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Ann Thorac Surg 1998;65:171-175
© 1998 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

One-Lung Fontan Operation: Hemodynamics and Surgical Outcome

Division of Pediatric Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

Accepted for publication July 15, 1997.

Dr Zachary, Division of Pediatric Cardiology, The Milton S. Hershey Medical Center, PO Box 850, Hershey, PA 17033.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Background. This study examined the results of a Fontan operation for patients with acquired atresia of one main branch pulmonary artery.

Methods. The data for 7 patients identified as having a hypoplastic left pulmonary artery discontinuous from the right pulmonary artery were compared with those for 65 patients with continuous pulmonary arteries who consecutively underwent a completion Fontan procedure.

Results. No significant differences were found preoperatively with respect to right atrial pressure, aortic saturation, ventricular end-diastolic pressure, pulmonary artery pressure, pulmonary blood flow, or pulmonary vascular resistance. In the first 24 postoperative hours, there were no significant differences in heart rate, urine output, systemic venous pressure, or pulmonary venous pressure. Also, data regarding hospitalization length, effusions, and mortality were similar between the two groups. Postoperative systemic arterial saturation was lower in the one-lung group. There were no early postoperative deaths in the one-lung group, and 5 of the 7 patients are long-term survivors.

Conclusions. A completion Fontan procedure can be successfully performed in patients with a hypoplastic and discontinuous left pulmonary artery, although postoperative systemic arterial saturation is not as high as in patients with continuous pulmonary arteries.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Assessment of the pulmonary artery architecture is an important component of the preoperative evaluation of a patient with single-ventricle physiology who is to undergo a Fontan operation [1][2][3][4][5]. Most patients, even after previous palliation, are found to have confluent pulmonary arteries, although some degree of distortion is not uncommon [6]. We have noted a subgroup of patients in whom pulmonary artery distortion is so severe that the right and left main branch pulmonary arteries are discontinuous and the left pulmonary artery is seriously hypoplastic or atretic. In some instances, the branch pulmonary arteries can be made confluent once again and the left pulmonary artery, augmented. However, there are occasions when this is either unsuccessful or not possible. For these patients, completion of a Fontan operation would direct systemic venous blood flow solely to one pulmonary artery with the concomitant physiologic consequences. In this situation, the morbidity, the mortality, and the physiology of Fontan completion have not been addressed. The purpose this study is to review our experience with the Fontan operation in patients with acquired atresia of one main branch pulmonary artery.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Review of the cardiology patient database at The Children’s Hospital of Philadelphia from 1991 to 1995 identified 7 patients who, prior to completion of a Fontan operation, had a left pulmonary artery that was hypoplastic and discontinuous from the right pulmonary artery. This was visualized by angiography, magnetic resonance imaging, or both. All 7 patients underwent a completion Fontan operation. No patient identified as having discontinuous pulmonary arteries was excluded from a completion Fontan. A retrospective review of cardiac catheterization data, operative notes, and medical records provided the preoperative characteristics and postoperative courses of these 7 patients. Their preoperative and postoperative data were compared with those of 65 patients with continuous pulmonary arteries who consecutively underwent a completion Fontan procedure by one surgeon at The Children’s Hospital of Philadelphia after June 1991.

Preoperative hemodynamic data obtained by cardiac catheterization included mean values for right atrial pressure, aortic saturation, ventricular end-diastolic pressure, and pulmonary artery pressure. Derived values included calculated pulmonary blood flow and pulmonary vascular resistance. Postoperative data included mean values over the first 24 hours for heart rate, urine output, systemic venous pressure, and pulmonary venous pressure. Also considered were systemic arterial saturation after extubation, length of hospital stay, presence of effusions persisting for more than 14 days and requiring intervention, and early (< 30 days) and late mortality.

The two-way unpaired Student’s t test was used to compare means of both groups when the distribution was normal. Nonparametric data were analyzed using the Mann-Whitney U test. Categoric data analysis was done with Fisher exact test.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Study Group
The study group (group 1 or one-lung group) comprised 7 patients, 6 boys and 1 girl. The anatomic cardiac diagnoses are listed in Table 1. All patients except the patient with pulmonary atresia underwent stage 1 Norwood reconstruction in the newborn period followed by a hemi-Fontan operation when they were between 5 and 7 months of age. The patient with a single ventricle and pulmonary atresia underwent a modified left Blalock-Taussig shunt at 2 days of age followed by a hemi-Fontan procedure at 21 months of age.

