Ann Thorac Surg 1999;68:1698-1703
© 1999 The Society of Thoracic Surgeons
Original Articles
Modified Fontan without use of cardiopulmonary bypass
Vincent K.H. Tam, MDa,
Bruce E. Miller, MDb,
Kathy Murphy, MSNc
a Section of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, Georgia, USA
b Department of Anesthesiology, Emory University School of Medicine, Atlanta, Georgia, USA
c Sibley Heart Center, Egleston Children’s Hospital, Atlanta, Georgia, USA
Address reprint requests to Dr Tam, Emory Clinic, 1365 Clifton Rd, Suite A2236, Atlanta, GA 30322
e-mail: vtam01{at}emory.edu
Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 26–28, 1998.
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Abstract
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Background. Direct cavopulmonary connection using an extracardiac conduit has a number of theoretical advantages in the staged management of children with single ventricular congenital heart defects. With appropriate planning, completion Fontan using an extracardiac connection may be accomplished without the use of cardiopulmonary bypass.
Methods. From January 1995 to October 1997, 32 consecutive patients underwent completion Fontan using an extracardiac cavopulmonary connection. Twenty-one of these patients had completion Fontan without the use of cardiopulmonary bypass (No CPB group). Their postoperative outcome was retrospectively compared with a second group of 11 patients who underwent completion Fontan with an extracardiac conduit with the use of cardiopulmonary bypass.
Results. There was no operative or hospital mortality in either group. Early postoperative hemodynamics appear to be significantly improved in the No CPB group. Transfusion of cryoprecipitate and platelets was significantly less in the group without the use of cardiopulmonary bypass (p = 0.026, p < 0.001, respectively). Review of the most recent 12 patients also demonstrated a substantially shorter extubation time and intensive care unit stay. The length of hospital stay was significantly shorter (p = 0.036).
Conclusions. Completion Fontan without the use of cardiopulmonary bypass results in improved immediate postoperative hemodynamics, and decreased use of blood and blood products. The most recent group appears to demonstrate a more rapid recovery time and shorter hospital stay (p = 0.036).
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Introduction
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Since the original description of right heart bypass by Fontan and Baudet in 1971 [1] as treatment for children with tricuspid atresia, many modifications have been suggested. De Leval and colleagues [2] proposed the concept of direct cavopulmonary connection as a way to minimize kinetic energy loss in the Fontan circuit. Interest on the use of an extracardiac inferior vena cava to pulmonary artery conduit has recently been rekindled by Marcelleti and colleagues [3, 4].
The extracardiac modified Fontan has a number of theoretical advantages. Because the conduit is constructed outside the heart, the operation may be performed with the patient supported on cardiopulmonary bypass, without arresting the heart. In selected patients, the extracardiac Fontan may be accomplished without the use of cardiopulmonary bypass. In January 1995, an 18-month-old 11-kg child with hypoplastic left heart syndrome successfully underwent placement of an extracardiac inferior vena cava to pulmonary artery conduit without the use of cardiopulmonary bypass. This patient had bilateral bidirectional Glenn shunts placed at the age of 6 months, and the presence of dual sources of blood flow to the lungs allowed the construction of an inferior vena cava to pulmonary artery conduit without the use of cardiopulmonary bypass. The early gratifying result stimulated us to plan for and develop techniques to accomplish the extracardiac Fontan without the use of cardiopulmonary bypass. This report describes the first 21 consecutive patients and compares their early outcome with 11 other consecutive patients who underwent an extracardiac Fontan with the use of cardiopulmonary bypass during the same time period.
