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Transposition of the great arteries
Transposition of the great arteries Reoperation for dysfunctional intra-atrial baffle Correction of transposition oj the great arteries is accomplished by a modified Rastelli procedure, if an adequate ventricular septal defect is present, or by an intra-atrial baffle procedure, if the ventricular septal defect is small or absent. The Albert or Mustard procedure for transposition of the great arteries should be regarded more precisely as the first stage in a series of procedures which may be necessary in any given case. Prosthetic material for the intra-atrial baffle is no guarantee that venous inflow obstruction will not eventuate. Less difficulty has resulted from initial use of pericardium. It is easier to redo an obstructing pericardial atrial baffle than a prosthetic baffle. A pseudointima attaches to either side of the plastic baffle, dictating total replacement with pericardium or a new prosthesis. Caval cannulation technique [or correction of transposition of the great arteries depends on whether it is the initial correction or reoperation for intra-atrial baffle dysfunction that is being dictated by the intra-atrial anatomy in the two situations.
Todd M. Grehl, M.D., and Norman E. Shumway, M.D., Stanford, Calif.
TransPosition of the great arteries has been considered the most common cause of death in infants with congenital heart disease.' Approximately half of these infants die within 1 month of birth and 86 per cent die within 6 months- without surgical intervention. The introduction of the intra-atrial baffle for diversion of venous return (Albert" or Mustard- procedure) to the heart for the correction of transposition of the great arteries has allowed these children to enjoy active lives. The longevity of these patients is still unknown. There have been problems with partial occlusion of venous inflow in patients submitted to correction by the intraatrial baffle technique. This report deals with reoperation for revision of the intraatrial baffle in the correction of transposition of the great arteries. From the Department of Cardiovascular Surgery, Stanford University School of Medicine, Stanford, Calif. 94305. Received for publication March I, 1974. Address for reprints: Todd M. Grehl, M.D., Department of Cardiovascular Surgery, Stanford University Medical Center, Stanford, Calif. 94305.
Case reports CASE l. C. W., a female child, was born following an uncomplicated pregnancy and delivery. She was noted to be cyanotic and in congestive heart failure at 2Y:z weeks of age. Cardiac catheterization at that time revealed transposition of the great arteries with an intact ventricular septum. A Blalock-Hanlon procedure was then carried out. She developed well over the next several years except for frequent respiratory infections. At the age of 7 years she developed a seizure disorder which was well controlled with diphenylhydantoin and phenobarbital. In November, 1972, at the age of 81/ 2 years, she underwent a Mustard procedure with a pericardial intra-atrial baffle. Four weeks postoperatively she developed the postpericardiotomy syndrome for which she was treated with aspirin. Six weeks postoperatively she was noted to have gained 2 kilograms over a I week period and to have developed ascites, peripheral edema, and tachycardia. Repeat cardiac catheterization in January, 1973, revealed partial severe inflow occlusion of the venae cavae at the cava-atrial junctions (Fig. 1). Pressure recordings revealed the following: superior vena cava 11 mm. Hg, inferior vena cava 18 mm. Hg, and left atrium 10 mm. Hg mean. At reoperation the pericardial baffle was thickened and shrunken, causing almost complete obstruc-
863
The Journal of
864
Grehl and Shumway
Thoracic and Cardiovascular Surgery
Fig. 1. Patient C. W. A, Anteroposterior view of superior vena cavagram shows dilation of the superior vena cava and narrowing of the intracardiac baffle. The inferior vena cavagram revealed only collateral venous return to the heart via the azygos system with complete occlusion at the inferior cava-atrial junction. B, Lateral view of superior vena cavagram shows narrowing and tortuosity at the superior cava-atrial junction.
