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Ann Thorac Surg 2002;74:S1416-S1421
© 2002 The Society of Thoracic Surgeons

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Supplement: Cardiothoracic Techniques and Technologies

The extracardiac Fontan procedure without cardiopulmonary bypass: technique and intermediate-term results

^a Department of Cardiovascular Surgery, The University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, Arkansas, USA
^b Department of Pediatric Cardiovascular Anesthesiology, The University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, Arkansas, USA
^c Department of Pediatric Cardiology, The University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, Arkansas, USA

* Address reprint requests to Dr Drummond-Webb, Chief, Department of Pediatric Cardiovascular Surgery, Arkansas Children’s Hospital, 800 Marshall Street, Slot 677, Little Rock, AR 72202-3591 USA
e-mail: drummond-webbjonathan{at}uams.edu

Presented at the Eighth Annual Cardiothoracic Techniques and Technologies Meeting 2002, Miami Beach, FL, Jan 23–26, 2002.

	Abstract

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
BACKGROUND: The extracardiac Fontan procedure (ECF) usually requires cardiopulmonary bypass (CPB). In this report, the results and techniques of this procedure without CPB at a single institution are presented.

METHODS: Between August 1992 and December 2001, ECF without CPB was achieved in 24 of 44 patients undergoing an ECF. Mean age at surgery was 5.9 ± 2.9 years, and mean weight was 20.7 ± 12.6 kg. Diagnoses were tricuspid atresia in 9 patients, single-ventricle with pulmonary outflow tract obstruction in 7, pulmonary atresia/intact septum in 5, and other complex single-ventricle physiology in 3. Initial palliation was by arterial to pulmonary artery shunt in 21 and pulmonary artery banding in 1. A bidirectional cavopulmonary connection was created in 23 patients. A temporary inferior vena caval–to–atrial shunt was used to complete the procedure without CPB. Median graft size was 16 mm (range 14 to 20 mm).

RESULTS: There was no early mortality, and 68% of patients were discharged without complications. Complications included persistent cyanosis in 4 patients, persistent pleural effusions in 2 (one chylous), and phrenic nerve injury in 1. Median postoperative hospital stay was 16 days (range 10 to 50) days. At a mean follow-up of 44 ± 28 months, there was no conduit obstruction. One patient died 11 months postoperatively, and 1 patient received a heart transplant 26 months post-ECF.

CONCLUSIONS: At intermediate term follow-up, the ECF without CPB appears to be safe and technically reproducible in selected cases. Ongoing follow-up of these patients is necessary to document the theoretical advantages of avoiding CPB.

	Introduction

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Since the introduction of the classic Fontan procedure in 1971 [1], a variety of surgical and technical modifications have been introduced [2–4], with associated improvements in morbidity and mortality. Modifications have included a staged approach to completion of the Fontan by means of a bidirectional cavopulmonary connection (BCPC) [2], the lateral tunnel modification, and the concept of a total cavopulmonary connection [1, 3]. Most recently, use of an extracardiac conduit to achieve a total cavopulmonary connection (ie, the extracardiac Fontan [ECF]) has become the strategy of many surgeons [4]. The theoretical benefits include the following: avoidance of aortic crossclamping and myocardial ischemia [5], shortened duration of cardiopulmonary bypass (CPB) [5], improved long-term hemodynamics [6], better hydrodynamics and flow characteristics [6], and a lower incidence of atrial arrhythmias [7].

Although outcomes have improved with this procedure [8], perioperative complications are not insignificant; some are related to the need for CPB, with the attendant risks of activation of vasoactive cytokines secondary to exposure to a foreign surface. The technique of performing the ECF without the use of CPB has been reported in small series [9, 10]. Tam and colleagues [9] reported on 21 patients undergoing ECF off CPB, but a variety of conduit types and sizes were used. Immediate postoperative hemodynamics appeared superior when compared to those patients undergoing ECF with CPB [9]. Long-term benefits and potential problems, however, are still unknown. We report our technique of performing the ECF procedure using a polytetrafluoroethylene conduit for the ECF without the use of cardiopulmonary bypass. Intermediate term results of this strategy are presented.

