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Ann Thorac Surg 1996;61:1797-1804
© 1996 The Society of Thoracic Surgeons
Departments of Pediatrics, Thoracic-Cardiovascular Surgery, and Anesthesiology, Loyola University Medical Center, Maywood, and Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois
Accepted for publication February 13, 1996.
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Abstract |
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Methods. Over a 34-month period, 9 patients underwent Fontan revision. The mean age was 11 ± 5 years and the mean interval from Fontan operation to revision was 3 ± 2 years. The reason for revision included marked impairment in exercise capacity, inability to go to school consistently, and chronic fatigue in 6 patients, 3 of whom also had serious atrial arrhythmias. Five of the 6 patients had a classic Glenn shunt. The mean right atrial pressure was greater than the pressure of the Glenn shunt (20 ± 1.6 versus 17 ± 0.8 mm Hg). Three of the 6 patients also showed a significant gradient between the right or left pulmonary artery wedge and ventricular end-diastolic pressure, indicating pulmonary vein obstruction from the bulging atrial septum or partitioning patch (13 ± 3 versus 6.8 ± 1 mm Hg). The remaining 3 patients had revision because of malabsorption (1), hepatomegaly and obstructed right pulmonary veins from bulging atrial septum (1), and tricuspid insufficiency (1). Fontan revision was accomplished with creation of a lateral atrial tunnel and Glenn reconnection in 6 patients, Glenn reconnection in 2, and creation of a lateral atrial tunnel in 1. Four patients had additional procedures.
Results. One patient died of Pseudomonas pneumonia. Early extubation, chest tube removal, and postoperative hospital discharge were accomplished in 8 patients (mean = 1.4 ± 1, 2.8 ± 1, and 8 ± 3 days, respectively). One patient died 8 months postoperatively of brain damage after ventricular fibrillation from attempted cardioversion for atrial flutter. The remaining patients had marked improvement in exercise capacity with ability to consistently go to school, improvement in duration and tolerance to arrhythmias on less medication, and resolution of malabsorption up to 37 months postoperatively (mean, 20 ± 12 months).
Conclusions. We conclude that creation of lateral atrial tunnel with excision of a bulging atrial septum or atrial partitioning patch that causes pulmonary venous obstruction, reconnection of the Glenn shunt, which allows better distribution of flow based on the pulmonary vascular bed and resistance of each lung, or a combination of these procedures will improve Fontan patients.
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Introduction |
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The Fontan operation has been well established in the correction of patients with a single functioning ventricle. The mortality rate has been negligible in tricuspid atresia and single ventricle with pulmonary stenosis [1, 2]. In the presence of risk factors such as increased pulmonary blood flow, pulmonary artery distortion, left-sided obstruction, and anomalous veins, the perioperative mortality rate has also decreased because of staged approaches and early correction preserving the myocardial integrity [3–6]. Staged approaches include creation of a Glenn shunt followed by a completion Fontan operation some time later or leaving an atrial fenestration at the time of the Fontan operation [7–11]. As the perioperative mortality continues to decline and late outcome is forthcoming, attention is now being directed toward improving the quality of life of surviving patients.
There is a significant number of Fontan patients who suffer from incapacitating arrhythmias and impaired exercise tolerance in the absence of residual defects or whose residual defects appear inconsequential. Having reoperated on some of these patients in an attempt to improve their hemodynamics, we reviewed our experience to determine whether such approach is warranted.
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Material and Methods |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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).
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Two patients had single ventricle who both had coarctation repair and pulmonary artery banding performed in infancy. Atrial septectomy was also performed in 1 patient because of mitral valve stenosis. Main pulmonary artery to ascending aorta connection was performed at 4 years and 10 years, respectively, because of subaortic stenosis. One patient also had a subclavian pulmonary artery shunt. Both patients had a Glenn shunt established before the Fontan operation.
Three patients had tricuspid atresia. Two patients had a central systemic pulmonary artery shunt and modified Blalock-Taussig shunts (bilateral in 1 patient). The Glenn shunt was also established in both patients before the Fontan operation. The third patient only had a modified Blalock-Taussig shunt before the Fontan operation.
Two patients had pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle. One patient also had significant sinusoids. One patient who had multiple systemic pulmonary artery shunts underwent creation of a Glenn shunt before the Fontan operation. The other patient with sinusoids only had a modified Blalock-Taussig shunt done in the newborn period.