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Material and methods
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Patient population
From January 1995 to October 1997, 32 consecutive patients between the ages of 18 months and 5 years, weighing between 8.6 to 19 kg, underwent the modified Fontan operation with an extracardiac inferior vena cava to pulmonary artery connection (Table 1). Twenty-one patients underwent surgery without the use of cardiopulmonary bypass (No CPB group), while 11 patients had surgery with the use of cardiopulmonary bypass (CPB group). All patients except 1 had previously undergone placement of a superior vena cava to pulmonary artery anastomosis. One patient in each group had bilateral bidirectional Glenn shunts. Cardiac catherization was performed before surgery in all patients. The calculated pulmonary vascular resistance for the No CPB group was 2.0 ± 0.5, while the calculated pulmonary vascular resistance for the CPB group was 2.2 ± 0.9. One patient in the No CPB group has a calculated pulmonary vascular resistance greater than 3.0 Woods unit, while 2 of the CPB group had similarly elevated calculated pulmonary vascular resistance. These differences were not statistically significant. Additional features that may confer increased risk for the completion Fontan operation are listed for both groups in Table 2.
It has been our practice to address all potential anatomical issues at the bidirectional Glenn stage, such that at completion Fontan, connection between inferior vena cava and pulmonary artery only needs to be established. Of these 32 consecutive patients, 26 patients needed inferior vena cava (IVC) to pulmonary artery connections only, and were candidates for completion Fontan without the use of cardiopulmonary bypass. Six patients required pulmonary artery reconstruction and were not felt to be candidates. Of the 26 patients for whom no cardiopulmonary bypass was planned, in 5 patients, cardiopulmonary bypass was used because of difficulty with placement of the IVC cannula.
Surgical technique
In all patients, abnormal systemic arterial to pulmonary arterial collateral vessels are divided as much as possible. In the cardiopulmonary bypass group, standard bicaval venous and ascending aortic cannulation is used. In both groups, right and left branch pulmonary arteries are dissected completely to the hilar branches. All patients are heparinized initially with a 400-U/kg dose of heparin. A vascular clamp is used to isolate the right branch pulmonary artery, diverting superior vena cava blood flow to one lung only (Fig 1). The extracardiac conduit to pulmonary artery anastomosis is then performed using a continuous polypropylene suture technique. Once this anastomosis is completed, superior vena cava blood flow is reestablished to both lungs (Fig 2). Next, an inferior vena cava to atrial shunt is constructed using two right-angle metal-tip venous cannulas (DLP, Grand Rapids, MI). This inferior vena cava to atrial shunt is first placed in the inferior vena cava, and then the atrium, to provide for an alternative pathway for inferior vena cava blood flow to the atrium (Fig 3). A vascular clamp is then placed across the inferior portion of the right atrium, cephalad to the inferior vena cava junction. The inferior vena cava to extracardiac conduit anastomosis is performed (Fig 4). The conduit is then deaired and blood flow from the inferior vena cava is established to the branch pulmonary arteries. The inferior vena cava to atrial shunt is then removed (Fig 5). All patients had a 4-mm fenestration created between the IVC conduit and the pulmonary venous atrium (Fig 6). Because of the concern with the lack of growth of the extracardiac conduit, typically a 20- to 22-mm conduit is used.
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Fig 1. Right branch pulmonary artery has been isolated and a longitudinal arteriotomy made. A vascular clamp is used to direct superior vena cava blood flow to the left lung only.
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Fig 2. The extracardiac conduit to pulmonary artery anastomosis has been completed. Superior vena cava blood flow is now reestablished to both right and left lungs. An inferior vena cava to right atrial shunt has been placed.
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Fig 3. The inferior vena cava is divided from the right atrium and the cardiac end oversewn. The inferior vena cava to the right atrial shunt allows venous blood to continue to return to the right atrium.
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Fig 5. Completion Fontan using an extracardiac conduit has been accomplished without the use of cardiopulmonary bypass. A fenestration may be placed between the extracardiac conduit and the atrium.
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Fig 6. Angiogram demonstrating direct inferior vena cava to main pulmonary artery connection, accomplished without the use of cardiopulmonary bypass. Because of the slight rotation of the patient, the branch pulmonary artery confluence is superimposed on the Glenn anastomosis, which had been placed in the very distal right branch pulmonary artery.
