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The Mustard procedure in transposition of the great arteries associated with juxtaposition of the atrial appendages with and without dextrocardia
J THoRAc
CARDIOVASC SURG
85:451-456, 1983
The Mustard procedure in transposition of the great arteries associated with juxtaposition of the atrial appendages with and without dextrocardia Left-sided juxtaposition of the right atrial appendage (LJRAA) was seen in 10 patients in a series of 361 consecutive Mustard procedures for transposition of the great arteries (TGA). Dextrocardia complicated LJRAA in four cases. Right atrial capacity and free atrial wall size were smaller than normal in all patients, and a Mustard intra-atrial baffle repair was performed in all instances. Direct caval cannulation or cannulation of either atrial appendage facilitated intra-atrial repair. Nine patients in whom the pulmonary venous atrium was enlarged with a patch survived. One child who did not have a right atrial patch died 1 hour postoperatively of pulmonary edema. Two late deaths occurred 1 year postoperatively. Seven children survived and are well. In one child, superior vena caval baffle obstruction is controlled by digoxin and diuretic therapy. TGA associated with juxtaposition of the atrial appendages (JAA) and dextrocardia may be successfully repaired by the Mustard procedure, provided that the tricuspid valve and right ventricle are normal, an adequate superior vena caval channel is created, and patch enlargement of the pulmonary venous atrium is undertaken.
Alfred E. Wood, M.B., B.Ch., Robert M. Freedom, M.D., William G. Williams, M.D., and George A. Trusler, M.D. Toronto, Ontario, Canada
Juxtaposition of the atrial appendages (JAA) is a rare congenital anomaly that usually is associated with transposition of the great arteries (TGA) and is an indication of severe cyanotic congenital heart disease. It is identified in 1.6% to 6.0% of patients with TGA.!-3 In JAA the atrial appendages lie side by side to the left or right of the great arteries (left and right juxtaposition).4 Left-sided juxtaposition of the right atrial appendage (LJRAA) is the more common condition! and in 56% of such cases the right atrial appendage is bifid, with one portion lying in the normal position to the right of the great arteries and the other to the left, adjacent to the left atrial appendage. I, 2 From the Divisions of Cardiovascular Surgery (Department of Surgery) and Cardiology (Department of Paediatrics), the University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada. Received for publication April 8, 1982. Accepted for publication May 17, 1982. Address for reprints: W. G. Williams, M.D., Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario, Canada M5G IX8.
Dextrocardia occurs twice as often in TGA with juxtaposition as in simple TGA.4 The presence of JAA reduces right atrial volume and free wall size." Dextrocardia, because of overlying of the right atrium, reduces the right atrial free wall even more and makes exposure difficult. We report our experience at The Hospital for Sick Children, Toronto, using the Mustard procedure for TGA associated with JAA with or without dextrocardia. Patients and methods
Ten patients with LJRAA have been encountered in a series of 361 (2.8%) consecutive Mustard procedures performed for TGA since October, 1963. All patients had complete TGA. Four also had dextrocardia. The associated cardiac anomalies for each patient are given in Table I. All 10 patients underwent preoperative cardiac catheterization with angiography and most had echocardiography. Previous operations had been performed in five cases, Blalock-Hanlon procedures in three and unsuccessful attempts at a Blalock-Hanlon procedure and a Mustard procedure in one each. 451
452
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Table I. Patient data, operative procedure, and follow-up Case
Diagnosis
Procedure
Right atrial patch (mm)
Postop. catheterization
2.4
d-TGA, ASD, PS, 1AA
Mustard
Pericardium (20 by 40)
None
2
1.9
d-TGA, ASD, 1AA
Mustard
None
3
3.3
d-TGA, YSD, PA, 1AA
Mustard
Pericardium (20 by 20) None
d-TGA, ASD (B-H), 1AA
Mustard
PTFE (35 by 30)
d-TGA, YSD, PS, bilateral B-T,1AA d-TGA, YSD, PS, LSYC, 1AA, dextrocardia
Mustard, ligation of B-T, LY-PA conduit Mustard, YSD closure, pulmonary valvotomy
Pericardium (35 by 35) Pericardium (35 by 50)
S YC obstruction, A = 37 mm Hg, v = 20 mm Hg Small residual YSD, shunt 1.8: I None
4
5
8.3
6
4.9
7
1.4
d-TGA, multiple YSD, ASD (B-H), 1AA, dextrocardia
Mustard, YSD closure
Pericardium (40 by 40)
8
0.3
Mustard
9
4.2
10
1.0
d-TGA, 1AA, attempted B-H, dextrocardia d-TGA, 1AA, attempted Mustard, dextrocardia d-TGA, 1AA, PS, ASD (B-H)
Xenograft pericardium (20 by 45) Xenograft pericardium (30 by 35) Xenograft pericardium (25 by 40)
Mustard Mustard
None
Small residual YSD, shunt 1.3: 1, mild TI None None None
Comment Died I yr postop., severe RYH on autopsy Arrhythmia Died I hr postop., acute pulmonary edema
Died I yr postop., severe RYH with EFE on autopsy
Bilateral anterior thoracotomy Bilateral anterior thoracotomy T-shaped atrial incision
Legend: ASD, Atrial septal defect. B-H, Blalock-Hanlon. B-T. Blalock-Taussig shunt. d-TGA, Dextro-transposition of the great aneries. EFE, Endocardial fibroelastosis. JAA, Juxtaposed atrial appendages. LSVC, Left superior vena cava. LV-PA, Left ventricle-pulmonary artery. PA, Pulmonary anery. PS. Pulmonary stenosis. PTFE, Polytetrafluoroethylene. RVH. Right ventricular hypertrophy. SVC, Superior vena cava. Tl. Tricuspid incompetence. VSD. Ventricular septal defect.
