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Eur J Cardiothorac Surg 2003;24:255-259
© 2003 Elsevier Science NL
a Division of Cardiothoracic Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Suite 8527, Philadelphia, PA 19104, USA
b Division of Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Suite 8527, Philadelphia, PA 19104, USA
c Division of Anesthesiology and Critical Care Medicine, The Cardiac Center at The Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Suite 8527, Philadelphia, PA 19104, USA
Received 20 September 2002; received in revised form 24 March 2003; accepted 2 April 2003.
* Corresponding author. Tel.: +1-215-590-2708; fax: +1-215-960-2715
e-mail: gaynor{at}email.chop.edu
| Abstract |
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Key Words: Fontan procedure Reoperation Protein losing enteropathy Pacemaker Heart transplantation
| 1. Introduction |
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| 2. Methods and patients |
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2.2. Patient-related variables
Demographic and anatomic variables were recorded from the medical record. Patients were assigned to one of six diagnostic groups on the basis of cardiac anatomy (Table 1). Details of the previous surgeries including staging, type of Fontan procedure (atriopulmonary connection, lateral tunnel, extra-cardiac conduit), as well as the use of baffle fenestration were also recorded.
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2.4. Statistical analysis
Data are presented as medians and ranges. Specific outcomes evaluated for each procedure category included, hospital mortality (prior to hospital discharge or less than 30 days postoperatively), incidence of postoperative pleural effusions, duration of postoperative mechanical ventilation, duration of postoperative hospital stay, and postoperative complications. Late mortality and need for repeat reinterventions were also noted. Because of the variety of surgical indications and type of procedure, as well as the heterogeneity of the patient population, it was not possible to specifically evaluate predictors of need for reintervention or outcome.
| 3. Results |
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Cardiac diagnoses are presented in Table 1. Interim staging with a superior cavopulmonary connection had been utilized in 48 patients (68%). The median age at Fontan was 2.5 years (0.919 years). An atriopulmonary Fontan or variant had been performed in 17 of the patients (24%), a lateral tunnel Fontan in 49 patients (69%), and an extra-cardiac conduit Fontan in five patients (7%). Baffle fenestration had been performed in 22 patients (30%) and partial exclusion of the hepatic veins performed in an additional 16 patients (23%). The median time to the first reintervention was 3.6 years (range 0.120 years). Indications for reoperation included arrhythmia or pacemaker malfunction, cyanosis, failing Fontan circulation, protein losing enteropathy (PLE), and AVV regurgitation (Table 2). Because of the heterogeneous nature and small number of many of the reoperations, it was not possible to determine if there was a correlation between diagnosis or type of Fontan procedure and need for reoperation except when partial exclusion of the hepatic veins had been performed. There were five early and five late deaths. Four of the five deaths occurred following cardiac transplantation secondary to graft dysfunction and/or multi-organ failure. The other occurred following Fontan revision in a patient with IVC occlusion and multi-organ failure prior to surgery.
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3.1.2. Hepatic vein reinclusion
Sixteen patients underwent reinclusion of partially excluded hepatic veins for progressive cyanosis. The median time from Fontan was 3.6 years (range 0.46.5 years). The median duration of intubation was less than 1 day (range 02 days). The median duration of CICU stay was 1 day (range 119 days). The median duration of hospitalization was 7 days (range 336 days). Perioperative complications included complete heart block in one patient who underwent simultaneous tricuspid valve replacement, postoperative seizure in one patient, cardiac arrest at the time of anesthetic induction in one patient with no sequelae, and a pleural effusion requiring drainage in one patient.
3.1.3. Fontan revision
Thirteen patients underwent Fontan revision during the study period. A Maze procedure was performed in five patients and in five patients, concomitant AVV repair or replacement was performed. Indications included arrhythmia, pathway obstruction, failing Fontan circulation, PLE, and AVV regurgitation. The Fontan had been an atriopulmonary connection or a variant in ten patients and lateral tunnel Fontan in three patients. Six patients were converted to a lateral tunnel type of Fontan, six patients were converted to an extra-cardiac conduit Fontan, and one patient was converted to intra-atrial conduit Fontan. The median time from Fontan to reoperation was 9 years (range 0.820 years). The median duration of intubation was 1 day (range 071 days). The median duration of CICU stay was 3 days (range 171 days). The median duration of hospitalization was 13 days (range 679 days). There was one hospital death and one late death. Postoperative complications included complete heart block in one patient undergoing AVV replacement, chylothorax in one patient, sepsis in two patients, acute renal failure in one patient, persistent PLE in one patient, and respiratory failure requiring tracheostomy in one patient.
