan secondary to a malignancy. The late mortality, while slightly higher in the EC cohort, did not achieve significance (Table 6).


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Table 6. Outcomes
 
The actuarial survival curve comparing the lateral tunnel with the extracardiac conduit, including hospital deaths, is shown in Figure 1. There were four cardiac and one noncardiac death in each group. The two curves parallel one another out to 3 years, with no significant difference in actuarial survival (EC, 90% at 5 years; LT, 95% at 5 years).


Figure 1
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Fig 1. Kaplan-Meier survival in 162 patients undergoing an extracardiac conduit (EC) (n = 49) or lateral tunnel (LT) (n = 113) Fontan operations. The hatch marks along each curve represent the number of patients at risk. The cause of death for each group is shown. Survival is expressed as percentage. There is no significant difference in survival between patients in the EC and LT cohorts.

 
Morbidity
Fontan takedown was necessary in 3 additional patients, all in the EC cohort. The first patient had HLHS with a McGoon ratio of 1.4, a transpulmonary gradient of 4 mm Hg, and a right ventricular end-diastolic pressure of 9 mm Hg. He underwent an uncomplicated fenestrated Fontan, but bilateral pleural effusions and ascites with hepatomegaly developed. At 2 weeks postoperatively, the 6 mm fenestration was enlarged to 10 mm without clinical improvement. At 2 months, the Fontan was taken down uneventfully. A second patient with small pulmonary arteries (McGoon ratio, 1.3) and elevated pulmonary artery pressure required takedown of a fenestrated Fontan because of low cardiac output on postoperative day 30. Recovery was uneventful. The remaining takedown occurred secondary to low cardiac output 2 days postoperatively in a patient with depressed right ventricular function preoperatively, AV septal defect, and mild tricuspid regurgitation. He recovered uneventfully.

Protein-losing enteropathy was observed in 3 patients (1 EC; 2 LT). One patient with protein-losing enteropathy and an extracardiac conduit Fontan is described under Mortality. A second patient with protein-losing enteropathy, dextrocardia, L-transposition, and a double-outlet right ventricle with pulmonary atresia underwent a successful transplant 11 years after lateral tunnel Fontan. The third patient, with hypoplastic right heart syndrome and complete AV septal defect, developed protein-losing enteropathy 4 years after lateral tunnel Fontan and is awaiting transplantation.

Other complications included early reoperation to revise fenestration in 1 LT patient and reexploration for bleeding in 1 EC patient. No patient had recognized thrombosis in the Fontan pathway; however, neurologic events with a thromboembolic origin developed in 8 patients.

Fontan failure from any cause, takedown, or early or late death was analyzed with respect to the presence or absence of a fenestration and the type of caval pulmonary connection. Singularly or together, these three variables for Fontan failure were not significantly different in fenestrated versus nonfenestrated patients. Grouped together, however, Fontan failure was significantly higher in the EC cohort at 16.3% compared with the LT group at 4.4% (p < 0.05).

Neurologic Events
The occurrence of any neurologic event at discharge and at latest follow-up was observed with similar frequency in patients in the EC and LT cohorts. Six neurologic events occurred in the EC cohort. In 3 patients (2 fenestrated), transient ischemic attack developed that resolved spontaneously with full recovery, and 1 patient with heterotaxy syndrome, low cardiac output, and multiorgan system failure died while being treated for generalized seizures. One fenestrated patient had significant recovery after an embolic right middle cerebral artery infarction, and a final patient (nonfenestrated) with HLHS experienced a large temporal and frontal hemorrhage that occurred in the setting of low cardiac output.

The neurologic events experienced by the 2 patients in the LT cohort were embolic in origin and resulted in a cerebral infarction. One event occurred immediately postoperatively, with full recovery, whereas the remaining neurologic event was observed in a fenestrated patient not on Coumadin therapy 2.5 years after a lateral tunnel Fontan.

Arrhythmias During Follow-Up
Normal sinus rhythm was observed in 85% of the EC and 82% of the LT patients (p = NS). The frequency of nonsinus rhythm (EC, 7/47, 15% versus LT, 20/111, 18%; p = NS) and the need for permanent pacemaker insertion (EC, 4/47, 9% versus LT, 11/111, 10%; p = NS) was similar in both cohorts. In the EC group, a permanent pacemaker was implanted for sinus node dysfunction in 3 patients and for junctional bradycardia in 1 patient. Among seven pacemaker implantations in the LT group, three were for complete heart block, one was for ventricular tachycardia, and seven were for sinus node dysfunction.