Age at operation ranged from 4 months to 8 years. In all patients standard cardiopulmonary bypass techniques and profound hypothermia were used for the repair. Children weighing 10 kg and less were cooled to a rectal temperature of 18° C or less and circulatory arrest was instituted. A single atrial cannula was used in this instance. Direct caval cannulation was performed in six patients undergoing cardiopulmonary bypass. Cardioplegia and a cold pericardial bath were also used in the last seven patients (since 1977) to improve myocardial protection. The approach was a median sternotomy in eight patients and bilateral anterior thoracotomy in two patients who had dextrocardia (Cases 8 and 9). All patients had a small right atrium and a thin wedge of free right atrial wall (Fig. 1). The right atrial wall was especially narrow in the four patients with dextrocardia (Cases 6 to 9). Right atriotomy was performed in a standard manner-a longitudinal incision anterior to the crista terminalis. The interatrial septum, which is
also small in LJRAA, was excised as necessary, with an attempt made to preserve the sinoatrial node artery. A rectangular wedge-shaped baffle with a slight waist and rounded caval ends was used in all cases. Baffle measurement was 70 by 50 mm in most instances." The baffle material was autogenous pericardium in nine patients and xenograft pericardium in one (Case 9), whose own pericardium had been removed at a previous operation. A coronary sinus cutback was performed routinely. The baffle was sutured in place with the pulmonary veins and caval orifices as landmarks." The os of the juxtaposed right atrial appendage remained on the systemic venous side of the baffle in all 10 patients. In two, ventricular septal defects (VSDs) were repaired with patches, one through the tricuspid valve (Case 7) and one through a right ventriculotomy (Case 6). Left ventricular outflow tract obstruction was managed by transpulmonary fibromyomectomy in patient 6 and by insertion of a valved conduit from the left
Volume 85 Number 3 March, 1983
TGA with JAA and dextrocardia
453
Fig. 1. Artist's impression of the intra-atrial anatomy as seen at operation in transposition of the great arteries and juxtaposition of the atrial appendages. Dotted line represents baffle suture line. Inset, External appearance showing atriotomy with extension between pulmonary veins. SVC, Superior vena cava. IVC, Inferior vena cava. RAA, Right atrial appendage. UA, Left atrial appendage. A, Aorta.
ventricular apex to the pulmonary artery in Patient 5. Right atrial enlargement of the new pulmonary venous atrium was performed in nine patients. A transverse extension of the right atriotomy between the superior and inferior right pulmonary veins was necessary for adequate enlargement of the new pulmonary venous atrium in two patients (Cases 6 and 10). Various atrial patch materials were used; autogenous pericardium in five, xenograft pericardium in three, and polytetrafluoroethylene (PTFE) in one. Patch size ranged from 20 by 20 mm to 40 by 50 mm (see Table I). Results
All patients were successfully weaned from cardiopulmonary bypass. The one early death occurred in a 3-year-old child with TGA, VSD, pulmonary stenosis, and URAA (Case 3) who underwent a standard Mustard procedure without patch enlargement of the right atrium. Death resulted from acute pulmonary edema secondary to pulmonary venous obstruction. Autopsy confirmed the presence of intra-alveolar hemorrhage. All remaining patients had an uneventful postoperative course and were discharged from the hospital in sinus rhythm an average of 14 days after operation.
Patient follow-up
Follow-up in the nine patients ranged from 1 month to 10 years. Two late deaths occurred 1 year following operation. In one patient, who had TGA, atrial septal defect, LJRAA, and subvalvular pulmonary stenosis (Case 1), death occurred suddenly and autopsy revealed severe right ventricular hypertrophy. The repair was satisfactory and no significant left ventricular outflow tract obstruction was present. The second late death occurred in a patient with TGA, VSD, pulmonary stenosis, and dextrocardia (Case 6). A Mustard procedure, patch closure of the VSD through a right ventriculotomy, and pulmonary valvotomy had been performed. Autopsy showed severe right ventricular hypertrophy with endocardial fibroelastosis and a residual 5 mm atrial septal defect. The repair was satisfactory in every other way. The remaining seven patients are in good health. Six are in sinus rhythm and one in atrial flutter, which is well controlled by digoxin. The cardiothoracic ratio ranged from 0.45 to 0.65 (mean 0.55) on chest roentgenogram in all seven survivors. Cardiac catheterization has been performed on three patients postoperatively. Two have small residual VSDs left deliberately untreated at the original repair. The defect was not
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454 Wood et al.