Of the remaining 2 patients, 1 had transposition, ventricular septal defect, hypoplastic right ventricle, and tricuspid and subpulmonary stenosis. This patient did not have a palliative operation before the Fontan operation. The other patient had double-outlet right ventricle, mitral atresia, hypoplastic left ventricle, and subpulmonary stenosis. This patient had a modified Blalock-Taussig shunt at 1 year of age.
Fontan Operation
In 6 patients who all had a classic Glenn shunt, a right atrial to left pulmonary artery connection was made using the atrial appendage as a flap for the posterior part of the anastomosis. A polytetrafluoroethylene patch was used anteriorly to complete the anastomosis. In 5 of the 6 patients, the Glenn shunt was established 1 to 4 years (mean, 2.2 ± 1 years) before the Fontan procedure. One had an obligatory Glenn shunt established at the time of a fenestrated Fontan procedure [10]. In 2 patients who had a fenestrated Fontan procedure, obligatory Glenn shunts were established to the left pulmonary artery and the distal superior vena cava was connected to the right pulmonary artery for the Fontan connection [10]. In the remaining patient who did not have a Glenn shunt, a right atrial to main pulmonary artery connection was established after its transection and mobilization.
Closure of the atrial septal defect was performed in 5 patients. Three of these patients had tricuspid atresia, 1 had closure of a stenotic tricuspid valve, and 1 had minute right ventricle associated with pulmonary atresia and intact ventricular septum. In 2 of these patients, the atrial septal defect closure was accomplished 1 and 2 months after the fenestrated Fontan procedure.
In the remaining 4 patients, atrial partitioning [12] was performed in 3 and a lateral atrial tunnel was constructed in 1. Two of these patients had single ventricle, 1 had pulmonary atresia and hypoplastic right ventricle with sinusoids, and 1 had double-outlet right ventricle with mitral atresia and hypoplastic left ventricle.
Reason for Fontan Revision
Six patients had marked impairment in exercise capacity, were unable to go to school consistently, and took long day naps. Three of these patients had atrial arrhythmias that were difficult to control and were treated with various antiarrhythmic agents (procainamide, verapamil, and flecainide). In 1 of these 3 patients a sequential pacemaker was also implanted. One patient with tricuspid atresia continued to have moderate mitral regurgitation after mitral valvuloplasty at the time of the Fontan operation.
In 5 of the above 6 patients, a right-sided Glenn shunt and right atrial to left pulmonary artery connections were present. Cardiac catheterization before the Fontan revision showed the mean right atrial pressure to be greater than the Glenn shunt pressure (20 ± 1.6 versus 17 ± 0.8 mm Hg). In 1 patient, the Fontan resistance that was calculated separately from the Glenn shunt was greater (3.5 versus -1.8 U/m2). One patient showed unexpected right pulmonary artery stenosis distal to the Glenn shunt anastomosis with a 6-mm Hg gradient. In 2 of these 5 patients, who both had atrial partitioning for single ventricle, there was a gradient between the right or left pulmonary artery wedge and the single ventricle end-diastolic pressure, indicating some compression of the pulmonary veins by the bulging partitioning patch (12.5 and 12 versus 6 and 7.5 mm Hg). In the sixth patient, who had closure of the tricuspid valve and atrial septal defect and direct right atrial to main pulmonary artery connection for transposition with hypoplastic right ventricle, the right atrial pressure was low (13.5 mm Hg). However, there was a significant gradient between the right pulmonary artery wedge and the left ventricular end-diastolic pressure (11 versus 6 mm Hg), indicating obstruction of the right pulmonary veins by the bulging atrial septum (Fig 1). There was no gradient between the left pulmonary artery wedge and the left ventricular end-diastolic pressure.
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) due to compromise of the right upper lobe by the subclavian pulmonary artery shunt. The mean superior vena caval pressure was greater than the inferior vena caval pressure (14 versus 12 mm Hg).
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). The right atrial pressure also increased from 12 to 17.5 mm Hg. No gradient was demonstrated between the left pulmonary artery wedge and left ventricular end-diastolic pressure.
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). The atrial fenestration was closed a month later. This patient presented with moderate tricuspid valve insufficiency and a small residual atrial septal defect.