tion of the inferior vena cava and partial obstruction of the superior vena cava. The pulmonary veins were widely patent. The baffle was incised longitudinally from cava to cava, and a knitted Dacron patch was used to enlarge the baffle and relieve the venous obstruction. The postoperative course was normal, and the child has continued to do well. CASE 2. D. c., a male child, was born following a normal delivery, but the pregnancy was complicated by a threatened abortion at 1 month. The patient was cyanotic at birth. Cardiac catheterization in January, 1971, when he was 1 month of age, revealed transposition of the great arteries, patent ductus arteriosus, and a small atrial septal defect. A Rashkind balloon atrial septostomy carried out at the time provided little improvement. The patient was therefore operated upon for ligation of the patent ductus arteriosus and performance of a Blalock-Hanlon atrial septectomy. In May, 1973, when he was 16 months old, a Mustard procedure was carried out with knitted Dacron used for an intra-atrial baffle. The patient did well postoperatively until December, 1973, when he began to develop mild edema of the legs. Repeat cardiac catheterization revealed slight superior vena cava obstruction and high-grade inferior vena cava obstruction (Fig. 2). Pressure
measurements revealed the following: superior vena cava 8 mm. Hg, inferior vena cava 12 mm. Hg, and left atrium 10 mm. Hg mean. Reoperation in December of 1973, when the child was 2 years old, revealed a thick pseudointimal pannus on both sides of the Dacron baffle. The baffle was completely excised along with all previously placed suture material. Sufficient pericardium could be dissected with which to fashion a new baffle. He has continued to do well postoperatively. CASE 3. Patient K. K. Following an uncomplicated labor and delivery, this male child was noted to be cyanotic and in respiratory distress. Cardiac catheterization at 1 day of age revealed transposition of the great arteries, small atrial septal defect, small ventricular septal defect, and a small patent ductus arteriosus. A Rashkind balloon atrial septostomy was carried out at that time. He developed satisfactorily although he became tachypneic and cyanotic on exertion. Repeat cardiac catheterization in April, 1972, confirmed the diagnosis of transposition of the great arteries and atrial septal defect, but there was no residual ventricular septal defect or patent ductus arteriosus. In February, 1973, when he was 2Y<1 years old, total correction was carried out with a knitted Dacron atrial baffle. The patient did well for 6
Volume 67 Number 6 June, 1974
Transposition oi great arteries
865
Fig. 2. A, Inferior vena cavagrarn reveals marked stenosis at the inferior cava-atrial junction in the anteroposterior projection. B, Dilated inferior vena cava with narrowing of intra-atrial chamber in the right anterior oblique position. months until October, 1973, when he was noted to develop mild facial and arm edema after being recumbent. Repeat cardiac catheterization at that time revealed high-grade obstruction of the superior vena cava (Fig. 3). The inferior vena cava was wide open. Pressure determinations revealed the following: superior vena cava 24 mm. Hg, inferior vena cava 6 mm. Hg, and left atrium 6 mm. Hg mean. At reoperation in January of 1974, a thick pseudointimal pannus again was found on both the pulmonary and systemic sides of the Dacron baffle. The baffle again was completely excised and replaced with pericardium. The patient has continued to have an uneventful recovery.
Technique The cannulation technique for institution of cardiopulmonary bypass in the Albert or Mustard procedures for correction of transposition of the great arteries presents some interesting possibilities. The arterial cannulation in all these cases was via the ascending aorta. A straight Bardic catheter, secured to the ascending aorta as well as to the sternal retractor, was used. There are several possibilities for venous return cannulation. At present the cannulation technique for the primary procedure differs from the technique employed should a reoperation be necessary. For the initial operation the caval cannulas are inserted via the right atrium and secured with pursestring sutures. After the institution of cardio-
Fig. 3. Patient K. K. Lateral view of superior vena cavagram reveals high-grade stenosis at the superior cava-atrial junction.
pulmonary bypass, occlusive snares are applied to both cannulas at the cava-atrial junctions. The right atriotomy is placed in such a fashion as to divide the previously placed atrial purse-string sutures and to allow the cannulas to be retracted to the superior and inferior ends of the atriotomy, respectively (Fig. 4). After excision of the atrial septum, the atrial baffle is sutured into position in the routine fashion, thus diverting the vena caval return to the posterior ventricle via the mitral valve (Fig. 5). The atrial baffle is sutured up to the sites of exit of the caval
The Journal of
866
Grehl and Shumway
Thoracic and Cardiovascular Surgery
Fig. 4. Routine caval cannulation technique via the right atrium depicts position of the right atrial incision with retraction of the superior and inferior caval cannulas to their respective ends of the incision.