Fig. 2. Atrial angiogram demonstrating juxtaposition of the atrial appendages with displacement of the interatrial septum. Asterisk (*), Juxtaposed right atrial appendage. we, Inferior vena cava. RA, Right atrium. LA, Left atrium.
patched in Patient 5 because the shunt was considered insignificant (I. 8: 1). Patient 7 had multiple defects of which only the membranous one was patched. One patient (Case 4) has partial obstruction of the superior vena cava. A discrete narrowing opposite the septal ridge was seen on the angiogram and the gradient measured at catheterization was 15 mm Hg. The patient had mild symptoms that subsided with digoxin and diuretic therapy. Discussion
JAA is reported to occur in 1.6% to 6.0% of patients with TGA.!-3 We have encountered it in 10 of 361 consecutive Mustard procedures (2.8%). Although it may occur with relatively simple cardiac lesions, JAA is often associated with complex forms of transposition (i.e., those with tricuspid atresia or stenosis, hypopla-
Thoracic and Cardiovascular Surgery
sia of the right ventricle, and bilateral muscular subarterial infundibular.':" A bifid URAA accounts for 56% of cases reported with left-sided juxtaposition.' All patients in this series had LJRAA and none had right juxtaposition of the left atrial appendage. Three cases were diagnosed preoperatively from the chest roentgenograms and by selective right atriograms (Fig. 2)7 and the remainder at operation, as in the series of Urban, Stark, and Waterston." Palliative septectomy is difficult to perform in the presence of LJRAA, although it was done successfully in three of our patients. The narrow free right atrial wall makes it extremely difficult to apply a clamp to allow an adequate amount of septum to be resected. Since the septum is smaller than usual, the surgeon must avoid resecting an excessive amount of atrial tissue, which would have disastrous results. Dextrocardia, characterized by even more encroachment on the free right atrial wall, makes palliative septectomy very difficult. Indeed, in one patient (Case 8) an unsuccessful Blalock-Hanlon procedure had been attempted elsewhere. Atrial septectomy under inflow occlusion may allow successful palliation, although we did not use it. Early Mustard repair, as performed in Case 8, is probably the most appropriate approach in TGA with LJRAA and dextrocardia. Dextrocardia complicated LJRAA in four patients (Cases 6 to 9). Its presence reduced right atrial size even more and made exposure of the right atrium difficult. In two patients (Cases 8 and 9) total repair was performed through a bilateral thoracotomy incision and exposure was considerably better. Right thoracotomy would also give excellent exposure of the right atrium when atrial juxtaposition is present. Direct cannulation of the cavae facilitated the Mustard repair in the six patients while being maintained on cardiopulmonary bypass. The cannulas were well away from the small right atrium and did not restrict the right atriotomy. This is similar to the technique described by Urban, Stark, and Waterston." Direct atrial cannulation was performed in four patients in whom circulatory arrest and profound hypothermia were used. Three were cannulated through the URAA and one through the left atrial appendage, by means of the technique described by McKneally and colleagues." Atrial cannulation was frequently difficult but provided very satisfactory venous drainage. Rosenquist, Stark, and Taylor established that the free right atrial wall, right atrial cavity, and interatrial septum are smaller than in "simple" complete TGA and suggested that a Mustard intra-atrial repair might
Volume 85 Number 3 March, 1983
be difficult to carry out. However, Urban, Stark, and Waterston" showed that this repair was possible, and our experience supports this. Before the repair is begun, the tricuspid valve and right ventricle must be carefully inspected to determine whether they are normal, since there is a high incidence of tricuspid valvular and right ventricular hypoplasia in the presence of JAA.4 Such hypoplasia precludes the use of intra-atrial repair, which would undoubtedly lead to failure." The Mustard" procedure, which is our operation of choice for simple TGA, was performed on all 10 patients in this series. It currently has an operative risk of 1.7% and a 5 year survival rate of 93%. Pericardium, our preferred material, was used as the baffle in nine. 5. 8, 9 Xenograft pericardium was used in one patient because her pericardium had been previously resected during an attempted Mustard procedure. We hope this material is less likely to cause baffle obstruction, a common complication of other prosthetic materials. 10. 11 The baffle was sutured in place using the landmarks described previously. 5. 9, 10 The os of the juxtaposed right atrial appendage remained on the new physiological systemic venous side of the baffle in all patients. We did not attempt to leave the juxtaposed atrial appendage on the pulmonary venous side of the baffle since we felt that the superior vena caval channel would be too narrow and, therefore, easily obstructed (Fig. 1). Indeed, superior vena caval obstruction developed in one patient in this series (Case 4) and one in the series of Urban, Stark, and Waterston (Case 5),3 an incidence of 12.5% for the combined series. This is more than double the incidence occurring in our total series of Mustard procedures for "simple" TGA. Our patient responded to medical therapy without reoperation, as in our previous experience. 5 It was not necessary to excise the septum between the LJRAA and the left auricle to enlarge the atrial septal defect in any patient, as suggested by Rosenquist, Stark, and Taylor and performed by Urban, Stark, and Waterston." The physiological pulmonary venous atrium was en-larged in nine patients. The remaining child (Case 3) died 1 hour postoperatively of acute pulmonary venous obstruction. We and others" believe that enlargement of the atrium is necessary in all cases of TGA and JAA, since the capacity is severely reduced when the LJRAA remains on the systemic venous side and because of the narrow free right atrial wall. Various materials were used for atrial patches in this series; again, autogenous
TGA with fAA and dextrocardia
45 5
