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Fontan type operation for complex lesions
J
THoRAc CARDIOVASC SURG
92:1029-1037, 1986
Fontan type operation for complex lesions Surgical considerations to improve survival Twenty-five of 49 patients who underwent a Fontan type operation had complex lesions other than tricuspid atresia with ventriculoarterial concordance. Three patients had significant subaortic stenosis. Thirty-four palliative operations, including nine Glenn shunts, were performed before the Fontan operation. Direct atriopulmonary anastomosis was performed in 21 patients. In four, valved conduits were used, Twelve patients had right atrioventricular valve patch closure (three had running and nine had interrupted suture technique). On the basis of the presence of increased or decreased pulmonary blood flow before any surgical intervention, patients were divided into Group 1 (previoll'> pulmonary artery banding, N = 8) and Group II (pulmonic stenosis, N = 17). Postoperatively, in Group I, 87% had significant effusions, mean right atrial pressure was higher (20.6 ± 6.5 torr), and hospital stay longer (31 days), In Group II, 40% had significant effusions, mean right atrial pressure was lower (16.5 ± 4.3 torr), and hospital stay shorter (15 days). Significant atrioventricular valve patch disruption occurred in three patients (two had running suture technique), and conduit occlll'>ion occurred in two. Four patients (three with subaortic stenosis and pulmonary artery banding) without an established Glenn shunt required Fontan takedown for persistent low cardiac output, two of whom died (2/25 or 8%). There were three late deaths (3/23 or 13 %). Nineteen of 20 surviving patients observed from 2 months to 6 years are doing weD. We believe that early Fontan takedown in patients with persistent low cardiac output, interrupted suture technique for atrioventricular valveclosure, avoidance of valvedconduits, and a preliminary Glenn shunt in patients with pulmonary artery banding and/or subaortic stenosis can further improve the results with the Fontan operation for complex lesions.
Serafin Y. DeLeon, M.D. (by invitation), Michel N. Ilbawi, M.D. (by invitation), Farouk S. Idriss, M.D., Alexander J. Muster, M.D. (by invitation), Samuel S. Gidding, M.D. (by invitation), Teresa E. Berry, M.D. (by invitation), and Milton H. Paul, M.D. (by invitation), Chicago, Ill.
h e role of the Fontan type operation has been clearly established, and the procedure can now be performed with excellent results in tricuspid atresia with ventriculoarterial concordance.'? The mortality and morbidity, however, remain significantly high (12% to 33%) in
From the Divisions of Cardiovascular-Thoracic Surgery and Cardiology, The Children's Memorial Hospital, and the Departments of Surgery and Pediatrics, Northwestern University Medical School, Chicago, Ill. Research supported in part by the A. C. Buehler Foundation, Park Ridge, Ill. Read at the Sixty-sixth Annual Meeting of The American Association for Thoracic Surgery, New York, N. Y., April 28-30,1986. Address for reprints: Serafin Y. Deleon, M.D., Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, Ill. 60614.
tricuspid atresia with ventriculoarterial discordance and other forms of univentricular hearts.!":" To determine whether survival can be further improved in patients with other complex cardiac defects undergoing a Fontan type operation, we reviewed our experience. Patients and methods Twenty-five of 49 patients who underwent a Fontan type operation at our institution in the past 8 years had complex cardiac defects other than tricuspid atresia with ventriculoarterial concordance. Their ages ranged from 2 to 22 years with an average age of 8 years. There were 10 female and 15 male patients. Eleven patients had single ventricle, five had tricuspid atresia with transposition of the great arteries, and nine had various other complex cardiac defects (Table I). 1029
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DeLeon et al.
Table I
Table II Previous operation
No.
Glenn shunt Pulmonary artery banding Subclavian-pulmonary artery shunt Aortopulmonary shunt Atrial septectomy Coarctation Creation of aortopulmonary window for subaortic stenosis
9 8
No. of Diagnosis Single ventricle With two atrioventricular valves Right atrioventricular valve atresia Left atrioventricular valve atresia Tricuspid atresia with transposition of the great arteries Transposition of the great arteries, hypoplastic right ventricle Pulmonary atresia, hypoplastic right ventricle Double-outlet right ventricle, mitral valve atresia, dextroloop Double-outlet right ventricle, hypoplastic left ventricle, dextroloop Common atrioventricular canal, hypoplastic left ventricle (polysplenia) Ebstein's anomaly, hypoplastic right ventricle
patients II 8
2 I
5 3
7
5 3 I I
2 I
Two groups were identified according to whether increased or decreased pulmonary blood flow was present before any surgical palliation. Group I (N = 8) had increased pulmonary blood flow and pulmonary artery banding early in life. Group II (N = 17) had pulmonic stenosis or atresia, with eight patients having previous systemic-pulmonary artery shunts. Three patients in Group I had significant subaortic stenosis (gradients of 35, 80, and 80 torr). Thirty-four palliative operations (Table 11), including nine Glenn shunts, were performed before the Fontan operation. The mean pulmonary artery pressure and pulmonary vascular resistance ranged from 10 to 26 torr (mean 16.7 ± 4.7 torr) and 0.5 to 4 U . m 2 (mean 1.65 ± 0.9 U . m-), respectively. The mean pulmonary vascular resistance was higher, although not statistically significantly higher, in Group I (2.1 ± 1 U· m') than in Group II (1.7 ± 1 U . m-). The ejection fraction of the systemic ventricle ranged from 86% to 105% of normal (mean 96.5% ± 5.5%) and the end-diastolic pressure from 4 to 18 torr (mean 10 ± 3.8 torr). Atriopulmonary connections were established in different fashions. Early in the series, four Dacron valved conduits were used. Thereafter, in 21 patients a direct anastomosis (right atrium to pulmonary artery [N = 18] or right atrium to hypoplastic right ventricle [N = 3]) was performed with the right atrial appendage and complemented anteriorly with either a Dacron or preferably a polytetrafluoroethylene patch (Fig. 1). Seven valves were inserted, five in patients with right atrium-to-pulmonary artery connections and two in patients with right atrium-to-right ventricle connec-
tions. In the past 5 years, no valves have been inserted in patients having a direct right atrium-to-pulmonary artery connection. In 12 patients the right-sided atrioventricular valve was closed with either Dacron or polytetrafluoroethylene patches, leaving the coronary sinus draining to the right atrium. Three patients had running sutures and nine patients had interrupted, pledget-supported sutures similar to and applied in the same manner as for valve insertion (Fig. 2). Atrial partitioning!' resulting in drainage of the coronary sinus to the left atrium was performed in four patients (two with left-sided atrioventricular valve atresia, one with common atrium and right-sided atrioventricular canal, and one with single ventricle and large atrial septal defect). In patients with an established Glenn shunt, cardiopulmonary bypass was accomplished with a single venous cannula placed through the right atrial wall into the inferior vena cava, a ventricular sump, and an arterial cannula in the ascending aorta, which left the Glenn shunt undisturbed. In patients with varying forms of subaortic stenosis, a proximal main pulmonary artery-to-ascending aorta anastomosis was performed to bypass the obstruction.