Control Group
The control group (group 2 or two-lung group) comprised 65 consecutive patients undergoing completion Fontan with confluent and nonhypoplastic pulmonary arteries. There were 40 boys and 25 girls. The anatomic diagnoses are shown in Table 1. As in group 1, all patients underwent staged Fontan reconstruction involving a hemi-Fontan operation before Fontan completion. The surgical techniques for completion Fontan included a lateral tunnel type of intraatrial cavopulmonary connection with partial hepatic vein exclusion (8 patients), a fenestrated lateral tunnel type intraatrial baffle (53 patients), a lateral tunnel type intraatrial baffle with no fenestrations (2 patients), and an extracardiac conduit with a fenestration to the right atrium (2 patients). Of the fenestrated baffles, 26 had four to six fenestrations made with a 14-gauge needle, and 27 had three 2.7-mm punch holes. Each of the extracardiac conduits had a punch anastomosis to the right atrial appendage. In 1 patient, the punch size was 3.5 mm and in the other, 4.8 mm. The mean age at completion Fontan operation was 27.3 ± 40.3 months (range, 11 months to 14 years).

Preoperative Analysis
Table 2 displays the preoperative hemodynamic data of the two groups. Both mean values and standard deviations were similar for each hemodynamic variable compared between the two groups. No significant differences were found in right atrial pressure (6.0 mm Hg versus 6.2 mm Hg), aortic saturation (82.4% versus 84.2%), ventricular end-diastolic pressure (7.9 mm Hg versus 8.2 mm Hg), pulmonary artery pressure (11.7 mm Hg versus 11.2 mm Hg), pulmonary blood flow (2.1 L · min^-1 · m^-2 in both groups), or pulmonary vascular resistance (2.2 Wood units/m² versus 2.5 Wood units/m²). Comparisons of age revealed no significant difference.

Two deaths occurred in group 1. One was related to recurrent pleural effusions 17 months after a completion Fontan procedure. The other was associated with a fulminant respiratory syncytial viral infection. In group 2, 2 patients had severely diminished ventricular function in the immediate postoperative period resulting in early death, and 1 patient sustained a cardiac arrest after sedation (30-day mortality rate, 4.6%). Five late deaths occurred in patients with chronic or recurrent pleural effusions, and 1 patient died suddenly at home without a clear cause of death.

The mean length of follow-up for group 1 is 30 ± 23 months (range, 3 to 52 months). Of the 5 survivors, 3 had revision of the partial hepatic vein exclusion Fontan to a fenestrated Fontan because of increasing cyanosis associated with development of intrahepatic veno-venous collaterals. None of these patients had major effusions after the revision. The other 2 more recent patients underwent a fenestrated Fontan operation at the time of completion. In contrast, 1 of the 8 patients in group 2 with partial hepatic vein exclusion had subsequent conversion to a fenestrated Fontan. One patient had an aortopulmonary window created for recurrent pericardial effusions.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Since the original description of repair of tricuspid atresia by Fontan and Baudet [7] and the right atrium–right ventricle–pulmonary artery anastomosis of Kreutzer and co-workers [8], changes in surgical strategies have evolved [9][10][11], and application of the Fontan principle has been extended to a wider variety of patients. Physiologic or anatomic features that were thought to place a patient at high risk for a poor outcome after a Fontan operation (such as pulmonary artery distortion, elevated pulmonary artery resistance or pulmonary artery pressure, atrioventricular valve regurgitation, systemic ventricular dysfunction, complex venous anatomy, and subaortic obstruction) have been reassessed [12][13]. Although intuitively it might be expected that a one-lung Fontan would do less well than a two-lung Fontan, there has been little examination of this specific issue. One case report [14] described a patient with double-inlet left ventricle with subpulmonary stenosis, levotransposition of the great arteries, and a severely hypoplastic left pulmonary artery who underwent a Fontan operation at 10 years of age with placement of a Dacron conduit between the right atrium and the right pulmonary artery. Follow-up 9 years postoperatively revealed the patient was doing well clinically and hemodynamically [15].

During the period under review, the Fontan operation was routinely divided into two stages in our practice [16][17]. Performing a hemi-Fontan operation (bidirectional cavopulmonary anastomosis) allows early reduction of the volume work of the single ventricle and remodeling of the ventricular geometry before completion of the Fontan operation. Cardiac output is maintained at the cost of some degree of cyanosis [18][19]. The initial systemic–pulmonary artery shunt of a stage 1 Norwood operation, performed in infancy, is most often a modified right Blalock-Taussig shunt. The subsequent hemi-Fontan procedure usually involves anastomosis of the right superior vena cava to the right pulmonary artery, augmentation of the confluence of the pulmonary arteries, and takedown of the systemic–pulmonary artery shunt. Even with this operation, there exists potential for disproportionately low flow to the left pulmonary artery compared with the right, with a resultant discrepancy in pulmonary artery sizes. In a subset of patients, distortion of the left pulmonary artery is so severe that it results in hypoplasia of that artery and discontinuity from the right pulmonary artery. Thus, after completion of a total cavopulmonary anastomosis, where inferior vena caval blood flow is directed to the right pulmonary artery, all systemic venous return except for flow through excluded hepatic veins (in the case of partial hepatic vein exclusion) or flow crossing the baffle (in the case of a fenestrated baffle) would be directed through the right lung.