and xenograft pericardium are our materials of choice. The T-shaped extension of the right atrial incision permitted appropriate enlargement of the new physiological left atrium in two patients (Cases 6 and 10). The most commonly encountered additional lesions in JAA and TGA are VSD, secundum atrial septal defect, underdeveloped tricuspid valve and right ventricle, bilateral muscular infundibula, and left ventricular outflow tract obstruction.l" All our patients had at least one defect, as in the series reported by Rosenquist," Urban," and their co-workers. In retrospect, Patient 6, who had a Mustard procedure, patch closure of a VSD, and a transpulmonary resection of subpulmonary stenosis and who died 1 year later of congestive failure, would probably have been better managed by a Rastelli repair." In the other late death autopsy failed to determine a cause of death. Gross right ventricular hypertrophy was present and the repair was satisfactory. Presumably, death resulted from an arrhythmia. In view of the size of the free right atrial wall and the interatrial septum, a Mustard repair is probably easier to perform than a Senning repair. Since a satisfactory Senning repair requires at least 2 em of free atrial wall,13, 14 additional material may have to be added to provide an interatrial flap of sufficient length. This would certainly be necessary if a previous atrial septectomy had been performed. We are not aware of a successful case report of JAA and TGA repaired by the Senning technique. Dextrocardia and JAA complicating TGA reduce right atrial size significantly. Mustard's intra-atrial baffle repair can be accomplished with good results, provided the pulmonary venous atrium is enlarged and an adequate superior vena caval channel and the presence of normal tricuspid valve and right ventricular anatomy are ensured. Bilateral anterior thoracotomy facilitates repair in the presence of dextrocardia. Longterm follow-up demonstrates that as good results can be obtained in this small subset of TGA as in repairs of simple TGA.5, 13, 15, 16 We would like to express our appreciation to the artist, Mr. A. Wright. REFERENCES Charuzi Y, Spanos PK, Amplatz K, Edwards JE: Juxtaposition of the atrial appendages. Circulation 47:620627, 1973 2 Rosenquist GC, Stark J, Taylor JFN: Anatomical relationships in transposition of the great arteries. Juxtaposi-
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3
4
5
6
7
8 9
10
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tion of the atrial appendages. Ann Thorac Surg 18:456461,1974 Urban AE, Stark J, Waterston DJ: Mustard's operation for transposition of the atrial appendages. Ann Thorac Surg 21:304-310, 1976 Melhuish BPP, Van Praagh R: Juxtaposition of the atrial appendages. A sign of severe cyanotic congenital heart disease. Br Heart J 30:269-284, 1968 Trusler GA, Williams WG, Izukawa T, Olley PM: Current results with the Mustard operation in isolated transposition of the great arteries. J THORAC CARDIOVASC SURG80:381-389, 1980 McKneally MF, Wolff GS, Shaher RM, Alley RD: Cannulation through the left atrial appendage facilitates Mustard's operation for transposition of the great arteries. Ann Thorac Surg 18:462-465, 1974 Tyrrell MJ, Moes CAF: Congenital levoposition of the right atrial appendage. Am J Dis Child 121:508-510, 1971 Mustard WT: Successful two-stage correction of transposition of the great vessels. Surgery 55:469-472, 1964 Mustard WT, Keith JD, Trusler GA, Fowler R, Kidd L: The surgical management of transposition of the great vessels. J THoRAc CARDIOVASC SURG 48:953-958, 1964 Stark J, Silove ED, Taylor JFN, Graham GR: Obstruction
Thoracic and Cardiovascular Surgery
11
12
13
14 15
16
to systemic venous return following the Mustard operation for transposition of the great arteries. J THoRAc CARDIOVASC SURG 68:742-749, 1974 Oelert H, Laprell H, Piepenbrock S, Luhmer I, Kallfelz HC, Borst HG: Emergency and non-emergency intraatrial correction for transposition of the great arteries in 43 infants. Indications, details of the operative technique and results. Thoraxchir Vask Chir 25:305-313, 1977 Me Goon DC, Wallace RB, Danielson GK: The Rastelli operation. Its indications and results. J THoRAc CA,RDIOVASC SURG 65:65-75, 1973 Parenzan L, Locatelli G, Alfieri 0, Villani M, Invernizzi G: The Senning operation for transposition of the great arteries. J THoRAc CARDIOVASC SURG 76:305-311, 1978 Senning A: Surgical correction of transposition of the great vessels. Surgery 45: 966-980, 1959 Quaegebeur JM, Rohmer J, Brom AG, Tinkelenberg J: Revival of the Senning operation in the treatment of transposition of the great arteries. Preliminary report on recent experience. Thorax 32:517-524, 1977 Piccoli GP, Wilkinson JL, Arnold R, Musumeci F, Hamilton 01: Appraisal of the Mustard procedure for the physiological correction of •'simple " transposition of the great arteries. Eighty consecutive cases, 1970-1980, J THORAC CARDIOVASC SURG 82:436-446, 1981
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CARDIOVASC SURG
85:451-456, 1983
The Mustard procedure in transposition of the great arteries associated with juxtaposition of the atrial appendages with and without dextrocardia Left-sided juxtaposition of the right atrial appendage (LJRAA) was seen in 10 patients in a series of 361 consecutive Mustard procedures for transposition of the great arteries (TGA). Dextrocardia complicated LJRAA in four cases. Right atrial capacity and free atrial wall size were smaller than normal in all patients, and a Mustard intra-atrial baffle repair was performed in all instances. Direct caval cannulation or cannulation of either atrial appendage facilitated intra-atrial repair. Nine patients in whom the pulmonary venous atrium was enlarged with a patch survived. One child who did not have a right atrial patch died 1 hour postoperatively of pulmonary edema. Two late deaths occurred 1 year postoperatively. Seven children survived and are well. In one child, superior vena caval baffle obstruction is controlled by digoxin and diuretic therapy. TGA associated with juxtaposition of the atrial appendages (JAA) and dextrocardia may be successfully repaired by the Mustard procedure, provided that the tricuspid valve and right ventricle are normal, an adequate superior vena caval channel is created, and patch enlargement of the pulmonary venous atrium is undertaken.
