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Surgical correction of the transposition complex in infancy
Surgical correction of the transposition complex in infancy James W. Kilman, M.D., * Thomas E. Williams, Ir., M.D. * (by invitation), Gerard S. Kakos, M.D. * (by invitation), Josepha Craenen, M.D. ** (by invitation),
and Don M. Hosier, M.D. ** (by invitation), Columbus, Ohio
The procedure for transposition of the great vessels proposed by Mustard" in 1964 has proved to be a good hemodynamic operation for this' defect and can be done with a reasonable mortality rate. However, there are some problems concerning this anomaly which are still not resolved: (1) At what age should the corrective procedure be done? (2) How should the correction of accompanying defects be handled? (3) Should pericardium or artificial material be used for the baffle? (4) Should the "new" left atrium be enlarged? (5) If the operation is done in infancy, should high-flow normothermia bypass be used or should hypothermia and circulatory arrest be used? At The Columbus Children's Hospital, the Ohio State University College of Medicine, we have attempted to formulate our own answers to some of these questions during From The Division of Thoracic Surgery, Department of Surgery, and The Division of Pediatric Cardiology, Department of Pediatrics, Ohio State University College of Medicine, 410 West 10th Ave., Columbus, Ohio 43210. Read at the Fifty-third Annual Meeting of The American Association for Thoracic Surgery, Dallas, Texas, April 16, 17, and 18, 1973. Address for reprints: James W. Kilman, M.D., 410 West 10th Ave., Columbus, Ohio 43210. 'Division of Thoracic Surgery, Department of Surgery. "Division of Pediatric Cardiology, Department of Pediatrics.
the course of the total correction in our first 25 infants with transposition of the great vessels. Clinical material
Of the 25 patients in this series, the youngest was 3 months and the oldest was 24 months of age; 8 patients were younger than I year of age. Fifteen of the patients were boys and 10 were girls. The body weights ranged from 3 to 13 kilograms; 23 patients weighed 10 kilograms or below. The average body weight was 8.0 kilograms (Fig. I). Most of the children had a balloon atrial septostomy at I to 3 days of age (22 of 25), but 3 had required an atrial septostomy with inflow occlusion as neonates. Repeat balloon atrial septostomy was done for 4 patients. All of these secondary procedures were failures, and emergency total correction was then done at that time. Sixteen had uncomplicated transposition of the great vessels. Six patients had an additional ventricular septal defect. Two patients had pulmonary stenosis without ventricular septal defect, and 2 had the combination of ventricular septal defect and pulmonary stenosis in addition to transposition of the great vessels. Two patients had a small patent ductus arteriosus
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Fig. 1. Distribution of body weights shows that the majority of the patients in this series were 10 kilograms or under.
in addition to their primary anomaly (I of these patients also was among those with a small ventricular septal defect). Methods Anesthesia. Anesthesia is induced and maintained with halothane and succinylcholine. A Portex nasotracheal tube is put in place for intra- and postoperative ventilation. Small catheters are placed by cutdown into the left femoral artery and vein, and these are maintained during the postoperative period with a pressurized mini-drip system. Anesthesia is supervised by a constant group specializing in infant cardiac anesthesia. Operative techniques. Cardiopulmonary bypass at normothermia, a standard disposable infant oxygenator, * and high flow rates (average 220 c.c. per kilogram per minute) were used for all patients (Fig. 2). Fresh heparinized blood diluted by one third with lactated Ringer's solution is used for the prime. The ascending aorta is cannulated for arterial input and the cavae are cannulated at their junction with the right atrium. A sump catheter is placed in the anterior ventricle to decompress the heart and lessen the chance of air embolus. The heart is allowed to beat during the entire procedure except for periods during which the aorta is cross-clamped for exposure of ventricular septal defects or pulmonary stenosis. AT-shaped incision is *Temptrol, Bentley Laboratories, Inc., Santa Ana, Calif.