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) was constructed after excision of the atrial partitioning patch in 2 patients and the atrial septum in 5 patients. Of the remaining 2 patients, 1 already had a lateral tunnel created at the time of the Fontan operation and the other already had an atrial partitioning quite similar to a lateral atrial tunnel. In 8 patients, the Glenn shunt was taken down by reconnecting the superior vena cava to the atrial tunnel. This was performed in 6 patients with a right-sided Glenn shunt by making a longitudinal incision on the superior vena cava proximal to the ligature at the cavoatrial junction and a similar incision on the atrial side, avoiding injury to the sinus node (see Fig 5). The back wall was enlarged by approximation of the upper and lower edges of the incision on both sides. A polytetrafluoroethylene patch was placed anteriorly to complete the anastomosis. In 2 patients with the Glenn shunt connecting the superior vena cava to the left pulmonary artery, Glenn reconnection was accomplished with incisions at the heel of the Glenn shunt and the top of the right atrium. The posterior edges were approximated directly and the polytetrafluoroethylene patch was placed anteriorly. This anastomosis could be performed easily without cutting through the sinus node because of the proximity of the heel of the Glenn shunt to the right atrium. In the remaining patient, who had obstruction of the right pulmonary veins from the bulging atrial septum and who did not have a Glenn shunt, lateral atrial tunnel was the only procedure performed.
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Results |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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Eight patients survived the Fontan revision. Postoperative hospital stay ranged from 4 to 14 days (mean, 8 ± 3 days). Extubation was accomplished from the day of operation to 4 days postoperatively (mean, 1.4 ± 1 days). Two patients were extubated on the day of operation. The chest tubes were removed from 1 to 5 days postoperatively (mean, 2.8 ± 1 days).
In 2 patients who had atrial arrhythmias preoperatively and were receiving procainamide and flecainide, respectively, atrial arrhythmias developed postoperatively. Continuous procainamide infusion was given for 4 days postoperatively in both patients. Two patients required exploration for bleeding.
Late Results
The 8 surviving patients were followed up from 3 months to 37 months postoperatively (mean, 20 ± 12 months). Five patients whose current ages range from 11 years to 17 years (mean, 15 ± 2 years) and who had marked impairment in exercise capacity and chronic fatigue were unable to go to school consistently preoperatively because of lack of energy. These children were reported preoperatively to take long naps during the day. Postoperatively these children showed significant improvement. The parents reported fewer naps, fewer school absences, and markedly improved general activity.
Three of the above 5 patients who also had preoperative arrhythmias that were difficult to control despite antiarrhythmic medication showed significant improvement up to 37 months postoperatively. One patient who was treated with procainamide preoperatively is currently receiving diltiazem and digoxin. The other 2 patients who were given verapamil and flecainide, respectively, preoperatively are currently receiving diltiazem and propranolol, respectively. Although these patients would occasionally have atrial tachycardia, the events would be shorter lasting 10 to 15 minutes compared with 2 to 3 hours preoperatively. Additionally, symptoms associated with these events preoperatively such as sweating, nausea, and weakness were less or no longer present after revision.
The sixth surviving patient, who had malabsorption, had complete resolution of malabsorption with disappearance of hypoalbuminemia 3 months postoperatively. The seventh patient, a 3.8-year-old infant with obstruction of the right pulmonary veins by a bulging atrial septum, showed no obstruction on postoperative echocardiograms after creation of a lateral atrial tunnel and reconnection of the Glenn shunt.
The last patient, who had double-outlet right ventricle and hypoplastic left ventricle and underwent Glenn reconnection and tricuspid valvuloplasty, initially did well but was found to have atrial flutter with marked myocardial dysfunction on routine follow-up 8 months postoperatively. The ventricular rate was 134 beats/min. Cardioversion caused ventricular fibrillation. Although the patient was resuscitated, severe brain damage was sustained and the patient died 5 days later.