Results Persistent low cardiac output, necessitating Fontan takedown, developed postoperatively in four patients, two of whom died (early mortality [less than 30 days] 2/25 or 8%). One patient, who was moribund at takedown, died intraoperatively and the second patient, with persistent atelectasis of the left lung because of bronchial stenosis found at autopsy, died 8 hours later. None of the four patients had an established Glenn shunt at the time of the Fontan operation, three had subaortic stenosis and previous pulmonary artery banding, and one had hypoplastic pulmonary arteries despite previous systemic-pulmonary artery shunts. In this group, right atrial hypertension (average 24 torr), hepatomegaly, marked ascites, and decreasing lung
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Fontan operation 1 03 1
Fig. 1. Methods of direct atriopulmonary connections. A, Direct right atrium-to-pulmonary artery anastomosis complemented anteriorly with a patch used in 10 patients with single ventricle. E, A similar direct anastomosis used in five patients with tricuspid atresia with transposition and two patients with transposition and hypoplastic right ventricle. C, A direct right atrium-to-right ventricle anastomosis, also complemented anteriorly with a patch done in three patients with hypoplastic right ventricle with ventriculoarterial concordance. D, A patient with bilateral superior vena cavae and pulmonary atresia had right atrium-to-right pulmonary artery and left superior vena cava-to-Ieft pulmonary artery anastomoses.
compliance occurred after the Fontan operation, with systemic hypotension, persistent metabolic acidosis, and oliguria, despite massive colloid and crystalloid infusions (11,000 ml/m 2/24 hr) and intensive inotropic support. At Fontan takedown, 6 to 65 hours (average 23 hours) postoperatively, the wide open atriopulmonary anastomosis was disconnected and an atrial septal defect reestablished. Three patients were left with a Glenn shunt and an aorta-pulmonary artery shunt to the left lung. In one patient, a Glenn shunt could not be
performed because of bilateral superior venae cavae. The two patients who survived Fontan takedown had Glenn shunts and are well 9 months and 4Y2 years later. There were three late deaths (greater than 30 days, 3/23 or 13%). A 2-year-old patient with a single ventricle had had a Glenn shunt and closure of a severely regurgitant right-sided atrioventricular valve at 9 months. Candida sepsis developed and the child died 3V2 months after the Fontan operation. An 8-year-old
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The Journal of Thoracic and Cardiovascular Surgery
Fig. 2. Methods of closure of right atrioventricular (AV) valves. A, Valves closed in nine patients with a patch secured in place with interrupted sutures with pledgets. B, Continuous sutures used in three patients. C, Right atrial partitioning used in four patients (three had atresia of the left AV valve). RA, Right atrium. LA, Left atrium. RV, Right ventricle. L V. Left ventricle.
patient with double-outlet right ventricle and mitral atresia, who had previous atrial septectomy, pulmonary artery banding, and Glenn shunt, underwent a Fontan operation in which a Dacron valved conduit and atrial partition were used. Complete conduit occlusion occurred 2 months postoperatively and was managed with takedown of the Fontan operation, creation of an atrial septal defect, and aorta-left pulmonary artery shunt. Candida sepsis developed and the patient died 2 months later. A 16-year-old patient with transposition of the great arteries, ventricular septal defect, straddling tricuspid valve, and hypoplastic right ventricle initially had pulmonary artery banding and a Glenn shunt and
finally had the Fontan operation with insertion of a Dacron valved conduit. Six months later, significant tricuspid patch disruption occurred, necessitating surgical reclosure. Conduit occlusion necessitating replacement subsequently developed. Although the new conduit remained patent, the patient continued to have right atrial hypertension because of a markedly reduced pulmonary vascular bed and consequently died of chronic liver failure 5 years after the Fontan operation. The incidence of significant effusions (pleural effusion necessitating chest tube drainage for more than 5 days, pericardial effusion needing drainage, or marked ascites related to low cardiac output necessitating Fon-
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Fanton operation
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Fig. 3. A and E, Angiograms of a patient with single ventricle and pulmonary atresia showing a Glenn shunt with residual patency of the superior vena cava and a distorted left pulmonary artery with sparse branches to the lower lobe.
tan takedown) was compared in the two groups. Significant effusion developed in seven of eight patients (87%) in Group I compared with seven of 17 (40%) in Group II, which correlated with the postoperative mean right atrial pressure (Group I, 20.6 ± 6.5 torr; Group II, 16.5 ± 4.3 torr, p < 0.05). Consequently, the average postoperative hospital stay (excluding patients with infection) was longer in Group I (31 days) than in Group II (15 days). The incidence of significant effusion, the mean right atrial pressure, and the postoperative hospital stay were also compared in patients with an established Glenn shunt at the time of the Fontan operation (4/9 or 44%; 15.4 ± 4.7 torr; 13 days) and in patients without a previous Glenn shunt (10/16 or 62%; 17.8 ± 8 torr; 22 days). The venous assist device described by Heck and Doty" was used in a 9-year-old patient with a single ventricle and pulmonary atresia who initially had a left Blalock-Taussig shunt at 5 days of age. At 6 years of age, the patient had ligation of a persistent left superior vena cava followed by a Glenn shunt that appeared compromised because of residual patency of the right superior vena cava-right atrial junction. The left pulmonary artery was distorted, with sparse branches to the
lower lobe (Fig. 3). The Fontan operation was performed, consisting of atrial partition and a direct right atrium-to-left pulmonary artery anastomosis. Postoperatively, the patient had a mean right atrial pressure of 19 torr, systemic hypotension (82 torr), and acidosis despite inotropic and fluid support. The patient's condition improved after institution of the venous assist device on the seventh postoperative hour and this treatment was continued for 6 days. Additional morbidity included three instances of disruption of the patch used for right-sided atrioventricular valve closure. In addition to the patient already mentioned, two other patients had disruption 5 months and 2 years, respectively, after the Fontan operation. Of the three patients in whom the atrioventricular valve patch closure was performed with running suture, two had significant early patch disruption resulting in reduced left pulmonary blood flow that necessitated reclosure 5 and 6 months postoperatively. Both had a Glenn shunt, which may have been helpful in maintaining effective pulmonary blood flow after patch disruption. In one of nine patients who had interrupted sutures for the right-sided atrioventricular valve patch closure, patch disruption developed and reclosure was performed
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DeLeon et aJ.
2 years after the Fontan operation. Other postoperative morbidity (early and late) included a total of four infections (two Candida, two bacterial). One patient required early mediastinal drainage and another patient required replacement of an infected valve 1V2 years later. Three patients required early implantation of a cardiac pacemaker for complete heart block after tricuspid valve closure (normal conduction eventually resumed in one patient). Two patients required pacemaker implantation 5 months and 2 years postoperatively for control of sick sinus syndrome. Twenty patients (including the two patients who survived the Fontan takedown) have been observed from 2 months to 6 years (average 3 years) after the Fontan operation. Seventeen of the 18 patients with an intact Fontan anastomosis are doing well. One patient, however, has decreased exercise tolerance from myocardial dysfunction (ejection fraction 50% of normal) and high mean right atrial pressure (21 torr). The patient has a permanent ventricular cardiac pacemaker and is receiving antiarrhythmic agents for sick sinus node syndrome. Six patients had elective postoperative cardiac catheterization (average 1.5 years) that revealed right atrial pressures ranging from 12 to 16 torr (average 13 torr) and ejection fractions of the systemic ventricle ranging from 86% to 96% (mean 90%) of normal.