	Material and methods

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Since 1982, a total of 86 Fontan procedures were performed at our institution. The first ECF without the use of CPB was performed in August 1992. Between August 1992 and December 2001, we performed 44 ECF procedures, of which 24 were performed without CPB. We perform the ECF procedure electively at a weight of 15 kg. The ECF technique without CPB is our preferred procedure, provided that no intracardiac repair or extensive pulmonary arterial reconstruction is needed. Indications for use of CPB to achieve an ECF are shown in Table 1. In 4 patients, the off-bypass procedure was aborted. In 1 patient, a faulty vascular clamp slipped off the atrium, requiring urgent conversion to CPB. In 2 patients with a diagnosis of left atrial isomerism with azygos continuation of an interrupted inferior vena cava (IVC), exposure of the hepatic venous-right atrial junction resulted in significant hemodynamic compromise, requiring CPB. One patient experienced desaturation with placement of the pulmonary artery clamp, and CPB was used for a 10-minute period to complete the anastomosis.

Surgical technique
Our surgical technique is as follows. A repeat median sternotomy is performed in standard fashion. A CPB circuit is primed, with perfusion staff in attendance for all cases. Steroids (10 to 20 mg/kg of hydrocortisone) are administered preoperatively to all patients. All patients are partially heparinized, with the aim of an activated clotting time (ACT) in excess of 300 seconds. Heparin dosage is titrated to the preintervention ACT, with intraoperative monitoring of the ACT. Heparinization is not reversed at the conclusion of the procedure.

Key points of dissection include complete liberation of the IVC junction (dissecting below the diaphragm if necessary) and complete freeing up of the BCPC, main, and branch pulmonary arteries. We consider it particularly important to free up the lateral pericardial recess, as this allows the conduit to lie in place without compression of the underlying pulmonary veins and atrial mass, and also allows a longer graft to be placed with a gentle anterior curve providing some length for somatic growth.

The polytetrafluoroethylene (PTFE) tube size is restricted by the length of the pulmonary artery segment available for construction of the superior anastomosis. This is measured (Fig 1, distance X to Y) and the largest size conduit chosen. In the first 7 patients, the PTFE conduit was anastomosed end-to-side to the IVC using a side-biting clamp. Currently, the distal IVC to conduit anastomosis is performed first. After heparinization, an IVC to atrial shunt is constructed using two right-angle venous cannulas, which are connected with the shortest length of tubing so as to minimize resistance and to keep it out of the field. The shunt is placed as low as possible on the IVC (Fig 2). The shunt is very carefully deaired. A vascular clamp is placed across the inferior portion of the right atrium, with care taken to identify and to avoid the coronary sinus. A running suture is placed below the clamp before dividing the atrial–IVC junction (earlier in our experience, a faulty clamp dislodged). The junction is divided and the atrium is doubly oversewn. The IVC-to-extracardiac anastomosis is completed end-to-end (Fig 3).

View larger version (28K): [in this window] [in a new window]   Fig 1. Distance XtoYdetermines diameter of polytetrafluoroethylene conduit that can be placed. Inferior vena caval right atrial junction is divided and oversewn.

View larger version (27K): [in this window] [in a new window]   Fig 2. Temporary atrial-to–inferior vena cava shunt (arrow). Bidirectional cavopulmonary connection is already established. "Low" placement of inferior vena cava cannula is shown.

View larger version (35K): [in this window] [in a new window]   Fig 3. Diagram showing completion of distal anastomosis. Polytetrafluoroethylene conduit (*) is trimmed obliquely, and a partial occlusion clamp allows the bidirectional cavopulmonary connection to perfuse the lungs. Note offset of polytetrafluoroethylene conduit anastomotic site relative to bidirectional cavopulmonary connection.

In 1 patient, placement of the clamp across the main pulmonary artery resulted in unacceptable hypoxia. A temporary aortic-to–pulmonary artery shunt was created by placing a 14-mm Medtronic DLP arterial cannula (Medtronic Inc, Minneapolis, MN) into both the ascending aorta and left pulmonary artery (Fig 4), thus allowing completion of the ECF without CPB.

View larger version (26K): [in this window] [in a new window]   Fig 4. Temporary systemic arterial to pulmonary artery shunt in place (arrow). This allows improved pulmonary flow while the proximal polytetrafluoroethylene conduit (*) to pulmonary artery anastomosis is completed.