3.1.4. Heart transplantation
Nine patients underwent heart transplantation during the study period. The indication was failing Fontan circulation in six patients and unremitting PLE in three patients. The median time from Fontan to reoperation was 6 years (range 0.515 years). The median duration of intubation was 3 days (range 15 days). The median duration of CICU stay was 8 days (range 423 days). The median duration of hospitalization was 80 days (range 21120 days). There were four perioperative deaths occurring at a median of 40 days posttransplant. In addition, one patient developed graft failure and recovered. One patient had stenosis at the superior vena cava (SVC) anastomosis, requiring placement of a stent. There have been two late deaths following transplantation.
3.1.5. Fenestration creation/enlargement
Five patients underwent six procedures for fenestration creation or enlargement. The indication in all patients was PLE. Fenestration of the original baffle was performed in four operations and a new fenestrated patch inserted during two operations. The median time from Fontan to reoperation was 5.6 years (range 513.7 years). The median duration of intubation was 1 day (range 01 day). The median duration of CICU stay was 2 days (range 116 days) with a median hospitalization of 9 days (range 646 days). Postoperative complications included a stroke in one patient and pneumothorax in one patient.
3.1.6. AVV repair/replacement
In addition to the five patients who underwent AVV interventions during the Fontan revision, two patients underwent two isolated AVV procedures. The indication was AVV regurgitation in both. The times from Fontan to reoperation were 0.3 years and 4.9 years. The durations of hospitalization were 15 and 18 days. Postoperative complications included a pleural effusion in one patient.
3.1.7. Other procedures
Fifteen other patients underwent a variety of other procedures including placement of a pericardial window for pericardial drainage, pleural drainage for late effusions, peripheral thrombectomy, reconstruction of discontinuous pulmonary arteries, repair of Fontan baffle leak, and two patients underwent takedown of the Fontan circulation to a superior cavopulmonary connection. There has been one late death following takedown of the Fontan.
| 4. Discussion |
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Kaulitz recently evaluated the need for late interventions in 88 patients followed for at least 5 years following a Fontan type operation [6]. Freedom from reoperation was 82% at 5 years and 76% at 8 years. During the follow-up period, 20 reoperations were performed. Indications for reoperation included anastomotic stenosis, atrial tachyarrhythmia, persistent cyanosis, chronic pericardial effusion, and ventricular outflow tract obstruction. Reoperations included revision of the Fontan pathway anastomosis (n=4), resection of the subaortic obstruction (n=3), closure of residual intra-atrial shunting (n=3), drainage of pericardial effusion (n=5), takedown of the Fontan to a superior cavopulmonary connection (n=1), aortic valve replacement (n=1), Fontan conversion (n=2), and reinclusion of the hepatic veins with an extra-cardiac conduit in a patient who had undergone the Kawashima operation (n=1). In addition, ten patients required placement of an epicardial pacemaker because of bradycardia.
The current study provides an overview of the types of reoperations performed following the Fontan operation in the current era and demonstrates that most reoperations can be accomplished with very low mortality and morbidity. The most common reoperations are related to pacemaker placement and revisions. Sinus node dysfunction is common following both the superior cavopulmonary connection and the Fontan procedure [7]. Patients are also at risk for atrial tachyarrhythmias including atrial flutter especially following atriopulmonary Fontan procedure [3,5,7]. In addition, atrial pacing has been advocated as a potential therapy for PLE [8]. Placement of a pacemaker may be difficult following a Fontan operation; epicardial placement of the leads may be necessary to avoid the risk of thrombosis of the Fontan pathway, lack of available atrial wall for placement of the lead, and inability to access the ventricular chamber [9]. Cohen and colleagues from The Children's Hospital of Philadelphia evaluated the incidence of sinus node dysfunction in 287 patients undergoing a staged Fontan operation between 1990 and 1995 [7]. Of the 95 patients followed for more than 4 years after the Fontan operation, 44% developed sinus node dysfunction. Cohen and colleagues also evaluated the risk of epicardial pacemaker implantation following the Fontan operation and found no difference in the perioperative course for Fontan patients compared to non-Fontan patients undergoing epicardial pacemaker lead placement [9]. There was no mortality in either group and no significant incidence of prolonged pleural effusions in the Fontan group.