Bradycardia was observed with slightly greater frequency in the LT group, but the differences were not significant (EC, 10/42, 24% versus LT, 33/102, 32%). The need for antiarrhythmic medication was not significantly different in either cohort (EC, 3/44, 7% versus LT, 4/108, 4%).

Atrioventricular Valve Regurgitation During Follow-Up
The competency of the AV valve was nearly equally distributed between trace and moderate among the EC patients. The degree of AV valve regurgitation trended higher among patients in the LT group, but the difference was not significant.


    Comment
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
The achievement of total caval pulmonary connection to palliate patients with a univentricular heart has improved significantly during the last 20 years. This improvement can be attributed to proper patient selection and the elimination of known anatomic risk factors to achieve successful extracardiac total caval pulmonary connection at the time the superior caval pulmonary connection is made. The design of the inferior caval–pulmonary artery connection to achieve an anastomosis with minimal energy loss has been an important modification. The two most common techniques used are the lateral tunnel and the extracardiac conduit connection. The relative merits of both techniques have been described previously, but comparisons of the two procedures in the literature have been relatively limited. This report focuses on a retrospective analysis of the extracardiac and lateral tunnel operation with respect to early-term and mid-term mortality, morbidity, and postoperative outcomes.

Mortality and Morbidity
The overall early mortality was 1.8% (3/162), which compares favorably with reported series recently published [8]. Two hospital deaths occurred in Down syndrome patients with a lateral tunnel Fontan with complete AV canal. Both patients required a modified Blalock shunt at the time of hemi-Fontan to enhance oxygenation, suggesting an elevation in pulmonary vascular resistance. The etiology of higher pulmonary vascular resistance in patients with Down syndrome may be secondary to chronically elevated levels of carbon dioxide, which can cause pulmonary artery vasoconstriction.

The presence of an accessory source of antegrade blood flow may also be an associated risk factor for failure of the Fontan pathway [9]. Based on our limited experience, Down syndrome alone or in conjunction with an accessory source of pulmonary blood flow is considered a relative contraindication to the Fontan operation. The remaining hospital death occurred in a patient with heterotaxy syndrome, also a known risk factor for completion of the Fontan operation.

Perioperative risk factors are highly associated with mortality and failure of the Fontan operation. These include the absence of a fenestration, lower McGoon ratio, elevated pulmonary artery pressure, ventricular dysfunction, morphologic right ventricle, heterotaxy syndrome, and prolonged time on the extracorporeal circuit [10]. In this series, the pulmonary artery pressure, ventricular performance, and dominance were not significantly different in either group. CPB times, although not significantly different between cohorts, trended higher in the EC group. The patients in the EC group had a higher incidence of heterotaxy syndrome and a trend towards more frequent use of pulmonary artery banding and systemic–pulmonary artery shunting before their pre-Fontan staging procedure (Table 3).

In this report, patients in the LT group were preferentially at higher risk because they had a greater frequency of HLHS, were younger, and required aortic cross-clamping to complete the Fontan pathway. Most were fenestrated and yet were extubated earlier and experienced significantly shorter hospital stay than their counterparts in the EC group, where only one third of the patients were fenestrated.

Overall, the actuarial survival of 5 years was not significantly different between the groups (EC, 90%; LT, 95%; p = 0.08). The LT patients appeared to fair slightly better, but this could reflect the loss to follow-up rate as all of the 8 patients who were not monitored long-term were from the LT cohort.

The extended time in the hospital in the EC group is a marker for prolonged pleural fluid drainage. If more patients in the EC group had been fenestrated, resource utilization might have been significantly reduced. In addition, the use of epidural anesthesia to decrease postoperative pain and encourage early extubation in conjunction with Blake drains for outpatient management of pleural effusions occurred more often in the LT group, where resource utilization was significantly less.

The absence of a fenestration, higher postoperative transpulmonary gradients, and a longer duration of CPB have been associated with prolonged pleural fluid drainage [11]. These latter two variables were not significantly increased in the EC cohort. At the present time, we perform the cavoatrial and pulmonary artery dissection before instituting CPB to limit patient exposure to the extracorporeal circuit. The performance of the extracardiac conduit Fontan without CPB may offer additional advantages with respect to preservation of cardiac and pulmonary function and decrease resource utilization.