Fig. 5. Position of the intra-atrial baffle that diverts vena caval flow to the posterior ventricle.
cannulas from either end of the right atriotomy. A pericardial patch is then used to enlarge the right atrial chamber during the closure of the right atriotomy (Fig. 6). At the cessation of cardiopulmonary bypass, the caval cannulation sites are closed in routine fashion with purse-string sutures. During reoperation for revision of the atrial baffle, the venous cannulation technique is altered. The innominate vein is dissected free from the left internal jugular vein
to the superior vena cava. The superior vena caval cannula is inserted via a longitudinal incision in the innominate vein and advanced into the superior vena cava after the distal innominate vein has been occluded in an atraumatic fashion. The inferior vena caval cannula is inserted via the left atrial appendage and advanced into the inferior vena cava through the tunnel created by the previously placed atrial baffle (Fig. 7) . Should the obstruction at the inferior vena
Volume 67 Number 6
Transposition of great arteries
867
June, 1974
Fig. 6. Closure of the right atriotomy with a pericardial patch prior to removal of caval cannulas.
cava not allow passage of the cannula initially, the patient is placed on cardiopulmonary bypass and the cannula is repositioned after incision of the dysfunctional atrial baffle. Again, occlusive snares are applied to the superior and inferior venae cavae at the cava-atrial junctions. At the completion of the procedure, the incision in the innominate vein is carefully repaired with 6-0 Prolene to reconstitute the normal venous return into the superior vena cava. The left atrial cannulation site is simply closed with a purse-string suture. With these techniques, cervical and inguinal cannulation sites have been avoided. This has allowed the use of larger diameter arterial and venous cannulas as well as diminution of the potential cross examination of the sternotomy incision. Cardiopulmonary bypass is carried out with disposable bubble oxygenators, moderate hypothermia, and flow rates in the range of 80 c.c. per kilogram of body weight per minute. There have been no adverse neurologic or renal complications. During the postoperative period, the patients are ventilated with pressure-regulated ventilators until their pulmonary status is satisfactory. They are then extubated and placed in mist tents for further convalescence.
Fig. 7. Cannulation technique utilized for revision of intra-atrial baffle. At the completion of the pro. cedure, the left atrial appendage is ligated and the incision in the innominate vein repaired to reconstitute normal venous flow.
Discussion
The current philosophy concerning correction of transposition of the great arteries at this medical center is dependent upon the presence or absence of a ventricular septal defect. In those child~en with a ventricular septal defect of adequate size, a modified Rastelli" procedure is carried out. A woven
The Journal of
868
Grehl and Shumway
Thoracic and Cardiovascular Surgery
Dacron tube containing a heterograft aortic valve reconstitutes normal pulmonary arterial blood flow after appropriate closure of the ventricular septal defect. In children with intact ventricular septa or small ventricular septal defects, correction is obtained by the intra-atrial baffle procedure. A small ventricular septal defect is closed through the tricuspid valve with care taken not to distort the now systemic atrioventricular valve. Our recent experience with the intra-atrial baffie using pericardium and knitted Dacron has led to us return to the use of pericardium in the initial repair. This material is certainly easy to use and readily available. In the event that revision of the baffie is necessary during the child's development, insertion of a prosthetic patch of some kind to enlarge the intra-atrial baffle is carried out. If pericardium is used in the first procedure, it is not necessary to remove the entire intraatrial baffle should a second procedure be necessary. In those patients with a Dacron baffle who require a second procedure, it has been necessary to remove the entire intra-atrial Dacron baffle. In this circumstance the second baffle is constructed entirely of pericardium.
It is of interest to note that patients with partial venous inflow occlusion following the Mustard procedure tolerate the second corrective procedure extremely well. The postoperative recovery in all these patients has progressed approximately twice as rapidly as after their first procedure. REFERENCES
2
3
4 5
Keith, J. D., Neill, C. A, Vlad, P., Rowe, R. D., and Chute, A. L.: Transposition of the Great Vessels, Circulation 7: 870, 1953. Ochsner, 1. L., Cooley, D. A, Harris, L. C., and McNamara, D. G.: Treatment of Complete Transposition of the Great Vessels With the Blalock-Hanlon Operation, Circulation 24: 51, 1961. Albert, H. M.: Surgical Correction of Transposition of the Great Vessels, Surg. Forum, 1954, American College of Surgeons, Philadelphia, 1955, W. B. Saunders Company, p. 74. Mustard, W. T.: Successful Two-Stage Correction of Transposition of the Great Vessels, Surgery 55: 469, 1964. Rastelli, G. C., Wallace, R. B., and Ongley, P. A: Complete Repair of Transposition of the Great Arteries With Pulmonary Stenosis, Circulation 39: 83, 1969.