Cardiac catherization performed in 2 patients (patients 1 and 3; see Table 1
) 36 and 12 months postoperatively, respectively, showed improved hemodynamics. The first patient, who had a mean Fontan and Glenn pressure of 19 and 16.5 mm Hg, respectively, now had superior and inferior vena caval pressure of 15 mm Hg. The preoperative gradient between the left pulmonary artery wedge and ventricular and end-diastolic pressure had disappeared. The calculated cardiac index also improved to 3.47 from 2.05. In the other patient, the mean right atrial pressure preoperatively was 22 mm Hg. The Glenn shunt pressure was not obtained. Postoperatively, the superior and inferior vena caval pressure was 12 mm Hg. The left pulmonary artery wedge pressure was 8 mm Hg. The cardiac index improved to 3.9 from 2.6.
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Comment |
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TopFootnotesAbstractIntroductionMaterial and MethodsResultsCommentReferences |
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Because there is a variable interval, which could be significant, between the creation of the Glenn shunt and the completion of the Fontan operation, the pulmonary vascular resistance of the right lung might be less than that of the left lung, as seen in 1 of our patients. Because of this possibility in some Fontan patients and the presence of disproportion of flow inherent with the Glenn shunt, its takedown or reconnection should result in improved hemodynamics with decreased right atrial pressure, increased exercise capacity, and presumably less arrhythmia.
Compression of the right pulmonary veins from the bulging atrial septum in Fontan patients who had an intact atrial septum or simple closure of the atrial septal defect is not a well-recognized problem. It should be looked for in Fontan patients who present with decreased exercise tolerance, atrial arrhythmias, or undue hepatomegaly. Because patients are well diuresed at the time of cardiac catheterization, pulmonary venous obstruction may not be obvious, as seen in 1 of our patients. Infusion of saline solution as was performed in our patient should be done to unmask the obstruction. Creation of a lateral atrial tunnel and atrial septectomy solve the problem.
Detection of a gradient between the right or left pulmonary artery wedge and the systemic ventricular end-diastolic pressure in 2 patients with atrial partitioning was initially puzzling to us. One patient with mitral valve stenosis had a limited partitioning patch at the time of the Fontan operation because we were already aware of the possible obstruction of the right-sided tricuspid valve in these patients. In the other patient, the mitral valve was normal. Angiography and echocardiography did not clearly show the source of the gradient. It is possible, however, that because the posterior part of the atrial septum was used in the placement of the atrial partitioning patch, obstruction of the right pulmonary veins could still occur from the bulging atrial septum and partitioning patch. This obstruction is also possible with creation of lateral atrial tunnel if the posterior suture line is placed close to the atrial septum. Obstruction of both pulmonary veins would occur with redundant atrial partitioning patch.
Creation of a lateral atrial tunnel is being recommended because of the theoretic advantage of better hemodynamics due to maintenance of laminar flow and minimal loss of energy in a dilated right atrium [15]. It is also being recommended to minimize atrial arrhythmias, presumably by reducing the atrial muscle exposed to higher right-sided pressures. However, Rodefield and colleagues [16] reported experimental results that the lateral atrial tunnel itself predisposes to atrial flutter because of unidirectional block at the suture line. Because we did not have preoperative and postoperative Holter recording or electrophysiologic tests to compare, we could not definitely say that our patients had less arrhythmia postoperatively. Our patients with arrhythmias, however, subjectively had shorter periods of palpitations that were better tolerated on considerably less medication postoperatively. Improvement in our patients with arrhythmias might be more related to improvement of hemodynamics with Glenn reconnection [17, 18] and excision of the bulging septum or atrial partitioning patch compressing the right pulmonary veins.
The disappearance of malabsorption in 1 patient after reconnection of the Glenn shunt that was connected to the right pulmonary artery with a reduced vascular bed was a pleasant surprise to us. We had reservations in performing the procedure because of the presence of low Glenn shunt pressure and a small pressure gradient between the superior and inferior venae cavae.
Before undertaking Glenn shunt reconnection in patients being considered for Fontan revision, careful evaluation of the Glenn shunt should be performed. In the absence of symptoms related to the Glenn shunt, stenosis can still be present as seen in 1 of our patients. We could have simply reconnected the Glenn shunt, thereby missing the right pulmonary artery stenosis entirely.
The current preferred technique in patients who are good candidates for the Fontan procedure consists of direct cavopulmonary artery connection and creation of a lateral atrial tunnel. This approach undoubtedly will reduce the problems of disproportion of flow associated with the classic Glenn shunt and obstruction of the pulmonary veins from bulging atrial septum or atrial partitioning patch.
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