Discussion The Fontan type operation in complex cardiac defects other than tricuspid atresia with ventriculoarterial concordance remains associated with high morbidity and mortality. I, 8-12 A significant number of patients will have acute postoperative right atrial hypertension accompanied by fluid loss into the interstitial space so that adequate cardiac output can be maintained only by infusion of large amounts of colloid and crystalloid fluids. In some patients, right atrial hypertension, development of ascites with elevation of the diaphragm, and decreasing lung compliance create a vicious cycle resulting in persistent low cardiac output, which makes survival unlikely." In addition to significant effusions, renal failure, infections, and third-degree heart block are fairly common postoperative complications. However, most survivors eventually improve and become free of problems inherent with right-to-left shunting. The role of an established Glenn shunt at the time of the Fontan operation as a factor in improving results has been reported by US l6 and others." The incidence of significant effusions and renal failure is lower and the postoperative hospital stay shorter in such patients. Although the Glenn shunt is occasionally complicated by undesirable sequelae":" and there may be reluctance
The Journal of Thoracic and Cardiovascular Surgery
to its use, it remains a useful palliative procedure for many complex cardiac defects. 5, 17, 22-25 Additionally, there is some evidence that the abnormal distribution of pulmonary blood flow characterized by a decreased upper flower lobe perfusion ratio in some patients with the Glenn shunt is also present in some patients after the Fontan operation." The Glenn shunt is also helpful in patients who have significant right-sided atrioventricular valve patch disruption and certainly lifesaving in the event of conduit occlusion." 16,27,28 An argument against a Glenn shunt can be raised, however, that patients with reduced conduit flow may be predisposed to conduit obstruction because of excessive neointima formation. The presence of initially increased pulmonary blood flow followed by pulmonary artery banding seems to increase the risks of the Fontan operation." 29, 30 Such patients had a higher incidence of effusions, higher right atrial pressures, and longer postoperative hospital stay than patients with associated pulmonic stenosis. Significant subaortic stenosis.v" present in three patients in our series, all of whom required pulmonary artery banding, also affected results adversely. These patients had severe persistent low cardiac output postoperatively, which necessitated takedown of the Fontan anastomosis, with two patients surviving and doing well. These two patients were left with a Glenn shunt at takedown, so that another attempt at a Fontan type correction may become feasible in the future. The presence of an established Glenn shunt seems to protect patients with less than ideal hemodynamics undergoing the Fontan operation from severe postoperative hemodynamic instability. None of the nine patients who had an established Glenn shunt at the time of the Fontan operation required takedown, compared to four of 16 patients (25%) who did not. Simultaneous creation of a Glenn shunt and a Fontan operation does not offer the advantage of an established Glenn shunt because the postoperative elevation of systemic venous pressure will affect both systems equally. 16, 33, 34 Disruption of a right-sided atrioventricular valve patch remains a serious complication." 35 Suturing the patch to the valve leaflets or closure of the leaflets alone does not appear to be an effective technique. Continuous suture of the patch to the valve anulus is also accompanied by a high incidence of disruption (67%) in our experience, compared with the interrpted suture technique (11%). A satisfactory alternative in patients requiring right-sided atrioventricular valve closure is atrial partition.v" used successfully in patients with left-sided atrioventricular valve atresia. An atrial septal defect is created or enlarged and a patch is placed connecting the left atrium with the right-sided atrioven-
Volume 92 Number 6 December 1986
tricular valve. Leaving the coronary sinus drainage in the low-pressure left atrium appears to improve myocardial performance." Patch disruption and complete heart block are unlikely with atrial partition, but the capacity of the right atrium will be reduced. The venous assist device as described by Heck and Doty," Laks and colleagues," and others" appears helpful in improving the postoperative cardiac output in some patients. It is possible that the venous assist device, not available in our institution at the time of the Fontan takedown in four patients, could have avoided this drastic procedure. Our single experience with the venous assist device was in a 9-year-old patient with a single ventricle in whom hypotension and acidosis developed after the Fontan operation and improved after institution of the venous assist device. Although the hemodynamic instability was not as severe as that of the other four patients, it is possible that without the venous assist device the patient would have required takedown of the Fontan anastomosis. The majority of patients undergoing the Fontan operation can have a direct right atrium-to-pulmonary artery or right atrium-to-right ventricle connection." 38-41 A valved conduit is unnecessary and, in the absence of a potentially functional right ventricle, valves are not needed. The two eventual deaths from conduit occlusions in our series could probably have been prevented had a direct connection been established between the right atrium and pulmonary artery. The incidence of infection seems high in patients undergoingthe Fontan operation. I, 9, 34, 42 Because postoperative right atrial hypertension, effusions, and liver congestion will develop in the majority of these patients, their resistance may be impaired. Our four patients (4/25, 16%) with postoperative sepsis had significant effusion and hepatomegaly postoperatively. In summary, the Fontan operation can be performed in patients with complex cardiac defects other than tricuspid atresia with ventriculoarterial concordance with reasonable safety even if the suitability criteria outlined by Choussat and colleagues" are not entirely met. An established Glenn shunt appears to reduce postoperative hemodynamic instability and significant effusions and shorten postoperativehospital stay. It may also be protective in patients with right-sided atrioventricular valve patch disruption and conduit occlusion. A preliminary Glenn shunt probably should be considered in patients with subaortic stenosis, pulmonary artery banding, borderline pulmonary vascular resistance, and distorted pulmonary arteries. Direct atriopulmonaryright ventricular connections are feasible in most patients and interrupted suture technique for right-sided
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atrioventricular valve closure makes the occurrence of patch disruption unlikely. Takedown should be considered in patients with persistent low cardiac output after the Fontan operation, although a trial with a venous assist device is indicated. REFERENCES Fontan F, Deville C, Quaegebeur J, Ottenkamp J, Sourdille N, Choussat A, Brom GA: Repair of tricuspid atresia in 100 patients. J THORAC CARDIOVASC SURG 85:647-660, 1983
2 Sanders SP, Wright GB, Keane JF, Norwood WI, Castaneda AR:Clinical and hemodynamic results of the Fontan operation for tricuspid atresia. Am J Cardiol 49:17331740, 1982