Todd M. Grehl, M.D., and Norman E. Shumway, M.D., Stanford, Calif.
TransPosition of the great arteries has been considered the most common cause of death in infants with congenital heart disease.' Approximately half of these infants die within 1 month of birth and 86 per cent die within 6 months- without surgical intervention. The introduction of the intra-atrial baffle for diversion of venous return (Albert" or Mustard- procedure) to the heart for the correction of transposition of the great arteries has allowed these children to enjoy active lives. The longevity of these patients is still unknown. There have been problems with partial occlusion of venous inflow in patients submitted to correction by the intraatrial baffle technique. This report deals with reoperation for revision of the intraatrial baffle in the correction of transposition of the great arteries. From the Department of Cardiovascular Surgery, Stanford University School of Medicine, Stanford, Calif. 94305. Received for publication March I, 1974. Address for reprints: Todd M. Grehl, M.D., Department of Cardiovascular Surgery, Stanford University Medical Center, Stanford, Calif. 94305.
Case reports CASE l. C. W., a female child, was born following an uncomplicated pregnancy and delivery. She was noted to be cyanotic and in congestive heart failure at 2Y:z weeks of age. Cardiac catheterization at that time revealed transposition of the great arteries with an intact ventricular septum. A Blalock-Hanlon procedure was then carried out. She developed well over the next several years except for frequent respiratory infections. At the age of 7 years she developed a seizure disorder which was well controlled with diphenylhydantoin and phenobarbital. In November, 1972, at the age of 81/ 2 years, she underwent a Mustard procedure with a pericardial intra-atrial baffle. Four weeks postoperatively she developed the postpericardiotomy syndrome for which she was treated with aspirin. Six weeks postoperatively she was noted to have gained 2 kilograms over a I week period and to have developed ascites, peripheral edema, and tachycardia. Repeat cardiac catheterization in January, 1973, revealed partial severe inflow occlusion of the venae cavae at the cava-atrial junctions (Fig. 1). Pressure recordings revealed the following: superior vena cava 11 mm. Hg, inferior vena cava 18 mm. Hg, and left atrium 10 mm. Hg mean. At reoperation the pericardial baffle was thickened and shrunken, causing almost complete obstruc-
863
The Journal of
864
Grehl and Shumway
Thoracic and Cardiovascular Surgery
Fig. 1. Patient C. W. A, Anteroposterior view of superior vena cavagram shows dilation of the superior vena cava and narrowing of the intracardiac baffle. The inferior vena cavagram revealed only collateral venous return to the heart via the azygos system with complete occlusion at the inferior cava-atrial junction. B, Lateral view of superior vena cavagram shows narrowing and tortuosity at the superior cava-atrial junction.