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Analysis
The early postoperative course for these two groups of patients were compared. Variables analyzed include intraoperative and early postoperative hemodynamics, transfusion of blood and blood products in the first 24 hours of mechanical ventilation, length of intensive care unit stay, and length of hospital stay. Categorical variables were compared using Fisher’s exact test, while continuous variables were compared using Student’s t test. All results for the continuous variables are reported as mean ± standard deviation. Results were considered significant if the p value was less than 0.05.
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Results
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There was no operative or hospital mortality in either group. Patients in the No CPB group have significantly lower pulmonary venous atrial pressures at the end of surgery, 6 hours, and 12 hours postoperatively (Table 3). By 24 hours, these differences disappeared (Fig 7). Although proportionately more patients were treated with milrinone in the CPB group, this difference was not statistically significant. Cryoprecipitate and platelet transfusions were significantly more common in the CPB group (Table 4). Although there was a substantial difference in the use of more packed red blood cells for the CPB group, this difference was not statistically significant. Time to extubation, length of intensive care unit stay, and length of hospital stay were not statistically different. However, when the last 12 patients in the No CPB group were compared with the CPB group, the length of ventilator hours, intensive care unit stay, and hospital stay were all substantially different, with the length of hospital stay achieving statistical significance.
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Fig 7. Pulmonary venous atrial pressures at the end of surgery, 6, 12, and 24 hours postoperatively. * p < 0.05.
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Four patients in the No CPB group suffered complications. One patient developed refractory ventricular tachycardia and fibrillation secondary to digoxin toxicity approximately 18 hours after admission to the intensive care unit. A loading dose of digoxin had been given intravenously over a short period of time, 16 to 18 hours postoperatively because of the development of supraventricular tachycardia (even though this patient was on maintenance digoxin preoperatively). Because this was a witnessed event in the intensive care unit, cardiopulmonary resuscitation was begun immediately and the patient brought emergently to the operating room. The child was quickly placed on cardiopulmonary bypass. Despite being on CPB, multiple attempts at defibrillation were unsuccessful. This rhythm was resistant to multiple phamacologic medications. Dilantin briefly interrupted the ventricular tachycardia. Digoxin binding antibody, however, terminated the ventricular tachycardia upon administration. The digoxin level before binding antibody, while the patient was on cardiopulmonary bypass, was well above 5.0 ng/mL. He subsequently developed multiple complications, including renal failure, empyema, and remained in the intensive care unit for a period of 50 days. Fortunately, he has recovered since with no discernable neurologic deficit. One patient suffered a perioperative stroke with the development of left upper extremity paresis. The etiology of this insult remains unclear. Two patients developed superficial wound infections, requiring treatment with antibiotics only. In the CPB group, 1 patient developed a severe tracheitis and required continuous sedation, paralysis and prolonged endotracheal intubation for approximately 13 days. Persistent pleural effusions occurring after 2 weeks, requiring catheter drainage, occurred in 4 patients in the No CPB group and in 5 patients in the CPB group. This difference was not statistically significant.
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Comments
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Surgical management of children with single ventricular physiology has continued to evolve. Many institutions have adopted various approaches with improved early clinical outcomes. Staging the Fontan operation with the bidirectional Glenn anastomosis, and the deliberate creation of a residual right-to-left atrial shunt, have undoubtedly contributed significantly to the improved early outcome of these patients. The investigations of De Leval and associates [2] and Sharma and associates [5] have added further guidance in optimizing the geometry of the cavopulmonary connections. The use of an extracardiac inferior vena cava to pulmonary artery connection has a number of theoretical advantages. It avoids extensive atrial suture lines, exposure of the atrium to higher venous pressures, and theoretically better preservation of kinetic energy in the Fontan circuit. In addition, because the connection is created outside the heart, aortic cross-clamping could be entirely avoided. These theoretical advantages have prompted the application of the extracardiac cavopulmonary connection at our institution.