3 Cleveland DC, Kirklin JK, Naftel DC, Kirklin JW, Blackstone EH, Pacifico AD, Bargeron LM: Surgical treatment of tricuspid atresia. Ann Thorac Surg 38:447457, 1984
4 Mair DD, Rice MJ, Hagler DJ, Puga FJ, McGoon DC, Danielson GK: Outcome of the Fontan procedure in patients with tricuspid atresia. Circulation 72:1188-1192, 1985
5 DeBrux JL, Zannini L, Binet JP, Neveux JY, Lanlois J, Hazan E, Planche C, Leca F, Marchand M: Tricuspid atresia. Results of treatment in 115 children. J THORAC CARDIOVASC SURG 85:440-446, 1983 6 Laks H, Williams WG, Hellenbrand WE, freedom RM, Talner NS, Rowe RD, Trusler GA: Results of rightatrial to right ventricular and right atrial to pulmonary artery conduits for complex congenital heart disease. Ann Surg 192:382-389, 1980
7 Kreutzer GO, Vargas FJ, Schlichter AJ, LauraJP, Suarez JC, Coronel AR, Kreutzer EA: Atriopulmonary anastomosis. J THORAC CARDIOVASC SURG 83:427-436, 1982 8 Laks H, Milliken JC, Perloff JK, Hellenbrand WE, George BL, Chin A, DiSessa TG, Williams RG: Experience with the Fontan procedure. J THORAC CARDIOVASC SURG 88:939-951, 1984 9 Gale AW, Danielson GK, McGoon DC, Mair DD: Modified Fontan operation for univentricular heart and complicated congenital lesions. J THORAC CARDIOVASC SURG 78:831-838,1979 10 Helgason H, Mayer JE, Sanders SP, Lang P, Jonas RA, Castaneda AR: Fontan operation for complex congenital heart defects other than tricuspid atresia. (abstr). Am Heart J 110:705, 1985
II Marcelletti C, Mazzera E, Olthof H, Sebel PS, Duren DR, Losekoot TG, Becker AE: Fontan's operation. An expanded horizon. J THORAC CARDIOVASC SURG 80:764769, 1980 12 Danielson GK: Discussion of Fontan et all 13 Lamberti JJ, Thilenius 0, de la Fuente D, LinCY, Arcilla
R, Replogle RL: Right atrial partition and rightventricular exclusion. Another surgical approach for complex
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cyanotic congenital heart disease. J THoRAc CARDIOVASC SURG 71:386-391,1976 14 Heck HA, Doty DB: Assisted circulation by phasic external lower body compression. Circulation 64:Suppl 2:118-122, 1981 15 DeLeon SY, IIbawi MN, Idriss FS, Muster AJ, Gidding SS, Berry TE, Paul MH: Persistent low cardiac output after the Fontan operation. Should takedown be considered. J THoRAc CARDIOVASC SURG 92:402-405, 1986 16 DeLeon SY, Idriss FA, IIbawi MN, Muster AJ, Paul MH, Cole RB, Riggs TW, Berry TE: The role of the Glenn shunt in patients undergoing the Fontan operation. J THORAC CARDIOVASC SURG 85:669-677, 1983 17 Pennington DG, Nouri S, Ho J, Seeker-Walker R, Patel B, Sivakoff M, Willman VL: Glenn shunt. Long-term results and current role in congenital heart operation. Ann Thorac Surg 31:532-539, 1981 18 Bargeron LM, Karp RB, Barcia A, Kirklin JW, Hunt D, Deverall PB: Late deterioration of patients after superior vena cava to right pulmonary artery anastomosis. Am J Cardiol 30:211-216, 1972 19 Boruchow IB, Swenson EW, Elliot LP, Bartley TD, Wheat MW, Schiebler GL: Study of the mechanisms of shunt failure after superior vena cava-right pulmonary artery stenosis. J THORAC CARDIOVASC SURG 60:531-539, 1970 20 McFaul RC, Tajik AJ, Mair DD, Danielson GK, Seward JB: Development of pulmonary arteriovenous shunt after superior vena cava-right pulmonary artery (Glenn) anastomosis. Report of four cases. Circulation 55:212-216, 1977 21 Samanek M, Oppelt A, Kasalicky J, Voriskova M: Distribution of pulmonary blood flow after cavopulmonary anastomosis (Glenn operation). Br Heart J 31:511-516, 1969 22 Trusler GA, Williams WG: Long term results of shunt procedures for tricuspid atresia. Ann Thorac Surg 29:312316, 1980 23 Willman VL, Barner HB, Mudd JG, Fagan LF, Kaiser GC: Superior vena cava-pulmonary artery anastomosis. J THoRAc CARDIOVASC SURG 67:380-386, 1974 24 Laks H, Mudd JG, Standeven JW, Fagan L, Willman VL: Long-term effect of the superior vena cava-pulmonary artery anastomosis on pulmonary blood flow. J THoRAc CARDIOVASC SURG 74:253-260, 1977 25 DiCarlo D, Williams WG, Freedom RM, Trusler GA, Rowe RD: The role of cava-pulmonary (Glenn) anastomosis in the palliative treatment of congenital heart disease. J THoRAc CARDIOVASC SURG 83:437-442, 1982 26 Cloutier A, Ash JM, Smallhorn JF, Williams WG, Trusler GA, Rowe RD: Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure. Risk of development of arteriovenous fistulae. Circulation 72:471-479, 1985 27 DeLeon SY, Koopot R, Mair DD, Idriss FS, IIbawi MN, Muster AJ, Paul MH: Surgical management of occluded
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conduits after the Fontan operation in patients with Glenn shunts. J THoRAc CARDIOVASC SURG 88:601-605, 1984 Mair DD, Fulton RE, Danielson GK: Thrombotic occlusion of Hancock conduit due to severe dehydration after Fontan operation. Mayo Clin Proc 53:397-402, 1978 Juaneda E, Haworth SG: Double inlet ventricle. Lung biopsy findings and implications for management. Br Heart J 53:515-519,1985 Juaneda E, Haworth SG: Pulmonary vascular structure in patients dying after a Fontan procedure. The lung as a risk factor. Br Heart J 52:575-580, 1984 Penkoske PA, Freedom RM, Williams WG, Trusler GA, Rowe RD: Surgical palliation of subaortic stenosis in the univentricular heart. J THORAC CARDIOVASC SURG 87:767781, 1984 Barber G, Hagler DJ, Edwards WD, Puga FJ, Danielson G K, McGoon DC, Driscoll DJ: Surgical repair of univentricular heart (double inlet left ventricle) with obstructed anterior subaortic outlet chamber. J Am Coil Cardiol 4:771-778, 1984 Tatooles CJ, Ardekani RG, Miller RA, Seratto M: Operative repair for tricuspid atresia. Ann Thorac Surg 21:499-503, 1976 Hopkins RA, Armstrong BE, Serwer GA, Peterson RJ, Oldham HN: Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle. J THoRAc CARDJOVASC SURG 90:391398, 1985 diDonato R, Becker AE, Nijveld A, Lam J, Bulterijs A, Squitieri C, Marcelletti C: Ventricular exclusion during Fontan operation. An evolving technique. Ann Thorac Surg 39:283-285, 1985 I1bawi MN, Idriss FS, Muster AJ, DeLeon SY, Berry TE, Duffy CE, Paul MH: Effects of elevated coronary sinus pressure on left ventricular function after the Fontan operation. An experimental and clinical correlation. J THORAC CARDIOVASC SURG 92:231-237, 1986 Guyton RA, Davis SC, Michalik RE, Williams WH, Hatcher CP: Right heart assist by intermittent abdominal compression after surgery for congenital heart disease. Circulation 72:Suppl 2:97-100, 1985 Molina JE, Wang Y, Lucas R, Moller J: The technique of the Fontan procedure with posterior right atrium-pulmonary artery connection. Ann Thorac Surg 39:371-375, 1985 Oelert H, Borst HG: Modified Fontan procedure using a retroaortic atriopulmonary anastomosis. Thorac Cardiovase Surg 32:392-394, 1984 Doty DB, Marvin WJ, Lauer RM: Modified Fontan procedure. Methods to achieve direct anastomosis of right atrium to pulmonary artery. J THORAC CARDIOVASC SURG 81:470-475,1981 Bjork YO, Olin CL, Bjarke BB, Thoren CA: Right atrial-right ventricular anastomosis for correlation of tricuspid atresia. J THoRAc CARDIOVASC SURG 77:452458, 1979
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42 Ottenkamp J, Pohmer J, Quaegebeur JM, Brom AG, Fontan F: Nine years' experience of physiological correction of tricuspid atresia. Long term results and current surgical approach. Thorax 37:718-726, 1982 43 Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H: Selection criteria for Fontan's procedure,
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Pediatric Cardiology, RH Anderson, EA Shinebourne, eds., Edinburgh, 1978, Churchill Livingstone, pp 559566 (For Discussion see page 1044.)