Gaynor and associates [12] reported their outcomes for the Fontan operation in 332 patients. They demonstrated that HLHS was no longer a risk factor for early Fontan failure and that single-punch fenestration in the lateral tunnel operation is associated with a decrease in mortality, pleural drainage, and hospital stay. This strategy has been confirmed by other investigators [13, 14]. Currently, we have increased the use of fenestration in the extracardiac conduit, especially in patients with HLHS alone or in combination with coexisting preoperative risk factors.

Fontan patients have abnormal venous hemodynamics with chronically elevated venous pressures and limited ability to mobilize venous blood to increase cardiac output. Hsia and colleagues [15] demonstrated in an elegant study that after the Fontan operation, there is loss of the normal augmentation of hepatic venous and portal venous flow associated with inspiration and expiration. They further demonstrated that fenestration lowers venous pressures and restores respiratory-mediated hepatic venous flow [16]. This augmentation of forward venous flow from the liver and splanchnic circulation in fenestrated patients may decrease the late development of protein-loosing enteropathy.

The use of a fenestration, although advantageous, can be the nidus for paradoxic embolization that results in a potential neurologic deficit. Thrombotic complications after the Fontan operation have ranged from 10% to 30% [17]. The causes of clot formation include a low flow rate through the caval pulmonary circuit, atrial arrhythmias, and intraatrial suture lines. Patients with univentricular heart are also known to be in a hypercoagulable state, with deficiencies of protein C, protein S, anti-thrombin III, and elevated levels of factor VIII [18].

In our series, thrombosis of the pathway was not demonstrated in either cohort, but embolic events occurred in 5 fenestrated patients (3 EC; 2 LT). Each was managed with heparin perioperatively and Coumadin postoperatively. It is not clear whether thromboembolism can be prevented with inhibition of the coagulation cascade using Coumadin or platelet function with aspirin. Our own practice is to maintain patients on Coumadin therapy as long as the fenestration is patent and add aspirin if the thromboembolic event occurs. Coumadin alone or with aspirin can be associated with increased bleeding complications. One patient in the EC group did experience an intracranial hemorrhage while on Coumadin therapy. Follow-up catheterizations have demonstrated spontaneous fenestration closure in 35% to 50% of patients at a median of 12 months [19].

Atrial Arrhythmias
Atrial arrhythmias occur in 10% to 40% of patients after the Fontan operation [20]. These may include sinus node dysfunction and supraventricular tachycardia. The extracardiac Fontan, which leaves the common atrium in a low-pressure environment and without any atrial suture lines (if nonfenestrated), may have a lower incidence of atrial arrhythmias.

The lateral tunnel procedure contains extensive intraatrial suture lines and has the potential for atrial distension, two variables identified as risk factors for the development of sinus node dysfunction. The frequency of sinus node dysfunction at follow-up after the extracardiac conduit or lateral operations is variable. Kumar and associates [21] identified the extracardiac conduit as a risk factor for sinus node dysfunction in a series of 33 patients. Ovroutski and colleagues [22] documented a greater degree of sinus rhythm in extracardiac conduit patients and a greater incidence of pacemaker implantation for sinus node dysfunction in lateral tunnel patients.

In our own series, the need for pacemaker implantation and the preservation of sinus rhythm was equally distributed between the EC and LT patients. This agrees with the findings of Cohen and associates [23]. In our report, however, the frequency of sinus node dysfunction may be underestimated because Holter monitoring and exercise testing to assess chronotropic competence of the sinus node was not used.

Study Limitations
A number of important limitations are present in this analysis. Data were acquired retrospectively from two institutions in a nonrandomized manner. The operations were performed by four surgeons. The extracardiac conduit operation was introduced into this series at a later period in time, and the decision to perform either an extracardiac conduit or lateral tunnel Fontan was determined by the patient’s anatomy and surgeon’s preference.

Echocardiographic and ECG analysis were performed by multiple nonblinded examiners, and Holter monitoring to assess continuous cardiac rhythm was not used. The incidence of thromboembolic complications may be underestimated because only clinically relevant events received attention. A significant limitation relates to inter institutional differences in methodology and management strategy, which would influence patient management and outcome.

Conclusion
This report retrospectively compares the extracardiac conduit and lateral tunnel operations to achieve total caval pulmonary connection at two institutions. The hospital morality and late survival did not differ significantly between the groups. The need for readmission to manage chylous effusions and the preservation of sinus rhythm at the time of follow-up were similar in both cohorts. The frequency of all neurologic events was also similar, but a trend to greater embolic events was observed in the extracardiac conduit patients. The absence of a fenestration in most of the patients receiving the extracardiac conduit Fontan was associated with increased resource usage. The lateral tunnel operation does incorporate growth potential and could be used in a younger patient population. A larger prospective randomized series would more likely address differences between the two surgical techniques more adequately.