The outcome after completion Fontan operations may be further optimized with appropriate planning. Meticulous attention is given to achieving appropriate growth and development of branch pulmonary arteries with avoidance of any significant pulmonary artery stenosis (Fig 8). In the newborn period, excessive pulmonary blood flow is avoided. A bidirectional Glenn anastomosis is constructed in early to mid infancy. We have not utilized the hemi-Fontan approach. The superior vena cava (SVC) anastomosis is either diverted toward the right, or more typically toward the left branch pulmonary artery (Fig 9). This tends to encourage appropriate growth of the left branch pulmonary artery, simplifying the later completion Fontan procedure. Having the SVC anastomosis in the very proximal right branch pulmonary artery, occasionally into the proximal left branch pulmonary artery, easily allows construction of the extracardiac Fontan without the use of cardiopulmonary bypass. Need for simultaneous extensive pulmonary artery reconstruction, atrioventricular valve repair, and other procedures would exclude the use of this technique. Theoretically, we avoid the inflammatory sequelae associated with the use of cardiopulmonary bypass. The results in this preliminary group indeed suggest that the immediate hemodynamics are improved compared with the CPB group. The use of blood and blood products are more limited. With further refinement, shorter immediate recovery time and shorter hospital stays are achievable. However, because blood is exposed to the foreign material of the IVC to right atrial shunt, we can not assume the usual inflammatory response to cardiopulmonary bypass is entirely avoided. A prospective randomized trial with measurement of inflammatory mediators may indeed provide interesting results.
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Fig 8. Optimal growth and development of the right and left branch pulmonary arteries in an infant 5 months after the Norwood operation for hypoplastic left heart syndrome.
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Fig 9. Construction of the Glenn anastomosis toward the left branch pulmonary artery in the same patient with hypoplastic left heart syndrome.
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The obvious single disadvantage of the extracardiac Fontan is the lack of growth of the conduit. To minimize the likelihood for replacement of the extracardiac conduit, we have utilized a conduit of 20 to 22 mm in diameter. Whether the IVC will grow to compensate for the fixed length of the conduit remains to be seen. In terms of conduit material, we have generally preferred a valveless ascending aortic homograft conduit. Typically, the homograft is divided at or above the level of the sinotubular ridge. Valve tissue and associated ventricular muscle are excised. Because of the posterior location of the IVC and the more anterior location of the right pulmonary artery, with the potential for right upper pulmonary vein compression, the arch of the homograft provides the perfect geometry for this extracardiac conduit. Since the homograft provides a nonthrombogenic surface, we have not routinely used anticoagulation, except for once daily aspirin.
Conclusions
Review of our early experience with the construction of extracardiac cavopulmonary connection without the use of cardiopulmonary bypass has been encouraging. Early postoperative hemodynamics appear to be improved with less frequent use of blood and blood products. Analysis of the most recent group has also suggested a quicker recovery and shorter hospital stay (Tables 5, 6). Hopefully, with further refinement, this would translate into not only improved short-term but perhaps long-term outcome for our patients as well.
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Acknowledgments
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We acknowledge the support and advise by Drs Robert A. Guyton, Willis H. Williams, Kirk R. Kanter, James M. Bailey, Steve Toscone, Nina Guzetta, Shiva Sharma, David Jones, and the physicians of the Children’s Heart Center, Atlanta, Georgia. We are also grateful for the expert assistance by Vertis Walker in the preparation of this manuscript.
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Footnotes
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This article has been selected for the open discussion forum on the STS Web site: http://www.sts.org/section/atsdiscussion/
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References
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Marcelletti C., Corno A., Giannico S., Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. J Thoracic Cardiovasc Surg 1990;100:228-232.[Abstract]
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Black M.D., van Son J.A.M., Haas G.S. Extracardiac Fontan operation with adjustable communication. Ann Thorac Surg 1995;60:716-718.[Abstract/Free Full Text]
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Sharma S., Goudy S., Walker P., et al. In-vitro flow experiments for the determination of the optimal geometry of the total cavopulmonary connection for surgical repair of children with functional single ventricle. J Am Coll Cardiol 1996;27:1264-1269.[Abstract]
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Top
Abstract
Introduction