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Fontan type operation for complex lesions Surgical considerations to improve survival Twenty-five of 49 patients who underwent a Fontan type operation had complex lesions other than tricuspid atresia with ventriculoarterial concordance. Three patients had significant subaortic stenosis. Thirty-four palliative operations, including nine Glenn shunts, were performed before the Fontan operation. Direct atriopulmonary anastomosis was performed in 21 patients. In four, valved conduits were used, Twelve patients had right atrioventricular valve patch closure (three had running and nine had interrupted suture technique). On the basis of the presence of increased or decreased pulmonary blood flow before any surgical intervention, patients were divided into Group 1 (previoll'> pulmonary artery banding, N = 8) and Group II (pulmonic stenosis, N = 17). Postoperatively, in Group I, 87% had significant effusions, mean right atrial pressure was higher (20.6 ± 6.5 torr), and hospital stay longer (31 days), In Group II, 40% had significant effusions, mean right atrial pressure was lower (16.5 ± 4.3 torr), and hospital stay shorter (15 days). Significant atrioventricular valve patch disruption occurred in three patients (two had running suture technique), and conduit occlll'>ion occurred in two. Four patients (three with subaortic stenosis and pulmonary artery banding) without an established Glenn shunt required Fontan takedown for persistent low cardiac output, two of whom died (2/25 or 8%). There were three late deaths (3/23 or 13 %). Nineteen of 20 surviving patients observed from 2 months to 6 years are doing weD. We believe that early Fontan takedown in patients with persistent low cardiac output, interrupted suture technique for atrioventricular valveclosure, avoidance of valvedconduits, and a preliminary Glenn shunt in patients with pulmonary artery banding and/or subaortic stenosis can further improve the results with the Fontan operation for complex lesions.
Serafin Y. DeLeon, M.D. (by invitation), Michel N. Ilbawi, M.D. (by invitation), Farouk S. Idriss, M.D., Alexander J. Muster, M.D. (by invitation), Samuel S. Gidding, M.D. (by invitation), Teresa E. Berry, M.D. (by invitation), and Milton H. Paul, M.D. (by invitation), Chicago, Ill.
h e role of the Fontan type operation has been clearly established, and the procedure can now be performed with excellent results in tricuspid atresia with ventriculoarterial concordance.'? The mortality and morbidity, however, remain significantly high (12% to 33%) in
From the Divisions of Cardiovascular-Thoracic Surgery and Cardiology, The Children's Memorial Hospital, and the Departments of Surgery and Pediatrics, Northwestern University Medical School, Chicago, Ill. Research supported in part by the A. C. Buehler Foundation, Park Ridge, Ill. Read at the Sixty-sixth Annual Meeting of The American Association for Thoracic Surgery, New York, N. Y., April 28-30,1986. Address for reprints: Serafin Y. Deleon, M.D., Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, Ill. 60614.
tricuspid atresia with ventriculoarterial discordance and other forms of univentricular hearts.!":" To determine whether survival can be further improved in patients with other complex cardiac defects undergoing a Fontan type operation, we reviewed our experience. Patients and methods Twenty-five of 49 patients who underwent a Fontan type operation at our institution in the past 8 years had complex cardiac defects other than tricuspid atresia with ventriculoarterial concordance. Their ages ranged from 2 to 22 years with an average age of 8 years. There were 10 female and 15 male patients. Eleven patients had single ventricle, five had tricuspid atresia with transposition of the great arteries, and nine had various other complex cardiac defects (Table I). 1029
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Table I
Table II Previous operation
No.
Glenn shunt Pulmonary artery banding Subclavian-pulmonary artery shunt Aortopulmonary shunt Atrial septectomy Coarctation Creation of aortopulmonary window for subaortic stenosis
9 8
No. of Diagnosis Single ventricle With two atrioventricular valves Right atrioventricular valve atresia Left atrioventricular valve atresia Tricuspid atresia with transposition of the great arteries Transposition of the great arteries, hypoplastic right ventricle Pulmonary atresia, hypoplastic right ventricle Double-outlet right ventricle, mitral valve atresia, dextroloop Double-outlet right ventricle, hypoplastic left ventricle, dextroloop Common atrioventricular canal, hypoplastic left ventricle (polysplenia) Ebstein's anomaly, hypoplastic right ventricle
patients II 8
2 I
5 3
7
5 3 I I
2 I
Two groups were identified according to whether increased or decreased pulmonary blood flow was present before any surgical palliation. Group I (N = 8) had increased pulmonary blood flow and pulmonary artery banding early in life. Group II (N = 17) had pulmonic stenosis or atresia, with eight patients having previous systemic-pulmonary artery shunts. Three patients in Group I had significant subaortic stenosis (gradients of 35, 80, and 80 torr). Thirty-four palliative operations (Table 11), including nine Glenn shunts, were performed before the Fontan operation. The mean pulmonary artery pressure and pulmonary vascular resistance ranged from 10 to 26 torr (mean 16.7 ± 4.7 torr) and 0.5 to 4 U . m 2 (mean 1.65 ± 0.9 U . m-), respectively. The mean pulmonary vascular resistance was higher, although not statistically significantly higher, in Group I (2.1 ± 1 U· m') than in Group II (1.7 ± 1 U . m-). The ejection fraction of the systemic ventricle ranged from 86% to 105% of normal (mean 96.5% ± 5.5%) and the end-diastolic pressure from 4 to 18 torr (mean 10 ± 3.8 torr). Atriopulmonary connections were established in different fashions. Early in the series, four Dacron valved conduits were used. Thereafter, in 21 patients a direct anastomosis (right atrium to pulmonary artery [N = 18] or right atrium to hypoplastic right ventricle [N = 3]) was performed with the right atrial appendage and complemented anteriorly with either a Dacron or preferably a polytetrafluoroethylene patch (Fig. 1). Seven valves were inserted, five in patients with right atrium-to-pulmonary artery connections and two in patients with right atrium-to-right ventricle connec-
tions. In the past 5 years, no valves have been inserted in patients having a direct right atrium-to-pulmonary artery connection. In 12 patients the right-sided atrioventricular valve was closed with either Dacron or polytetrafluoroethylene patches, leaving the coronary sinus draining to the right atrium. Three patients had running sutures and nine patients had interrupted, pledget-supported sutures similar to and applied in the same manner as for valve insertion (Fig. 2). Atrial partitioning!' resulting in drainage of the coronary sinus to the left atrium was performed in four patients (two with left-sided atrioventricular valve atresia, one with common atrium and right-sided atrioventricular canal, and one with single ventricle and large atrial septal defect). In patients with an established Glenn shunt, cardiopulmonary bypass was accomplished with a single venous cannula placed through the right atrial wall into the inferior vena cava, a ventricular sump, and an arterial cannula in the ascending aorta, which left the Glenn shunt undisturbed. In patients with varying forms of subaortic stenosis, a proximal main pulmonary artery-to-ascending aorta anastomosis was performed to bypass the obstruction.