	Results

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Preoperative data
There were 8 female and 16 male patients. Anatomical diagnoses included tricuspid atresia in 9 and single ventricle with obstructed pulmonary blood flow in 7; of these, 3 patients had dextrocardia, single ventricle with left atrial isomerism, unobstructed pulmonary blood flow, and azygos continuation of an interrupted IVC in 2, pulmonary atresia/intact ventricular septum in 5, and hypoplastic left heart in 1. Prior surgical procedures on the pulmonary arteries included pulmonary artery banding in 2 patients and creation of a systemic arterial to pulmonary artery shunt in 22.

All patients except 1 had a BCPC before completion of the Fontan procedure at a median age of 13 months (range 6 to 60 months). The mean interval from BCPC to Fontan was 43.2 ± 12.6 months. The ECF off CPB was performed at a mean age of 5.7 ± 2.9 years and a mean weight of 20.3 ± 10.3 kg.

Perioperative data
The PTFE conduit sizes ranged from 14 to 20 mm (median 16 mm). The relationship of conduit size to body weight is shown in Figure 5. The average hospital stay was 16 days (range 10 to 50 days), which was reduced to 6.5 days for the year 2001. The median time of mechanical ventilation time was 20 hours (range 2 to 165 hours), which was also reduced to 9.3 hours for the year 2001. A single transfusion of packed red blood cells (10 mL/kg) was required postoperatively in 10 of the 24 patients to maintain a hematocrit of 33 or greater. One additional patient required more than one transfusion. It is our standard protocol to provide inotropic support with dopamine 5 µgkg^-1min^-1 and afterload reduction with nitroglycerin 2.5 µgkg^-1min^-1 for 24 hours postoperatively after which time inotropic support is stopped and a nitropatch placed. No patient required additional inotropic support.

View larger version (16K): [in this window] [in a new window]   Fig 5. Relationship between body weight and polytetrafluoroethylene conduit size.

One patient was given a bolus dosage of digoxin for an episode of supraventricular tachycardia while the chest was being closed after an uneventful off-pump ECF. Severe hemodynamic compromise with ventricular fibrillation ensued. The etiology was immediately recognized and the patient was placed onto CPB and an extracorporeal membrane oxygenation circuit prepared. The patient was converted to extracorporeal membrane oxygenation and supported for 48 hours. He was subsequently weaned, separated, and suffered no apparent immediate ill effect, but died 11 months postoperatively of plastic bronchitis.

Follow-up
Patients were followed for a period of up to 97.2 months with a mean follow-up duration of 44.4 ± 28.8 months. One patient was lost to follow-up after a period of 2 years. There was no early death, but one late death. Three patients received a pacemaker for symptomatic sinus bradycardia with junctional escape. Routine electrocardiography and 24-hour ambulatory Holter monitoring were performed in all patients. All patients were noted to be in sinus rhythm on most recent evaluation, with the exception of 2 of the 3 patients who had pacemaker implantation after ECF who received atrial pacing. One patient who had complete atrioventricular block before ECF remained DDD (dual chamber sensing, pacing, and inhibited and triggered) paced. Intermittent episodes of asymptomatic sinus bradycardia with junctional escape were noted in 2 patients. No patient had supraventricular or ventricular tachycardia. All patients were followed-up routinely with annual echocardiography for assessment of ventricular function, atrioventricular valve regurgitation, and assessment of obstruction of the Fontan circuit. One patient who was chronically DDD paced developed progressive systemic ventricular dysfunction and severe atrioventricular valve regurgitation; he underwent cardiac transplantation 26 months after ECF. The only late mortality in the series occurred 11 months post-ECF in a patient dying of septic complications in the setting of spina bifida and plastic bronchitis. Two patients were noted to have mild ventricular dysfunction and 8 patients mild atrioventricular valve regurgitation. Echocardiography revealed no evidence of pulmonary artery distortion or obstruction of the Fontan circuit on late follow-up. All surviving patients were in New York Heart Association class I or II.

Long-term complications were rare. Patients were not routinely maintained on Coumadin (Du Pont Pharmaceuticals, Wilmington, DE) until the last year of the study because of a general change in management strategy. Currently, all patients undergoing the Fontan procedure receive anticoagulation with Coumadin. Despite lack of anticoagulation in most patients, there were no incidents of graft thrombosis or obstruction. No patient has required conduit replacement; no patient has developed bacterial endocarditis; and no patient undergoing ECF without CPB has developed protein-losing enteropathy.