Alfred E. Wood, M.B., B.Ch., Robert M. Freedom, M.D., William G. Williams, M.D., and George A. Trusler, M.D. Toronto, Ontario, Canada
Juxtaposition of the atrial appendages (JAA) is a rare congenital anomaly that usually is associated with transposition of the great arteries (TGA) and is an indication of severe cyanotic congenital heart disease. It is identified in 1.6% to 6.0% of patients with TGA.!-3 In JAA the atrial appendages lie side by side to the left or right of the great arteries (left and right juxtaposition).4 Left-sided juxtaposition of the right atrial appendage (LJRAA) is the more common condition! and in 56% of such cases the right atrial appendage is bifid, with one portion lying in the normal position to the right of the great arteries and the other to the left, adjacent to the left atrial appendage. I, 2 From the Divisions of Cardiovascular Surgery (Department of Surgery) and Cardiology (Department of Paediatrics), the University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada. Received for publication April 8, 1982. Accepted for publication May 17, 1982. Address for reprints: W. G. Williams, M.D., Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario, Canada M5G IX8.
Dextrocardia occurs twice as often in TGA with juxtaposition as in simple TGA.4 The presence of JAA reduces right atrial volume and free wall size." Dextrocardia, because of overlying of the right atrium, reduces the right atrial free wall even more and makes exposure difficult. We report our experience at The Hospital for Sick Children, Toronto, using the Mustard procedure for TGA associated with JAA with or without dextrocardia. Patients and methods
Ten patients with LJRAA have been encountered in a series of 361 (2.8%) consecutive Mustard procedures performed for TGA since October, 1963. All patients had complete TGA. Four also had dextrocardia. The associated cardiac anomalies for each patient are given in Table I. All 10 patients underwent preoperative cardiac catheterization with angiography and most had echocardiography. Previous operations had been performed in five cases, Blalock-Hanlon procedures in three and unsuccessful attempts at a Blalock-Hanlon procedure and a Mustard procedure in one each. 451
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Table I. Patient data, operative procedure, and follow-up Case
Diagnosis
Procedure
Right atrial patch (mm)
Postop. catheterization
2.4
d-TGA, ASD, PS, 1AA
Mustard
Pericardium (20 by 40)
None
2
1.9
d-TGA, ASD, 1AA
Mustard
None
3
3.3
d-TGA, YSD, PA, 1AA
Mustard
Pericardium (20 by 20) None
d-TGA, ASD (B-H), 1AA
Mustard
PTFE (35 by 30)
d-TGA, YSD, PS, bilateral B-T,1AA d-TGA, YSD, PS, LSYC, 1AA, dextrocardia
Mustard, ligation of B-T, LY-PA conduit Mustard, YSD closure, pulmonary valvotomy
Pericardium (35 by 35) Pericardium (35 by 50)
S YC obstruction, A = 37 mm Hg, v = 20 mm Hg Small residual YSD, shunt 1.8: I None
4
5
8.3
6
4.9
7
1.4
d-TGA, multiple YSD, ASD (B-H), 1AA, dextrocardia
Mustard, YSD closure
Pericardium (40 by 40)
8
0.3
Mustard
9
4.2
10
1.0
d-TGA, 1AA, attempted B-H, dextrocardia d-TGA, 1AA, attempted Mustard, dextrocardia d-TGA, 1AA, PS, ASD (B-H)
Xenograft pericardium (20 by 45) Xenograft pericardium (30 by 35) Xenograft pericardium (25 by 40)
Mustard Mustard
None
Small residual YSD, shunt 1.3: 1, mild TI None None None
Comment Died I yr postop., severe RYH on autopsy Arrhythmia Died I hr postop., acute pulmonary edema
Died I yr postop., severe RYH with EFE on autopsy
Bilateral anterior thoracotomy Bilateral anterior thoracotomy T-shaped atrial incision
Legend: ASD, Atrial septal defect. B-H, Blalock-Hanlon. B-T. Blalock-Taussig shunt. d-TGA, Dextro-transposition of the great aneries. EFE, Endocardial fibroelastosis. JAA, Juxtaposed atrial appendages. LSVC, Left superior vena cava. LV-PA, Left ventricle-pulmonary artery. PA, Pulmonary anery. PS. Pulmonary stenosis. PTFE, Polytetrafluoroethylene. RVH. Right ventricular hypertrophy. SVC, Superior vena cava. Tl. Tricuspid incompetence. VSD. Ventricular septal defect.