Progressive cyanosis resulting from inter-atrial shunting, secondary to baffle fenestration or a baffle leak, may prompt reintervention either surgical or with transcatheter techniques. Previously, at our institution, partial exclusion of a hepatic vein was utilized instead of baffle fenestration. These patients have uniformly developed worsening cyanosis secondary to intra-hepatic shunting. In the current study, reinclusion of the excluded vein was necessary in 16 patients.
Patients in whom an atriopulmonary or atrioventricular Fontan was performed are at risk for progressive right atrial dilation with right atrial thrombus, pulmonary venous obstruction, and atrial tachyarrhythmias and may require revision to an extra-cardiac or intra-atrial conduit Fontan. Performance of a Maze operation and right atrial reduction in association with revision may decrease the severity of atrial arrhythmias, particularly atrial flutter. Marcelletti and associates reported outcome following revision to an extra-cardiac conduit in 31 patients from four institutions. There were two early deaths and one late death [10]. All surviving patients improved symptomatically except for one who ultimately required transplantation. The findings of the current study are similar suggesting that conversion operation can be performed in appropriately selected patients with low mortality and morbidity. These complications are likely to be less frequent in patients who initially underwent either the lateral tunnel Fontan operation or extra-cardiac Fontan operation. Stamm and colleagues from Boston Children's Hospital reported the outcome of 220 patients who underwent lateral tunnel Fontan completion between 1987 and 1991 [3]. There were 12 early deaths and seven late deaths. Four patients required takedown of the Fontan circulation and four patients underwent heart transplantation. The estimated survival was 93% at 5 years and 91% at 10 years with a freedom from failure of 90% at 5 years and 87% at 10 years.
Cardiac transplantation is sometimes necessary for ventricular dysfunction and failure of the Fontan circulation. In the current study, nine patients underwent cardiac transplantation with four deaths resulting in an operative mortality of 44%. Carey and associates reported outcomes for cardiac transplantation in nine patients (four adults and five children) with a failing Fontan circulation [11]. The operative mortality was 33% with eight early deaths, secondary to graft failure in two cases and postoperative hemorrhage in the other. Early mortality for patients with the Fontan circulation undergoing cardiac transplantation is greater than other patients with congenital heart disease or those with cardiomyopathy. The patients are often severely debilitated with poor nutritional status. Repeat sternotomy in patients who have undergone multiple previous operations is associated with an increased risk of postoperative hemorrhage. Complex reconstruction of the pulmonary arteries, as well as the systemic and pulmonary veins is often necessary. In addition, transplantation is often performed for patients with PLE, which may not resolve following transplantation. Nevertheless transplantation may be the only therapeutic option for patients with a failing Fontan circulation.
PLE is a particularly difficult problem following the Fontan and is often resistant to therapy [12]. In addition to transplantation, several patients with PLE in the current series underwent baffle fenestration and placement of an atrial pacemaker [8,13]. These interventions are designed to increase systemic output and mesenteric perfusion. These interventions can be safely accomplished but the long-term results are less satisfactory.
There are several limitations to the current study. It is retrospective and evaluates reoperation for all patients during a specific era rather than longitudinally evaluating a cohort of patients. The study addresses only the short-term outcomes and not the long-term effectiveness of the intervention. For example, PLE may not resolve following successful baffle fenestration or successful cardiac transplantation resulting in a poor long-term outcome despite a technically successful reoperation. In addition, the patient population is relatively small and heterogeneous with a large variety of reoperations precluding statistical analysis of predictors of outcome.
The current study demonstrates that a variety of reoperations may be required following the Fontan operation. The most common procedures are related to pacemaker insertion and revision. Most reoperations can be accomplished with minimal mortality and morbidity. The outcome for patients who require cardiac transplantation following the Fontan operation remains poor.
| Footnotes |
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| References |
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