Results Persistent low cardiac output, necessitating Fontan takedown, developed postoperatively in four patients, two of whom died (early mortality [less than 30 days] 2/25 or 8%). One patient, who was moribund at takedown, died intraoperatively and the second patient, with persistent atelectasis of the left lung because of bronchial stenosis found at autopsy, died 8 hours later. None of the four patients had an established Glenn shunt at the time of the Fontan operation, three had subaortic stenosis and previous pulmonary artery banding, and one had hypoplastic pulmonary arteries despite previous systemic-pulmonary artery shunts. In this group, right atrial hypertension (average 24 torr), hepatomegaly, marked ascites, and decreasing lung
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Fontan operation 1 03 1
Fig. 1. Methods of direct atriopulmonary connections. A, Direct right atrium-to-pulmonary artery anastomosis complemented anteriorly with a patch used in 10 patients with single ventricle. E, A similar direct anastomosis used in five patients with tricuspid atresia with transposition and two patients with transposition and hypoplastic right ventricle. C, A direct right atrium-to-right ventricle anastomosis, also complemented anteriorly with a patch done in three patients with hypoplastic right ventricle with ventriculoarterial concordance. D, A patient with bilateral superior vena cavae and pulmonary atresia had right atrium-to-right pulmonary artery and left superior vena cava-to-Ieft pulmonary artery anastomoses.
compliance occurred after the Fontan operation, with systemic hypotension, persistent metabolic acidosis, and oliguria, despite massive colloid and crystalloid infusions (11,000 ml/m 2/24 hr) and intensive inotropic support. At Fontan takedown, 6 to 65 hours (average 23 hours) postoperatively, the wide open atriopulmonary anastomosis was disconnected and an atrial septal defect reestablished. Three patients were left with a Glenn shunt and an aorta-pulmonary artery shunt to the left lung. In one patient, a Glenn shunt could not be
performed because of bilateral superior venae cavae. The two patients who survived Fontan takedown had Glenn shunts and are well 9 months and 4Y2 years later. There were three late deaths (greater than 30 days, 3/23 or 13%). A 2-year-old patient with a single ventricle had had a Glenn shunt and closure of a severely regurgitant right-sided atrioventricular valve at 9 months. Candida sepsis developed and the child died 3V2 months after the Fontan operation. An 8-year-old
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Fig. 2. Methods of closure of right atrioventricular (AV) valves. A, Valves closed in nine patients with a patch secured in place with interrupted sutures with pledgets. B, Continuous sutures used in three patients. C, Right atrial partitioning used in four patients (three had atresia of the left AV valve). RA, Right atrium. LA, Left atrium. RV, Right ventricle. L V. Left ventricle.
patient with double-outlet right ventricle and mitral atresia, who had previous atrial septectomy, pulmonary artery banding, and Glenn shunt, underwent a Fontan operation in which a Dacron valved conduit and atrial partition were used. Complete conduit occlusion occurred 2 months postoperatively and was managed with takedown of the Fontan operation, creation of an atrial septal defect, and aorta-left pulmonary artery shunt. Candida sepsis developed and the patient died 2 months later. A 16-year-old patient with transposition of the great arteries, ventricular septal defect, straddling tricuspid valve, and hypoplastic right ventricle initially had pulmonary artery banding and a Glenn shunt and
finally had the Fontan operation with insertion of a Dacron valved conduit. Six months later, significant tricuspid patch disruption occurred, necessitating surgical reclosure. Conduit occlusion necessitating replacement subsequently developed. Although the new conduit remained patent, the patient continued to have right atrial hypertension because of a markedly reduced pulmonary vascular bed and consequently died of chronic liver failure 5 years after the Fontan operation. The incidence of significant effusions (pleural effusion necessitating chest tube drainage for more than 5 days, pericardial effusion needing drainage, or marked ascites related to low cardiac output necessitating Fon-
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Fanton operation
1 03 3
Fig. 3. A and E, Angiograms of a patient with single ventricle and pulmonary atresia showing a Glenn shunt with residual patency of the superior vena cava and a distorted left pulmonary artery with sparse branches to the lower lobe.
tan takedown) was compared in the two groups. Significant effusion developed in seven of eight patients (87%) in Group I compared with seven of 17 (40%) in Group II, which correlated with the postoperative mean right atrial pressure (Group I, 20.6 ± 6.5 torr; Group II, 16.5 ± 4.3 torr, p < 0.05). Consequently, the average postoperative hospital stay (excluding patients with infection) was longer in Group I (31 days) than in Group II (15 days). The incidence of significant effusion, the mean right atrial pressure, and the postoperative hospital stay were also compared in patients with an established Glenn shunt at the time of the Fontan operation (4/9 or 44%; 15.4 ± 4.7 torr; 13 days) and in patients without a previous Glenn shunt (10/16 or 62%; 17.8 ± 8 torr; 22 days). The venous assist device described by Heck and Doty" was used in a 9-year-old patient with a single ventricle and pulmonary atresia who initially had a left Blalock-Taussig shunt at 5 days of age. At 6 years of age, the patient had ligation of a persistent left superior vena cava followed by a Glenn shunt that appeared compromised because of residual patency of the right superior vena cava-right atrial junction. The left pulmonary artery was distorted, with sparse branches to the
lower lobe (Fig. 3). The Fontan operation was performed, consisting of atrial partition and a direct right atrium-to-left pulmonary artery anastomosis. Postoperatively, the patient had a mean right atrial pressure of 19 torr, systemic hypotension (82 torr), and acidosis despite inotropic and fluid support. The patient's condition improved after institution of the venous assist device on the seventh postoperative hour and this treatment was continued for 6 days. Additional morbidity included three instances of disruption of the patch used for right-sided atrioventricular valve closure. In addition to the patient already mentioned, two other patients had disruption 5 months and 2 years, respectively, after the Fontan operation. Of the three patients in whom the atrioventricular valve patch closure was performed with running suture, two had significant early patch disruption resulting in reduced left pulmonary blood flow that necessitated reclosure 5 and 6 months postoperatively. Both had a Glenn shunt, which may have been helpful in maintaining effective pulmonary blood flow after patch disruption. In one of nine patients who had interrupted sutures for the right-sided atrioventricular valve patch closure, patch disruption developed and reclosure was performed
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DeLeon et aJ.
2 years after the Fontan operation. Other postoperative morbidity (early and late) included a total of four infections (two Candida, two bacterial). One patient required early mediastinal drainage and another patient required replacement of an infected valve 1V2 years later. Three patients required early implantation of a cardiac pacemaker for complete heart block after tricuspid valve closure (normal conduction eventually resumed in one patient). Two patients required pacemaker implantation 5 months and 2 years postoperatively for control of sick sinus syndrome. Twenty patients (including the two patients who survived the Fontan takedown) have been observed from 2 months to 6 years (average 3 years) after the Fontan operation. Seventeen of the 18 patients with an intact Fontan anastomosis are doing well. One patient, however, has decreased exercise tolerance from myocardial dysfunction (ejection fraction 50% of normal) and high mean right atrial pressure (21 torr). The patient has a permanent ventricular cardiac pacemaker and is receiving antiarrhythmic agents for sick sinus node syndrome. Six patients had elective postoperative cardiac catheterization (average 1.5 years) that revealed right atrial pressures ranging from 12 to 16 torr (average 13 torr) and ejection fractions of the systemic ventricle ranging from 86% to 96% (mean 90%) of normal.