Age at operation ranged from 4 months to 8 years. In all patients standard cardiopulmonary bypass techniques and profound hypothermia were used for the repair. Children weighing 10 kg and less were cooled to a rectal temperature of 18° C or less and circulatory arrest was instituted. A single atrial cannula was used in this instance. Direct caval cannulation was performed in six patients undergoing cardiopulmonary bypass. Cardioplegia and a cold pericardial bath were also used in the last seven patients (since 1977) to improve myocardial protection. The approach was a median sternotomy in eight patients and bilateral anterior thoracotomy in two patients who had dextrocardia (Cases 8 and 9). All patients had a small right atrium and a thin wedge of free right atrial wall (Fig. 1). The right atrial wall was especially narrow in the four patients with dextrocardia (Cases 6 to 9). Right atriotomy was performed in a standard manner-a longitudinal incision anterior to the crista terminalis. The interatrial septum, which is
also small in LJRAA, was excised as necessary, with an attempt made to preserve the sinoatrial node artery. A rectangular wedge-shaped baffle with a slight waist and rounded caval ends was used in all cases. Baffle measurement was 70 by 50 mm in most instances." The baffle material was autogenous pericardium in nine patients and xenograft pericardium in one (Case 9), whose own pericardium had been removed at a previous operation. A coronary sinus cutback was performed routinely. The baffle was sutured in place with the pulmonary veins and caval orifices as landmarks." The os of the juxtaposed right atrial appendage remained on the systemic venous side of the baffle in all 10 patients. In two, ventricular septal defects (VSDs) were repaired with patches, one through the tricuspid valve (Case 7) and one through a right ventriculotomy (Case 6). Left ventricular outflow tract obstruction was managed by transpulmonary fibromyomectomy in patient 6 and by insertion of a valved conduit from the left
Volume 85 Number 3 March, 1983
TGA with JAA and dextrocardia
453
Fig. 1. Artist's impression of the intra-atrial anatomy as seen at operation in transposition of the great arteries and juxtaposition of the atrial appendages. Dotted line represents baffle suture line. Inset, External appearance showing atriotomy with extension between pulmonary veins. SVC, Superior vena cava. IVC, Inferior vena cava. RAA, Right atrial appendage. UA, Left atrial appendage. A, Aorta.
ventricular apex to the pulmonary artery in Patient 5. Right atrial enlargement of the new pulmonary venous atrium was performed in nine patients. A transverse extension of the right atriotomy between the superior and inferior right pulmonary veins was necessary for adequate enlargement of the new pulmonary venous atrium in two patients (Cases 6 and 10). Various atrial patch materials were used; autogenous pericardium in five, xenograft pericardium in three, and polytetrafluoroethylene (PTFE) in one. Patch size ranged from 20 by 20 mm to 40 by 50 mm (see Table I). Results
All patients were successfully weaned from cardiopulmonary bypass. The one early death occurred in a 3-year-old child with TGA, VSD, pulmonary stenosis, and URAA (Case 3) who underwent a standard Mustard procedure without patch enlargement of the right atrium. Death resulted from acute pulmonary edema secondary to pulmonary venous obstruction. Autopsy confirmed the presence of intra-alveolar hemorrhage. All remaining patients had an uneventful postoperative course and were discharged from the hospital in sinus rhythm an average of 14 days after operation.
Patient follow-up
Follow-up in the nine patients ranged from 1 month to 10 years. Two late deaths occurred 1 year following operation. In one patient, who had TGA, atrial septal defect, LJRAA, and subvalvular pulmonary stenosis (Case 1), death occurred suddenly and autopsy revealed severe right ventricular hypertrophy. The repair was satisfactory and no significant left ventricular outflow tract obstruction was present. The second late death occurred in a patient with TGA, VSD, pulmonary stenosis, and dextrocardia (Case 6). A Mustard procedure, patch closure of the VSD through a right ventriculotomy, and pulmonary valvotomy had been performed. Autopsy showed severe right ventricular hypertrophy with endocardial fibroelastosis and a residual 5 mm atrial septal defect. The repair was satisfactory in every other way. The remaining seven patients are in good health. Six are in sinus rhythm and one in atrial flutter, which is well controlled by digoxin. The cardiothoracic ratio ranged from 0.45 to 0.65 (mean 0.55) on chest roentgenogram in all seven survivors. Cardiac catheterization has been performed on three patients postoperatively. Two have small residual VSDs left deliberately untreated at the original repair. The defect was not
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Fig. 2. Atrial angiogram demonstrating juxtaposition of the atrial appendages with displacement of the interatrial septum. Asterisk (*), Juxtaposed right atrial appendage. we, Inferior vena cava. RA, Right atrium. LA, Left atrium.