tion of the inferior vena cava and partial obstruction of the superior vena cava. The pulmonary veins were widely patent. The baffle was incised longitudinally from cava to cava, and a knitted Dacron patch was used to enlarge the baffle and relieve the venous obstruction. The postoperative course was normal, and the child has continued to do well. CASE 2. D. c., a male child, was born following a normal delivery, but the pregnancy was complicated by a threatened abortion at 1 month. The patient was cyanotic at birth. Cardiac catheterization in January, 1971, when he was 1 month of age, revealed transposition of the great arteries, patent ductus arteriosus, and a small atrial septal defect. A Rashkind balloon atrial septostomy carried out at the time provided little improvement. The patient was therefore operated upon for ligation of the patent ductus arteriosus and performance of a Blalock-Hanlon atrial septectomy. In May, 1973, when he was 16 months old, a Mustard procedure was carried out with knitted Dacron used for an intra-atrial baffle. The patient did well postoperatively until December, 1973, when he began to develop mild edema of the legs. Repeat cardiac catheterization revealed slight superior vena cava obstruction and high-grade inferior vena cava obstruction (Fig. 2). Pressure
measurements revealed the following: superior vena cava 8 mm. Hg, inferior vena cava 12 mm. Hg, and left atrium 10 mm. Hg mean. Reoperation in December of 1973, when the child was 2 years old, revealed a thick pseudointimal pannus on both sides of the Dacron baffle. The baffle was completely excised along with all previously placed suture material. Sufficient pericardium could be dissected with which to fashion a new baffle. He has continued to do well postoperatively. CASE 3. Patient K. K. Following an uncomplicated labor and delivery, this male child was noted to be cyanotic and in respiratory distress. Cardiac catheterization at 1 day of age revealed transposition of the great arteries, small atrial septal defect, small ventricular septal defect, and a small patent ductus arteriosus. A Rashkind balloon atrial septostomy was carried out at that time. He developed satisfactorily although he became tachypneic and cyanotic on exertion. Repeat cardiac catheterization in April, 1972, confirmed the diagnosis of transposition of the great arteries and atrial septal defect, but there was no residual ventricular septal defect or patent ductus arteriosus. In February, 1973, when he was 2Y<1 years old, total correction was carried out with a knitted Dacron atrial baffle. The patient did well for 6
Volume 67 Number 6 June, 1974
Transposition oi great arteries
865
Fig. 2. A, Inferior vena cavagrarn reveals marked stenosis at the inferior cava-atrial junction in the anteroposterior projection. B, Dilated inferior vena cava with narrowing of intra-atrial chamber in the right anterior oblique position. months until October, 1973, when he was noted to develop mild facial and arm edema after being recumbent. Repeat cardiac catheterization at that time revealed high-grade obstruction of the superior vena cava (Fig. 3). The inferior vena cava was wide open. Pressure determinations revealed the following: superior vena cava 24 mm. Hg, inferior vena cava 6 mm. Hg, and left atrium 6 mm. Hg mean. At reoperation in January of 1974, a thick pseudointimal pannus again was found on both the pulmonary and systemic sides of the Dacron baffle. The baffle again was completely excised and replaced with pericardium. The patient has continued to have an uneventful recovery.
Technique The cannulation technique for institution of cardiopulmonary bypass in the Albert or Mustard procedures for correction of transposition of the great arteries presents some interesting possibilities. The arterial cannulation in all these cases was via the ascending aorta. A straight Bardic catheter, secured to the ascending aorta as well as to the sternal retractor, was used. There are several possibilities for venous return cannulation. At present the cannulation technique for the primary procedure differs from the technique employed should a reoperation be necessary. For the initial operation the caval cannulas are inserted via the right atrium and secured with pursestring sutures. After the institution of cardio-
Fig. 3. Patient K. K. Lateral view of superior vena cavagram reveals high-grade stenosis at the superior cava-atrial junction.
pulmonary bypass, occlusive snares are applied to both cannulas at the cava-atrial junctions. The right atriotomy is placed in such a fashion as to divide the previously placed atrial purse-string sutures and to allow the cannulas to be retracted to the superior and inferior ends of the atriotomy, respectively (Fig. 4). After excision of the atrial septum, the atrial baffle is sutured into position in the routine fashion, thus diverting the vena caval return to the posterior ventricle via the mitral valve (Fig. 5). The atrial baffle is sutured up to the sites of exit of the caval
The Journal of
866
Grehl and Shumway
Thoracic and Cardiovascular Surgery
Fig. 4. Routine caval cannulation technique via the right atrium depicts position of the right atrial incision with retraction of the superior and inferior caval cannulas to their respective ends of the incision.
