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Ann Thorac Surg 2000;69:1873-1879
© 2000 The Society of Thoracic Surgeons
a Section of Cardiothoracic Surgery, Riley Hospital for Children and Indiana University Medical Center, Indianapolis, Indiana, USA
Address reprint requests to Dr Bando, Department of Cardiovascular Surgery, National Cardiovascular Center, 57-1, Fujishirodai, Suita, Osaka, 5658565, Japan
e-mail: kobando{at}hsp.ncvc.go.jp
Presented at the Thirty-sixth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 31Feb 2, 2000.
| Abstract |
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Methods. Preoperative risk factors and operative results were analyzed by multivariate techniques in 129 patients undergoing modified Fontan operations since March 1988.
Results. Overall early and late mortality was 5.4% and 0.8%, respectively. Before 1993, completed Fontan operation using right atrial to pulmonary artery anastomosis without fenestration was performed in the majority of patients (44 of 58; 76%). During this period, 10 of 17 patients at high risk had completed Fontan with three takedowns. In 1994, the staged hemi-Fontan and modified Fontan with a lateral tunnel anastomosis and with or without small fenestration (2.5 to 4 mm) were introduced. The majority of patients at high risk during this period underwent hemi-Fontan followed by fenestrated Fontan with no takedowns. Late atrial dysrhythmias occurred in 6 patients (4.7%), generally with larger fenestrations or right atrial to pulmonary anastomoses. Three patients (2.3%) had a stroke, 2 with large (
4 mm) fenestrations. Of 38 fenestrations, 32 (84%) closed spontaneously by 1 year. No protein-losing enteropathy occurred. Most patients (118 of 121) were in New York Heart Association class I/II 4.5 years postoperatively. By multivariate analysis, only Downs syndrome (p < 0.001) predicted early mortality, whereas both Downs syndrome and a systemic right ventricle decreased late survival (p < 0.006).
Conclusions. Proper selection of patients for modifications of the Fontan procedure resulted in excellent early and late survival with a low incidence of atrial dysrhythmia and stroke. Midterm functional outcomes were excellent.
| Introduction |
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Early survival has improved from 75% to 83% in the 1970s to more than 90% in the current era, despite application of the operation to patients with complex forms of single ventricle and to those with hemodynamic or other factors previously considered to carry higher risk [912]. As more patients survive the operation and the duration of follow-up increases, the continuing risk of late failure and a poor functional outcome in some long-term survivors has become clear [11, 1315]. Atrial dysrhythmias are common, exercise capacity is reduced, and some patients develop thromboembolic events or a problematic protein-losing enteropathy.
The purpose of this study was to review a single-center experience that has evolved over a decade and to determine the patient- and procedure-related risk factors influencing early and late outcomes after the Fontan procedure.
| Material and methods |
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Surgical technique
The surgical technique of the modified Fontan procedure has evolved over the last 10 years at our institution. Initially (before 1993), the Fontan procedure was completed without a prior hemi-Fontan using a right atrial appendage to pulmonary artery anastomosis and no fenestration. In 1994, the first-stage hemi-Fontan and modified Fontan with a lateral tunnel anastomosis (with or without a small fenestration of 2.5 to 4 mm) were introduced. Our current surgical technique is as follows.
The hemi-Fontan procedure
Using median sternotomy, an arterial cannula is placed in the aorta. A straight venous cannula is placed in the right atrium and a small right-angled tube is inserted high in the superior vena cava. Immediately after initiating bypass, the Blalock shunt (if present) is ligated. The azygos vein is dissected free, doubly ligated, and divided. The superior vena cava is divided between clamps 3 to 4 mm above the atriocaval junction. Using continuous polydioxyanone sutures, an end-to side anastomosis is performed between the cephalic end of the divided superior vena cava and a longitudinal arteriotomy placed in the right medial superior aspect of the pulmonary artery. After snaring the tourniquet around the inferior vena cava cannula, the right atrium is opened (parallel to the atrioventricular groove) and a small patch of PTFE is sutured approximately 1 cm below the superior margin of the cardiac end of the divided superior vena cava. The cardiac end of the superior vena cava is then anastomosed to the pulmonary artery (Fig 1).
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Strategy of postoperative anticoagulation
Our current regimen for postoperative anticoagulation after either a staged or nonstaged Fontan procedure includes aspirin 10 mg/kg per day and warfarin 0.5 to 1 mg/kg per day. The International Normalized Ratio is measured only during the initial admission for a Fontan procedure and is adjusted to approximately 1.5. For the last 2 years, subcutaneous low molecular weight heparin (1 mg/kg every 12 hours) was used for 2 days immediately after surgery (but not before 1996). Moreover, the majority of patients after 1994 received ACE inhibitors.
Definition of outcome events
Early mortality was defined as death during the initial hospitalization or within 30 days of operation. Late mortality was defined as death after discharge after the initial hospitalization. Atrial dysrhythmia was defined as the new onset of supraventricular tachycardia, including atrial flutter and atrial fibrillation or need for permanent pacemaker implantation, at any time after the Fontan operation.
Statistical analysis
Early outcome was treated as a binary response variable. Univariate analyses for categoric predictor variables (eg, diagnostic group) (see Table 1) were carried out by means of
2 and Fishers exact tests. Cut points for continuous variables were constructed. For example preoperative pulmonary vascular resistance was classified as either low or high (< 3 WU vs
3 WU). Baffle fenestration was classified as present or absent. To assess the simultaneous effects of perioperative characteristics on early outcome, variables that were significant at the 0.1 level in a univariate analysis were included in a multivariate logistic regression model. A significance level of 0.05 was required for retention in the multivariate model.