patched in Patient 5 because the shunt was considered insignificant (I. 8: 1). Patient 7 had multiple defects of which only the membranous one was patched. One patient (Case 4) has partial obstruction of the superior vena cava. A discrete narrowing opposite the septal ridge was seen on the angiogram and the gradient measured at catheterization was 15 mm Hg. The patient had mild symptoms that subsided with digoxin and diuretic therapy. Discussion
JAA is reported to occur in 1.6% to 6.0% of patients with TGA.!-3 We have encountered it in 10 of 361 consecutive Mustard procedures (2.8%). Although it may occur with relatively simple cardiac lesions, JAA is often associated with complex forms of transposition (i.e., those with tricuspid atresia or stenosis, hypopla-
Thoracic and Cardiovascular Surgery
sia of the right ventricle, and bilateral muscular subarterial infundibular.':" A bifid URAA accounts for 56% of cases reported with left-sided juxtaposition.' All patients in this series had LJRAA and none had right juxtaposition of the left atrial appendage. Three cases were diagnosed preoperatively from the chest roentgenograms and by selective right atriograms (Fig. 2)7 and the remainder at operation, as in the series of Urban, Stark, and Waterston." Palliative septectomy is difficult to perform in the presence of LJRAA, although it was done successfully in three of our patients. The narrow free right atrial wall makes it extremely difficult to apply a clamp to allow an adequate amount of septum to be resected. Since the septum is smaller than usual, the surgeon must avoid resecting an excessive amount of atrial tissue, which would have disastrous results. Dextrocardia, characterized by even more encroachment on the free right atrial wall, makes palliative septectomy very difficult. Indeed, in one patient (Case 8) an unsuccessful Blalock-Hanlon procedure had been attempted elsewhere. Atrial septectomy under inflow occlusion may allow successful palliation, although we did not use it. Early Mustard repair, as performed in Case 8, is probably the most appropriate approach in TGA with LJRAA and dextrocardia. Dextrocardia complicated LJRAA in four patients (Cases 6 to 9). Its presence reduced right atrial size even more and made exposure of the right atrium difficult. In two patients (Cases 8 and 9) total repair was performed through a bilateral thoracotomy incision and exposure was considerably better. Right thoracotomy would also give excellent exposure of the right atrium when atrial juxtaposition is present. Direct cannulation of the cavae facilitated the Mustard repair in the six patients while being maintained on cardiopulmonary bypass. The cannulas were well away from the small right atrium and did not restrict the right atriotomy. This is similar to the technique described by Urban, Stark, and Waterston." Direct atrial cannulation was performed in four patients in whom circulatory arrest and profound hypothermia were used. Three were cannulated through the URAA and one through the left atrial appendage, by means of the technique described by McKneally and colleagues." Atrial cannulation was frequently difficult but provided very satisfactory venous drainage. Rosenquist, Stark, and Taylor established that the free right atrial wall, right atrial cavity, and interatrial septum are smaller than in "simple" complete TGA and suggested that a Mustard intra-atrial repair might
Volume 85 Number 3 March, 1983
be difficult to carry out. However, Urban, Stark, and Waterston" showed that this repair was possible, and our experience supports this. Before the repair is begun, the tricuspid valve and right ventricle must be carefully inspected to determine whether they are normal, since there is a high incidence of tricuspid valvular and right ventricular hypoplasia in the presence of JAA.4 Such hypoplasia precludes the use of intra-atrial repair, which would undoubtedly lead to failure." The Mustard" procedure, which is our operation of choice for simple TGA, was performed on all 10 patients in this series. It currently has an operative risk of 1.7% and a 5 year survival rate of 93%. Pericardium, our preferred material, was used as the baffle in nine. 5. 8, 9 Xenograft pericardium was used in one patient because her pericardium had been previously resected during an attempted Mustard procedure. We hope this material is less likely to cause baffle obstruction, a common complication of other prosthetic materials. 10. 11 The baffle was sutured in place using the landmarks described previously. 5. 9, 10 The os of the juxtaposed right atrial appendage remained on the new physiological systemic venous side of the baffle in all patients. We did not attempt to leave the juxtaposed atrial appendage on the pulmonary venous side of the baffle since we felt that the superior vena caval channel would be too narrow and, therefore, easily obstructed (Fig. 1). Indeed, superior vena caval obstruction developed in one patient in this series (Case 4) and one in the series of Urban, Stark, and Waterston (Case 5),3 an incidence of 12.5% for the combined series. This is more than double the incidence occurring in our total series of Mustard procedures for "simple" TGA. Our patient responded to medical therapy without reoperation, as in our previous experience. 5 It was not necessary to excise the septum between the LJRAA and the left auricle to enlarge the atrial septal defect in any patient, as suggested by Rosenquist, Stark, and Taylor and performed by Urban, Stark, and Waterston." The physiological pulmonary venous atrium was en-larged in nine patients. The remaining child (Case 3) died 1 hour postoperatively of acute pulmonary venous obstruction. We and others" believe that enlargement of the atrium is necessary in all cases of TGA and JAA, since the capacity is severely reduced when the LJRAA remains on the systemic venous side and because of the narrow free right atrial wall. Various materials were used for atrial patches in this series; again, autogenous