Fig. 5. Position of the intra-atrial baffle that diverts vena caval flow to the posterior ventricle.
cannulas from either end of the right atriotomy. A pericardial patch is then used to enlarge the right atrial chamber during the closure of the right atriotomy (Fig. 6). At the cessation of cardiopulmonary bypass, the caval cannulation sites are closed in routine fashion with purse-string sutures. During reoperation for revision of the atrial baffle, the venous cannulation technique is altered. The innominate vein is dissected free from the left internal jugular vein
to the superior vena cava. The superior vena caval cannula is inserted via a longitudinal incision in the innominate vein and advanced into the superior vena cava after the distal innominate vein has been occluded in an atraumatic fashion. The inferior vena caval cannula is inserted via the left atrial appendage and advanced into the inferior vena cava through the tunnel created by the previously placed atrial baffle (Fig. 7) . Should the obstruction at the inferior vena
Volume 67 Number 6
Transposition of great arteries
867
June, 1974
Fig. 6. Closure of the right atriotomy with a pericardial patch prior to removal of caval cannulas.
cava not allow passage of the cannula initially, the patient is placed on cardiopulmonary bypass and the cannula is repositioned after incision of the dysfunctional atrial baffle. Again, occlusive snares are applied to the superior and inferior venae cavae at the cava-atrial junctions. At the completion of the procedure, the incision in the innominate vein is carefully repaired with 6-0 Prolene to reconstitute the normal venous return into the superior vena cava. The left atrial cannulation site is simply closed with a purse-string suture. With these techniques, cervical and inguinal cannulation sites have been avoided. This has allowed the use of larger diameter arterial and venous cannulas as well as diminution of the potential cross examination of the sternotomy incision. Cardiopulmonary bypass is carried out with disposable bubble oxygenators, moderate hypothermia, and flow rates in the range of 80 c.c. per kilogram of body weight per minute. There have been no adverse neurologic or renal complications. During the postoperative period, the patients are ventilated with pressure-regulated ventilators until their pulmonary status is satisfactory. They are then extubated and placed in mist tents for further convalescence.
Fig. 7. Cannulation technique utilized for revision of intra-atrial baffle. At the completion of the pro. cedure, the left atrial appendage is ligated and the incision in the innominate vein repaired to reconstitute normal venous flow.
Discussion
The current philosophy concerning correction of transposition of the great arteries at this medical center is dependent upon the presence or absence of a ventricular septal defect. In those child~en with a ventricular septal defect of adequate size, a modified Rastelli" procedure is carried out. A woven
The Journal of
868
Grehl and Shumway
Thoracic and Cardiovascular Surgery
Dacron tube containing a heterograft aortic valve reconstitutes normal pulmonary arterial blood flow after appropriate closure of the ventricular septal defect. In children with intact ventricular septa or small ventricular septal defects, correction is obtained by the intra-atrial baffle procedure. A small ventricular septal defect is closed through the tricuspid valve with care taken not to distort the now systemic atrioventricular valve. Our recent experience with the intra-atrial baffie using pericardium and knitted Dacron has led to us return to the use of pericardium in the initial repair. This material is certainly easy to use and readily available. In the event that revision of the baffie is necessary during the child's development, insertion of a prosthetic patch of some kind to enlarge the intra-atrial baffle is carried out. If pericardium is used in the first procedure, it is not necessary to remove the entire intraatrial baffle should a second procedure be necessary. In those patients with a Dacron baffle who require a second procedure, it has been necessary to remove the entire intra-atrial Dacron baffle. In this circumstance the second baffle is constructed entirely of pericardium.
It is of interest to note that patients with partial venous inflow occlusion following the Mustard procedure tolerate the second corrective procedure extremely well. The postoperative recovery in all these patients has progressed approximately twice as rapidly as after their first procedure. REFERENCES
2
3
4 5
Keith, J. D., Neill, C. A, Vlad, P., Rowe, R. D., and Chute, A. L.: Transposition of the Great Vessels, Circulation 7: 870, 1953. Ochsner, 1. L., Cooley, D. A, Harris, L. C., and McNamara, D. G.: Treatment of Complete Transposition of the Great Vessels With the Blalock-Hanlon Operation, Circulation 24: 51, 1961. Albert, H. M.: Surgical Correction of Transposition of the Great Vessels, Surg. Forum, 1954, American College of Surgeons, Philadelphia, 1955, W. B. Saunders Company, p. 74. Mustard, W. T.: Successful Two-Stage Correction of Transposition of the Great Vessels, Surgery 55: 469, 1964. Rastelli, G. C., Wallace, R. B., and Ongley, P. A: Complete Repair of Transposition of the Great Arteries With Pulmonary Stenosis, Circulation 39: 83, 1969.