Discussion The Fontan type operation in complex cardiac defects other than tricuspid atresia with ventriculoarterial concordance remains associated with high morbidity and mortality. I, 8-12 A significant number of patients will have acute postoperative right atrial hypertension accompanied by fluid loss into the interstitial space so that adequate cardiac output can be maintained only by infusion of large amounts of colloid and crystalloid fluids. In some patients, right atrial hypertension, development of ascites with elevation of the diaphragm, and decreasing lung compliance create a vicious cycle resulting in persistent low cardiac output, which makes survival unlikely." In addition to significant effusions, renal failure, infections, and third-degree heart block are fairly common postoperative complications. However, most survivors eventually improve and become free of problems inherent with right-to-left shunting. The role of an established Glenn shunt at the time of the Fontan operation as a factor in improving results has been reported by US l6 and others." The incidence of significant effusions and renal failure is lower and the postoperative hospital stay shorter in such patients. Although the Glenn shunt is occasionally complicated by undesirable sequelae":" and there may be reluctance
The Journal of Thoracic and Cardiovascular Surgery
to its use, it remains a useful palliative procedure for many complex cardiac defects. 5, 17, 22-25 Additionally, there is some evidence that the abnormal distribution of pulmonary blood flow characterized by a decreased upper flower lobe perfusion ratio in some patients with the Glenn shunt is also present in some patients after the Fontan operation." The Glenn shunt is also helpful in patients who have significant right-sided atrioventricular valve patch disruption and certainly lifesaving in the event of conduit occlusion." 16,27,28 An argument against a Glenn shunt can be raised, however, that patients with reduced conduit flow may be predisposed to conduit obstruction because of excessive neointima formation. The presence of initially increased pulmonary blood flow followed by pulmonary artery banding seems to increase the risks of the Fontan operation." 29, 30 Such patients had a higher incidence of effusions, higher right atrial pressures, and longer postoperative hospital stay than patients with associated pulmonic stenosis. Significant subaortic stenosis.v" present in three patients in our series, all of whom required pulmonary artery banding, also affected results adversely. These patients had severe persistent low cardiac output postoperatively, which necessitated takedown of the Fontan anastomosis, with two patients surviving and doing well. These two patients were left with a Glenn shunt at takedown, so that another attempt at a Fontan type correction may become feasible in the future. The presence of an established Glenn shunt seems to protect patients with less than ideal hemodynamics undergoing the Fontan operation from severe postoperative hemodynamic instability. None of the nine patients who had an established Glenn shunt at the time of the Fontan operation required takedown, compared to four of 16 patients (25%) who did not. Simultaneous creation of a Glenn shunt and a Fontan operation does not offer the advantage of an established Glenn shunt because the postoperative elevation of systemic venous pressure will affect both systems equally. 16, 33, 34 Disruption of a right-sided atrioventricular valve patch remains a serious complication." 35 Suturing the patch to the valve leaflets or closure of the leaflets alone does not appear to be an effective technique. Continuous suture of the patch to the valve anulus is also accompanied by a high incidence of disruption (67%) in our experience, compared with the interrpted suture technique (11%). A satisfactory alternative in patients requiring right-sided atrioventricular valve closure is atrial partition.v" used successfully in patients with left-sided atrioventricular valve atresia. An atrial septal defect is created or enlarged and a patch is placed connecting the left atrium with the right-sided atrioven-
Volume 92 Number 6 December 1986
tricular valve. Leaving the coronary sinus drainage in the low-pressure left atrium appears to improve myocardial performance." Patch disruption and complete heart block are unlikely with atrial partition, but the capacity of the right atrium will be reduced. The venous assist device as described by Heck and Doty," Laks and colleagues," and others" appears helpful in improving the postoperative cardiac output in some patients. It is possible that the venous assist device, not available in our institution at the time of the Fontan takedown in four patients, could have avoided this drastic procedure. Our single experience with the venous assist device was in a 9-year-old patient with a single ventricle in whom hypotension and acidosis developed after the Fontan operation and improved after institution of the venous assist device. Although the hemodynamic instability was not as severe as that of the other four patients, it is possible that without the venous assist device the patient would have required takedown of the Fontan anastomosis. The majority of patients undergoing the Fontan operation can have a direct right atrium-to-pulmonary artery or right atrium-to-right ventricle connection." 38-41 A valved conduit is unnecessary and, in the absence of a potentially functional right ventricle, valves are not needed. The two eventual deaths from conduit occlusions in our series could probably have been prevented had a direct connection been established between the right atrium and pulmonary artery. The incidence of infection seems high in patients undergoingthe Fontan operation. I, 9, 34, 42 Because postoperative right atrial hypertension, effusions, and liver congestion will develop in the majority of these patients, their resistance may be impaired. Our four patients (4/25, 16%) with postoperative sepsis had significant effusion and hepatomegaly postoperatively. In summary, the Fontan operation can be performed in patients with complex cardiac defects other than tricuspid atresia with ventriculoarterial concordance with reasonable safety even if the suitability criteria outlined by Choussat and colleagues" are not entirely met. An established Glenn shunt appears to reduce postoperative hemodynamic instability and significant effusions and shorten postoperativehospital stay. It may also be protective in patients with right-sided atrioventricular valve patch disruption and conduit occlusion. A preliminary Glenn shunt probably should be considered in patients with subaortic stenosis, pulmonary artery banding, borderline pulmonary vascular resistance, and distorted pulmonary arteries. Direct atriopulmonaryright ventricular connections are feasible in most patients and interrupted suture technique for right-sided
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atrioventricular valve closure makes the occurrence of patch disruption unlikely. Takedown should be considered in patients with persistent low cardiac output after the Fontan operation, although a trial with a venous assist device is indicated. REFERENCES Fontan F, Deville C, Quaegebeur J, Ottenkamp J, Sourdille N, Choussat A, Brom GA: Repair of tricuspid atresia in 100 patients. J THORAC CARDIOVASC SURG 85:647-660, 1983
2 Sanders SP, Wright GB, Keane JF, Norwood WI, Castaneda AR:Clinical and hemodynamic results of the Fontan operation for tricuspid atresia. Am J Cardiol 49:17331740, 1982