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| Results |
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Late atrial dysrhythmias occurred in 6 patients, all with a right atrial appendage to pulmonary artery anastomosis. No patients with lateral caval tunnel Fontan have experienced significant dysrhythmias. Actuarial freedom from atrial dysrhythmia at 10 years was 92.0% ± 0.4% (Fig 2). Thromboembolic events occurred in 3 patients, 2 with large fenestrations ( > 4 mm) and 1 without fenestration. All of these patients received aspirin and low-dose warfarin as previously described.
Fate of fenestration
Of the 46 patients with baffle fenestrations, 2 patients (1 patient with a 6-mm and 1 with a 2.5-mm fenestration) died (Table 5). Only 64% of fenestrations greater than 4 mm closed spontaneously within 1 year after Fontan, whereas 90% of small (2.5 mm) fenestrations closed spontaneously 1 year after surgery. Device closure was undertaken in 1 patient and 2 other patients underwent surgical closure of the fenestration.
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| Comment |
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Elevated pulmonary artery pressure, higher pulmonary vascular resistance, and pulmonary artery distortion were widely recognized risk factors for early failure [14, 15]. In this study, neither elevated pulmonary arterial resistance (> 3 Wood Units) higher pulmonary arterial pressure (mean, > 15 mm Hg), nor distortion of pulmonary artery was an independent predictor of early death. Improved preoperative medical management and surgical palliation reduced the development of elevated pulmonary arterial pressure, with only 7 of 106 patients demonstrating significant pulmonary hypertension. It remains unclear whether the staging of the cavopulmonary connection is actually responsible for neutralizing these risk factors, as these variables had a large number of missing values that limited the power of the analysis.
Younger age at operation was previously reported as an independent risk factor for early failure [11, 1416]. However, the impact of age on operative outcome has been neutralized in the last 10 years of this study. In our analysis of the data, neither age less than 2 years nor age less than 4 years at the time of a Fontan procedure was associated with increased early mortality (data not shown). Although Fontan candidates of younger age have smaller anatomical structures and a more reactive pulmonary vascular bed after cardiopulmonary bypass, early Fontan has several advantages, including (1) preservation of ventricular function through relief of chronic volume overload and chronic hypoxemia, and (2) protection of the pulmonary vasculature by removing systemic to pulmonary arterial shunts [17].
(Figures 4 and 5) The only risk factor for early mortality by multiple regression analysis was Downs syndrome. Children with Downs syndrome are known to have more reactive pulmonary vasculature and tend to be require longer endotracheal intubation. However, the retrospective nature of this study and the fact that there were only 3 patients with Downs syndrome would not allow us to analyze this further.
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By multiple regression analysis, only Downs syndrome and the presence of a systemic right ventricle seemed to be risk factors for late death after the modified Fontan procedure. Pulmonary artery distortion, preoperative pulmonary arterial pressure, and pulmonary vascular resistance were not associated with higher a risk for late death. No significant differences were identified between late outcomes in patients with either staged Fontan or fenestration and those with single-stage Fontan. These results may be due to the fact that the majority of the high-risk patients who had elevated pulmonary arterial pressure or resistance and who had pulmonary arterial distortion were treated with either a staged Fontan or fenestration, or with both.
Atrial dysrhythmias and poor functional outcome have been major concerns during late follow-up after a Fontan procedure. Two important studies from Boston and New York have shown that both the staged approach to a Fontan and total cavopulmonary connection are associated with sinoatrial node dysfunction early after Fontan type procedures [18, 19]. Both studies suggested that repeated dissection in the region of sinoatrial node during staged Fontan procedures resulted in an additive risk of abnormal sinoatrial node function and reported a 23% to 50% incidence of late atrial dysrhythmias in these patients. Our current results with a 92% freedom from late atrial arrhythmias at 10 years after the Fontan procedure compares favorably with these earlier reports and is consistent with their hypothesis that avoiding repeat dissection would improve late outcomes. In our series, all of the patients with late arrhythmias had right atrial to pulmonary arterial anastomosis and no patient with a hemi-Fontan has yet developed a significant late dysrhythmia. With this technique only the superior vena caval flow is committed to the pulmonary artery, as with a standard bidirectional Glenn procedure, and the subsequent intervention may be done completely within the atrium, avoiding dissection in the region of the SA node or its blood supply [18]. Another consideration is that in our technique of fenestrated Fontan, the lateral tunnel patch is placed in such a way as to avoid suturing to, or near, the crista terminalis. Additionally, the suture line is performed with multiple shallow bites rather than broad, deep advances. This may also help to reduce the incidence of late dysrhythmias after the modified Fontan procedure.
This review of a single center experience with the Fontan procedure has several limitations. First, the mean duration of follow-up (especially for the staged hemi-Fontan and subsequent completion of Fontan with lateral tunnel and baffle fenestration) is only 4.5 years. Thus, it may be too early to draw any definite conclusions regarding late outcomes, as the most statistically powerful determinant of morbidity was a longer duration of follow-up [20]. Second, because data regarding pulmonary vascular resistance and the size of the pulmonary arteries were missing in some patients because of the retrospective nature of the study as well as the low incidence of either death or late arrhythmias, the statistical power may not have been sufficient to identify all major risk factors in a sample of 129 patients.
Despite these limitations, we conclude that with proper selection and preoperative management of patients, modifications of the Fontan procedure result in excellent early and late survival with a low incidence of atrial dysrhythmia and stroke. Although midterm functional outcome is good, long-term follow-up is certainly warranted to continue the assessment of these operations.
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