TGA with fAA and dextrocardia
45 5
and xenograft pericardium are our materials of choice. The T-shaped extension of the right atrial incision permitted appropriate enlargement of the new physiological left atrium in two patients (Cases 6 and 10). The most commonly encountered additional lesions in JAA and TGA are VSD, secundum atrial septal defect, underdeveloped tricuspid valve and right ventricle, bilateral muscular infundibula, and left ventricular outflow tract obstruction.l" All our patients had at least one defect, as in the series reported by Rosenquist," Urban," and their co-workers. In retrospect, Patient 6, who had a Mustard procedure, patch closure of a VSD, and a transpulmonary resection of subpulmonary stenosis and who died 1 year later of congestive failure, would probably have been better managed by a Rastelli repair." In the other late death autopsy failed to determine a cause of death. Gross right ventricular hypertrophy was present and the repair was satisfactory. Presumably, death resulted from an arrhythmia. In view of the size of the free right atrial wall and the interatrial septum, a Mustard repair is probably easier to perform than a Senning repair. Since a satisfactory Senning repair requires at least 2 em of free atrial wall,13, 14 additional material may have to be added to provide an interatrial flap of sufficient length. This would certainly be necessary if a previous atrial septectomy had been performed. We are not aware of a successful case report of JAA and TGA repaired by the Senning technique. Dextrocardia and JAA complicating TGA reduce right atrial size significantly. Mustard's intra-atrial baffle repair can be accomplished with good results, provided the pulmonary venous atrium is enlarged and an adequate superior vena caval channel and the presence of normal tricuspid valve and right ventricular anatomy are ensured. Bilateral anterior thoracotomy facilitates repair in the presence of dextrocardia. Longterm follow-up demonstrates that as good results can be obtained in this small subset of TGA as in repairs of simple TGA.5, 13, 15, 16 We would like to express our appreciation to the artist, Mr. A. Wright. REFERENCES Charuzi Y, Spanos PK, Amplatz K, Edwards JE: Juxtaposition of the atrial appendages. Circulation 47:620627, 1973 2 Rosenquist GC, Stark J, Taylor JFN: Anatomical relationships in transposition of the great arteries. Juxtaposi-
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4
5
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7
8 9
10
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tion of the atrial appendages. Ann Thorac Surg 18:456461,1974 Urban AE, Stark J, Waterston DJ: Mustard's operation for transposition of the atrial appendages. Ann Thorac Surg 21:304-310, 1976 Melhuish BPP, Van Praagh R: Juxtaposition of the atrial appendages. A sign of severe cyanotic congenital heart disease. Br Heart J 30:269-284, 1968 Trusler GA, Williams WG, Izukawa T, Olley PM: Current results with the Mustard operation in isolated transposition of the great arteries. J THORAC CARDIOVASC SURG80:381-389, 1980 McKneally MF, Wolff GS, Shaher RM, Alley RD: Cannulation through the left atrial appendage facilitates Mustard's operation for transposition of the great arteries. Ann Thorac Surg 18:462-465, 1974 Tyrrell MJ, Moes CAF: Congenital levoposition of the right atrial appendage. Am J Dis Child 121:508-510, 1971 Mustard WT: Successful two-stage correction of transposition of the great vessels. Surgery 55:469-472, 1964 Mustard WT, Keith JD, Trusler GA, Fowler R, Kidd L: The surgical management of transposition of the great vessels. J THoRAc CARDIOVASC SURG 48:953-958, 1964 Stark J, Silove ED, Taylor JFN, Graham GR: Obstruction
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11
12
13
14 15
16
to systemic venous return following the Mustard operation for transposition of the great arteries. J THoRAc CARDIOVASC SURG 68:742-749, 1974 Oelert H, Laprell H, Piepenbrock S, Luhmer I, Kallfelz HC, Borst HG: Emergency and non-emergency intraatrial correction for transposition of the great arteries in 43 infants. Indications, details of the operative technique and results. Thoraxchir Vask Chir 25:305-313, 1977 Me Goon DC, Wallace RB, Danielson GK: The Rastelli operation. Its indications and results. J THoRAc CA,RDIOVASC SURG 65:65-75, 1973 Parenzan L, Locatelli G, Alfieri 0, Villani M, Invernizzi G: The Senning operation for transposition of the great arteries. J THoRAc CARDIOVASC SURG 76:305-311, 1978 Senning A: Surgical correction of transposition of the great vessels. Surgery 45: 966-980, 1959 Quaegebeur JM, Rohmer J, Brom AG, Tinkelenberg J: Revival of the Senning operation in the treatment of transposition of the great arteries. Preliminary report on recent experience. Thorax 32:517-524, 1977 Piccoli GP, Wilkinson JL, Arnold R, Musumeci F, Hamilton 01: Appraisal of the Mustard procedure for the physiological correction of •'simple " transposition of the great arteries. Eighty consecutive cases, 1970-1980, J THORAC CARDIOVASC SURG 82:436-446, 1981
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