3 Cleveland DC, Kirklin JK, Naftel DC, Kirklin JW, Blackstone EH, Pacifico AD, Bargeron LM: Surgical treatment of tricuspid atresia. Ann Thorac Surg 38:447457, 1984
4 Mair DD, Rice MJ, Hagler DJ, Puga FJ, McGoon DC, Danielson GK: Outcome of the Fontan procedure in patients with tricuspid atresia. Circulation 72:1188-1192, 1985
5 DeBrux JL, Zannini L, Binet JP, Neveux JY, Lanlois J, Hazan E, Planche C, Leca F, Marchand M: Tricuspid atresia. Results of treatment in 115 children. J THORAC CARDIOVASC SURG 85:440-446, 1983 6 Laks H, Williams WG, Hellenbrand WE, freedom RM, Talner NS, Rowe RD, Trusler GA: Results of rightatrial to right ventricular and right atrial to pulmonary artery conduits for complex congenital heart disease. Ann Surg 192:382-389, 1980
7 Kreutzer GO, Vargas FJ, Schlichter AJ, LauraJP, Suarez JC, Coronel AR, Kreutzer EA: Atriopulmonary anastomosis. J THORAC CARDIOVASC SURG 83:427-436, 1982 8 Laks H, Milliken JC, Perloff JK, Hellenbrand WE, George BL, Chin A, DiSessa TG, Williams RG: Experience with the Fontan procedure. J THORAC CARDIOVASC SURG 88:939-951, 1984 9 Gale AW, Danielson GK, McGoon DC, Mair DD: Modified Fontan operation for univentricular heart and complicated congenital lesions. J THORAC CARDIOVASC SURG 78:831-838,1979 10 Helgason H, Mayer JE, Sanders SP, Lang P, Jonas RA, Castaneda AR: Fontan operation for complex congenital heart defects other than tricuspid atresia. (abstr). Am Heart J 110:705, 1985
II Marcelletti C, Mazzera E, Olthof H, Sebel PS, Duren DR, Losekoot TG, Becker AE: Fontan's operation. An expanded horizon. J THORAC CARDIOVASC SURG 80:764769, 1980 12 Danielson GK: Discussion of Fontan et all 13 Lamberti JJ, Thilenius 0, de la Fuente D, LinCY, Arcilla
R, Replogle RL: Right atrial partition and rightventricular exclusion. Another surgical approach for complex
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cyanotic congenital heart disease. J THoRAc CARDIOVASC SURG 71:386-391,1976 14 Heck HA, Doty DB: Assisted circulation by phasic external lower body compression. Circulation 64:Suppl 2:118-122, 1981 15 DeLeon SY, IIbawi MN, Idriss FS, Muster AJ, Gidding SS, Berry TE, Paul MH: Persistent low cardiac output after the Fontan operation. Should takedown be considered. J THoRAc CARDIOVASC SURG 92:402-405, 1986 16 DeLeon SY, Idriss FA, IIbawi MN, Muster AJ, Paul MH, Cole RB, Riggs TW, Berry TE: The role of the Glenn shunt in patients undergoing the Fontan operation. J THORAC CARDIOVASC SURG 85:669-677, 1983 17 Pennington DG, Nouri S, Ho J, Seeker-Walker R, Patel B, Sivakoff M, Willman VL: Glenn shunt. Long-term results and current role in congenital heart operation. Ann Thorac Surg 31:532-539, 1981 18 Bargeron LM, Karp RB, Barcia A, Kirklin JW, Hunt D, Deverall PB: Late deterioration of patients after superior vena cava to right pulmonary artery anastomosis. Am J Cardiol 30:211-216, 1972 19 Boruchow IB, Swenson EW, Elliot LP, Bartley TD, Wheat MW, Schiebler GL: Study of the mechanisms of shunt failure after superior vena cava-right pulmonary artery stenosis. J THORAC CARDIOVASC SURG 60:531-539, 1970 20 McFaul RC, Tajik AJ, Mair DD, Danielson GK, Seward JB: Development of pulmonary arteriovenous shunt after superior vena cava-right pulmonary artery (Glenn) anastomosis. Report of four cases. Circulation 55:212-216, 1977 21 Samanek M, Oppelt A, Kasalicky J, Voriskova M: Distribution of pulmonary blood flow after cavopulmonary anastomosis (Glenn operation). Br Heart J 31:511-516, 1969 22 Trusler GA, Williams WG: Long term results of shunt procedures for tricuspid atresia. Ann Thorac Surg 29:312316, 1980 23 Willman VL, Barner HB, Mudd JG, Fagan LF, Kaiser GC: Superior vena cava-pulmonary artery anastomosis. J THoRAc CARDIOVASC SURG 67:380-386, 1974 24 Laks H, Mudd JG, Standeven JW, Fagan L, Willman VL: Long-term effect of the superior vena cava-pulmonary artery anastomosis on pulmonary blood flow. J THoRAc CARDIOVASC SURG 74:253-260, 1977 25 DiCarlo D, Williams WG, Freedom RM, Trusler GA, Rowe RD: The role of cava-pulmonary (Glenn) anastomosis in the palliative treatment of congenital heart disease. J THoRAc CARDIOVASC SURG 83:437-442, 1982 26 Cloutier A, Ash JM, Smallhorn JF, Williams WG, Trusler GA, Rowe RD: Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure. Risk of development of arteriovenous fistulae. Circulation 72:471-479, 1985 27 DeLeon SY, Koopot R, Mair DD, Idriss FS, IIbawi MN, Muster AJ, Paul MH: Surgical management of occluded
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conduits after the Fontan operation in patients with Glenn shunts. J THoRAc CARDIOVASC SURG 88:601-605, 1984 Mair DD, Fulton RE, Danielson GK: Thrombotic occlusion of Hancock conduit due to severe dehydration after Fontan operation. Mayo Clin Proc 53:397-402, 1978 Juaneda E, Haworth SG: Double inlet ventricle. Lung biopsy findings and implications for management. Br Heart J 53:515-519,1985 Juaneda E, Haworth SG: Pulmonary vascular structure in patients dying after a Fontan procedure. The lung as a risk factor. Br Heart J 52:575-580, 1984 Penkoske PA, Freedom RM, Williams WG, Trusler GA, Rowe RD: Surgical palliation of subaortic stenosis in the univentricular heart. J THORAC CARDIOVASC SURG 87:767781, 1984 Barber G, Hagler DJ, Edwards WD, Puga FJ, Danielson G K, McGoon DC, Driscoll DJ: Surgical repair of univentricular heart (double inlet left ventricle) with obstructed anterior subaortic outlet chamber. J Am Coil Cardiol 4:771-778, 1984 Tatooles CJ, Ardekani RG, Miller RA, Seratto M: Operative repair for tricuspid atresia. Ann Thorac Surg 21:499-503, 1976 Hopkins RA, Armstrong BE, Serwer GA, Peterson RJ, Oldham HN: Physiological rationale for a bidirectional cavopulmonary shunt. A versatile complement to the Fontan principle. J THoRAc CARDJOVASC SURG 90:391398, 1985 diDonato R, Becker AE, Nijveld A, Lam J, Bulterijs A, Squitieri C, Marcelletti C: Ventricular exclusion during Fontan operation. An evolving technique. Ann Thorac Surg 39:283-285, 1985 I1bawi MN, Idriss FS, Muster AJ, DeLeon SY, Berry TE, Duffy CE, Paul MH: Effects of elevated coronary sinus pressure on left ventricular function after the Fontan operation. An experimental and clinical correlation. J THORAC CARDIOVASC SURG 92:231-237, 1986 Guyton RA, Davis SC, Michalik RE, Williams WH, Hatcher CP: Right heart assist by intermittent abdominal compression after surgery for congenital heart disease. Circulation 72:Suppl 2:97-100, 1985 Molina JE, Wang Y, Lucas R, Moller J: The technique of the Fontan procedure with posterior right atrium-pulmonary artery connection. Ann Thorac Surg 39:371-375, 1985 Oelert H, Borst HG: Modified Fontan procedure using a retroaortic atriopulmonary anastomosis. Thorac Cardiovase Surg 32:392-394, 1984 Doty DB, Marvin WJ, Lauer RM: Modified Fontan procedure. Methods to achieve direct anastomosis of right atrium to pulmonary artery. J THORAC CARDIOVASC SURG 81:470-475,1981 Bjork YO, Olin CL, Bjarke BB, Thoren CA: Right atrial-right ventricular anastomosis for correlation of tricuspid atresia. J THoRAc CARDIOVASC SURG 77:452458, 1979
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42 Ottenkamp J, Pohmer J, Quaegebeur JM, Brom AG, Fontan F: Nine years' experience of physiological correction of tricuspid atresia. Long term results and current surgical approach. Thorax 37:718-726, 1982 43 Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H: Selection criteria for Fontan's procedure,
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Pediatric Cardiology, RH Anderson, EA Shinebourne, eds., Edinburgh, 1978, Churchill Livingstone, pp 559566 (For Discussion see page 1044.)