    Discussion
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
DR CHARLES D. FRASER, JR (Houston, TX): Andy, that was really elegantly presented and I think an important paper which certainly confirms a lot of what I believe, and that is, that the Fontan connection should be geared to the patient and not trying to gear the patient to the operation. And so I would certainly agree that the selective use of both methods can achieve really excellent outcomes like you have proven.

But one finding was puzzling to me, and that is, that the extracardiac group actually had a longer length of time on the ventilator than the lateral tunnel. Our own experience has been built on that of others, particularly the group at Stanford, that showed us that early extubation in these patients is predictable. And working with our anesthesiologists now, the majority of our extracardiac Fontan patients are now extubated in the operating room and that’s certainly shortened our ICU length of stay. I was just wondering about your thoughts on that.

DR FIORE: I fully agree that early extubation is most optimal. As we all know, negative pressure ventilation is better than positive pressure ventilation for these patients. Early in the series, we did not use epidural blocks to aid in early extubation. At the present time, we try and extubate patients as you do in the operating room.

DR MARSHALL L. JACOBS (Philadelphia, PA): Andy, my compliments on a beautiful presentation and a very nice series.

Noting the somewhat increased length of stay, presumably related to effusions in the extracardiac conduit group, I share that impression, while many series advocating the extracardiac conduit have shown the opposite data and that there was no greater frequency or duration of effusions. That having been said, I think that when it does occur, it is at least in part related to the predictability or reliability of achieving a right-to-left shunt through fenestration, which I think is more challenging with the extracardiac conduit, and your method of interposing a small graft is one of several that’s been proposed.

I wonder if you feel, based on human dynamic observations and arterial oxygen saturations postoperatively, number one, whether you really achieved a successful and reliable fenestration with that method in the extracardiac conduit group.

And second, related to the same problem in our series of comparable size, the only patient who post Fontan required a catheter ablation for recurrent reentry tachycardia had this easily accomplished by means of catheter access through a fenestration in the lateral tunnel. And I am concerned in the heterotaxy patients and others that those who come back later with supraventricular arrhythmias may pose a real access challenge to our EP colleagues who want to get in there for radiofrequency ablation. I wonder about your comment on that.

DR FIORE: With respect to your first question. The technique that we demonstrated to construct the fenestration was performed with a non-ringed Gore-Tex tube. I think at times we did not achieve the fenestration size that we wanted. In the majority of patients, however, we switched over to using a ringed Gore-Tex tube. We cut the tube flush with a ring and sew over the ring so that the ring acts like a stent and will keep the anastomosis open at the desired diameter. This technique is used for both anastomoses.

Marshall, could you ask me the second question one more time please?

DR JACOBS: In patients who have an extracardiac conduit and don’t have a patent fenestration, I suppose if one has to go back and intervene by way of ablation for arrhythmias, one would have to take a surgical approach rather than a transvenous approach.

DR FIORE: This is correct. The cardiologist cannot access the heart if there was no patent fenestration. I suppose this is another important reason to fenestrate the Fontan connection.

DR KIRK R. KANTER (Atlanta, GA): You mentioned one patient who died a year after transplantation. Were there other patients who required transplantation in this series and, if so, were they equally distributed between the two groups?

DR FIORE: There were two patients, one in the lateral tunnel and one in the extracardiac group, that required transplantation, both for PLE.

DR KANTER: You didn’t show a slide depicting the distribution of the two different techniques between institutions. Since you said it was surgeon preference, and if all the extracardiac Fontans were done at one institution, could institutional differences of patient care account for some of the differences you saw?

DR FIORE: This is a very good question. I don’t have the exact numbers, but the majority of the extracardiac Fontans were done in St. Louis and the majority of the lateral tunnels were done in Indianapolis. I agree with you that this does introduce institutional bias which could impact on our results. This bias is summarized in the manuscript.


    Acknowledgments
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 
We gratefully acknowledge the expert technical assistance of Terri Wriley in manuscript preparation and the assistance of Barbara Kountzman, RN, Mary Hohenberg, PA and A. Thomas Pezzella, MD, for their assistance with data acquisition and patient follow-up.


    References
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 Acknowledgments
 References
 

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