Attempts can be made to restore continuity to the pulmonary arteries with homograft augmentation. This was attempted in 3 of the study patients, but on follow-up catheterization, each was found to have discontinuous pulmonary arteries again. Others [20] have proposed using an inverted right atrial flap to create a posterior wall for the nonconfluent pulmonary arteries and a xenopericardial patch for the anterior wall. In some cases, restoring continuity is not feasible.

Before operation, both groups had similar central venous pressures and ventricular end-diastolic pressures, findings suggesting comparable ventricular function. Along with this, it appears that the single right lung in each study patient was able to accommodate pulmonary blood flow as effectively as both lungs in group 2, as the mean values for pulmonary artery pressure, pulmonary blood flow, and pulmonary vascular resistance were not significantly different between groups. Also, the mixing of inferior vena caval systemic blood flow and pulmonary venous blood flow gave comparable aortic saturations in the two groups.

Postoperatively, after the systemic and pulmonary circuits are separated, filling of the single ventricle is dependent on blood flow through the lungs. A diminution of cardiac output because of high transpulmonary gradients might be manifested by tachycardia, decreased urine output, and pleural effusions. These markers of cardiac output were not different between the two groups. Pulmonary venous pressures were consistently monitored and recorded in the initial postoperative period with no significant differences between the two groups. Systemic venous pressures were recorded in only a limited number of patients, but when measured, the values for the two groups were comparable. In addition, the volume load imposed on the single ventricle as a result of systemic collateral blood flow to the left lung did not appear to affect the early postoperative hemodynamic status of these patients.

One difference was that postoperative systemic arterial saturation, measured after extubation and while the patient was receiving 2 L of oxygen through a nasal cannula, revealed a somewhat lower measurement for group 1 (87.0% versus 91.6%). This could be due to increased systemic venous–pulmonary venous shunting, increased ventilation/perfusion mismatch, or a combination of the two. Although the systemic arterial saturation was not as high in group 1, other benefits from a completion Fontan operation are gained. These include decreasing the risk of paradoxical emboli, which could cause a stroke, and preventing the development of pulmonary arteriovenous malformations, which can occur with a long-standing bidirectional cavopulmonary anastomosis and lead to profound desaturation.

A major limitation to this study is the small number of one-lung patients having a Fontan operation. Although it is technically difficult to statistically compare mortality for the two groups, the study demonstrates that completion of the modified Fontan operation can be performed on patients with discontinuous pulmonary arteries with the majority of patients surviving. It is important to note that 5 (71%) of the 7 patients are long-term survivors.

In conclusion, the completion of the staged Fontan operation can be successfully performed in patients with a hypoplastic and discontinuous left pulmonary artery. Preoperative hemodynamics were comparable to those of patients with continuous pulmonary arteries. Postoperative hemodynamics were also similar aside from a lower systemic arterial saturation in group 1. The prevalence of effusions and the mortality rates for the two groups are alike. It is important to note that there were no early postoperative deaths among the one-lung patients and that 5 of the 7 patients survived.

	Acknowledgments

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
We thank Abbas Jawad, PhD, for his help in the statistical analysis of the data and John D. Murphy, MD, who performed the majority of the cardiac catheterizations. We acknowledge the enormous contribution of William I. Norwood, Jr, MD, PhD, to the strategy of staged surgical reconstruction of patients with single-ventricle hearts, including those in this report.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Doctor Jacobs’ current address is Department of Surgery, Deborah Heart and Lung Center, Browns Mills, NJ.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 

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9. De Leval M, Kilner P, Gewillig M, Bull C Total cavopulmonary connection. A logical alternative to atriopulmonary connection for complex Fontan operations. J Thorac Cardiovasc Surg 1988;96:682-695.[Abstract]
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11. Bridges N, Mayer JE, Lock JE, et al. Effect of fenestration on outcome of the modified Fontan repair. Circulation 1992;86:1762-1769.[Abstract/Free Full Text]
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14. Sade RM, Riopel DA, Taylor AB Orthoterminally corrective operation in the presence of severe hypoplasia of a pulmonary artery. J Thorac Cardiovasc Surg 1980;80:424-426.[Abstract]
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17. Jacobs ML, Norwood WI, Jr Fontan operation: influence of modifications on morbidity and mortality. Ann Thorac Surg 1994;58:945-952.[Abstract]
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