	Comment

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Surgical management of patients with single ventricle physiology has evolved considerably over the last few decades. Improved patient selection and understanding of risk factors, staged preparation toward a Fontan procedure, as well as improvements in perioperative management have resulted in substantial improvements in morbidity and mortality. The ECF procedure represents just one such technical evolution. Avoidance of intraatrial suture lines, intracardiac prosthetic material, and exposure of the atria to increased venous pressure are just a few of the reasons why this procedure has become the preferred technique [8]. Azakie and colleagues [3] demonstrated prolonged CPB time to be the only independent predictor of mortality and risk of complications in patients undergoing Fontan procedures. We have demonstrated that further optimization of the ECF procedure is possible and that completion of the procedure off CPB can be achieved in selected patients. Importantly, we do not compromise the Fontan principles or risk residual lesions to achieve off-CPB status. Despite initial concerns of an increased risk of thromboembolic complications with this procedure, we and others [11] have documented no thrombosis in these patients. We currently recommend life-long anticoagulation, as it has been suggested that there are abnormal coagulation factors in patients who have had a Fontan procedure [12]. In addition, Jahangiri and colleagues [13] have documented a 16% incidence of thromboembolism after Fontan procedure.

Concerns over the optimal size of the conduit and the lack of growth thereof have been raised. These issues are valid, as conduit replacement in the setting of a Fontan patient is a significant undertaking. It has previously been shown that at 2 to 4 years of age, and 12 to 15 kg of body weight, the IVC-to–pulmonary artery distance is up to 80% that of adult size [14]. We electively perform ECF when the patient has reached a weight of 15 kg. During a follow-up period in excess of 8 years, we have not had a patient require conduit replacement, nor have we documented any obstruction of the conduit within this time.

In conclusion, the ECF, with many of its inherent benefits, can be achieved without the use of CPB in selected patients. The surgical procedure and protocols are simple and easily reproducible. At intermediate term follow-up, this strategy has resulted in acceptable outcomes. Ongoing evaluation of this cohort is required to validate whether this strategy offers any objective advantage over those protocols that routinely use CPB.

	Acknowledgments

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This work was supported by funding from the Arkansas Log-A-Load Endowed Chair of Pediatric Cardiovascular Surgery at Arkansas Children’s Hospital. The surgical input of James E. Harrell, MD, Jean Anne Phillips, RN, Carole J. Wilson, RN, Kalen Rogers, RN, Patrick Young, RN, James Traylor, RN, Shawn Hill, RN, Michael Tucker, RN, and the anesthesia contribution of Gerald A. Bushman, MD, are recognized in this series. All data used in this report were obtained from an institutional review board–approved database.

	References

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 

1. Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26:240-248.[Abstract/Free Full Text]
2. DeLeval M., Kilner P., Gewellig M., Bull C. Total cavopulmonary connection: a logical alternative to atrial pulmonary connection for complex Fontan operations. J Thorac Cardiovasc Surg 1988;96:682-695.[Abstract]
3. Azakie A., McCrindle B.W., Van Arsdell G.S., et al. Extracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes. J Thorac Cardiovasc Surg 2001;122:1219-1228.[Abstract/Free Full Text]
4. Marcelletti C.F., Hanley F.L., Mavroudis C. Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: a multicenter experience. J Thorac Cardiovasc Surg 2000;199:340-346.
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7. Amodeo A., Galletti L., Marianeschi S., et al. Extracardiac Fontan operation for complex cardiac anomalies: seven years experience. J Thorac Cardiovasc Surg 1997;114:1020-1031.[Abstract/Free Full Text]
8. Van Arsdell G.S., McCrindle B.W., Einarson K.D., et al. Interventions associated with minimal Fontan mortality. Ann Thorac Surg 2000;70:568-574.[Abstract/Free Full Text]
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11. Marcelletti C.F., Iorio F.S., Abella R.F. Late results of extracardiac Fontan repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg 1999;2:131-142.
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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Jonathan Drummond-Webb William P. Fiser Michiaki Imamura Sana Ullah Charles E. Johnson Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Yetman, A. T. Articles by Van Devanter, S. H. Search for Related Content PubMed PubMed Citation Articles by Yetman, A. T. Articles by Van Devanter, S. H. Related Collections Congenital - cyanotic