BODY
4 WEIG~T
8 12 16 IN KILOGRAMS
Fig. 2. The perfusion technique used in this series of infants consisted of normothermic high-flow cardiopulmonary bypass as indicated in this chart of flow rates.
made in the right atrium and is extended down between the right pulmonary veins. The interatrial septum is then excised. We are especially thorough in removing the upper half, at times cutting tissue out of the atrium and repairing it with interrupted sutures as needed. At this point the pulmonary artery is opened and any stenotic areas are removed. The exposure is facilitated by the use of a small nasal speculum and by cross-clamping the aorta. A pituitary rongeur is used to resect subvalvular muscular obstruction until an adequate outflow tract is produced. After any obstruction at the pulmonary valve level has been relieved, a 4 to 5 mm. Bakes dilator is passed into the posterior ventricle through the pulmonary arteriotomy and, with the aorta cross-clamped and the septal leaflet of the tricuspid valve retracted with a nerve hook, any ventricular septal defect can be located easily, prolapsed forward, and sutured in the anterior edge with ease. It must be emphasized that, in this series, no ventriculotomy was needed in any patient treated by means of the above technique (Fig. 3). The atrial baffle was fashioned from pericardium in all except 2 cases (both had previous open septostomies and the amount and quality of the available pericardium was thought to be inadequate). In both of these cases, stretch Dacron material was used. The baffle was sewn in place in the manner described by Bonchek" and Aberdeen,' except that no effort to sculpture
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the baffle was made. The "Aberdeen flap" was used in all cases to incorporate the coronary sinus into the new "right atrium.?" The new left atrium was enlarged in all cases with a large triangle of pericardium as described by Waldhausen' " (Fig. 4). One of the problems noted with highflow cardiopulmonary bypass in infants is abdominal distention (evidence of hepatomegaly and ascites) and facial edema (evidence of obstructive type cerebral edema). This is managed by giving furosemide as soon as cardiopulmonary bypass is slowed. This usually results in a prompt diuresis and relief of the edema. Postoperative management. The patients are ail supported with a Bennett MA-l ventilator for the first 12 to 18 hours. Those who do not bleed excessively are taken off the ventilator the next morning. With evidence of a good P0 2 and adequate tidal volume, the endotracheal tube is immediately removed and the patients are given vigorous pulmonary care by specially trained nurses in an infant cardiopulmonary intensive care unit. Removal of the endotracheal tube is emphasized since an endotracheal tube without positive ventilation in a small infant can be detrimental due to the high resistance of the tube, which leads to the development of microatelectasis. Tracheostomy has not been required in any of these patients. One problem noted early in the series was the increased frequency of convulsive episodes in these small infants following cardiopulmonary bypass. These generalized convulsions seemed to result in no evidence of permanent residual effects but were bothersome due to their frequency. These have been treated by the administration of diphenylhydantoin and a pharmacologic dose of steroid (Solu-Medrol*) immediately after bypass. Since the institution of this therapy, convulsive episodes in the infants have not been noted frequently . Temporary pacing wires are put in ail patients; these have been used mainly for 'The Upjohn Co .• Kal amazoo, M ich .
Fig. 3. A drawing of the method of exposure of the ventricular septal defect without ventriculotomy. A Bakes dil ator is introduced through the pulmonary artery to aid in placement of the sutures.
Fig. 4. A drawing demonstrating the method of enlargement of the new "left atrium" by a large triangular patch of pericardium.
slow nodal rhythms. Supraventricular arrhythmias have not been a major problem; most patients have been discharged with a normal sinus rhythm or well-controlled nodal rhythms. No instances of atrioventricular block have occurred in this series of patients. Elective pacing has been used to maintain the heart rate at a desirable rate, and these
The Journal of
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Thoracic and Cardiovascular Surgery
temporary wires have been removed about 6 to 7 days postoperatively. No complications of the pacing wires have been seen. We have elected to manage these patients in the immediate postoperative period by maintaining a high right atrial pressure (16 to 18 ern. H 2 0 ) . Blood is infused at the rate of 2 c.c. per kilogram per hour, and any chest tube output is replaced as well. This method of management is maintained for the first 24 hours or until bleeding ceases to be a problem. Fluid management has consisted of electrolyte solution at a rate of 800 c.c. per square meter. In general these patients tend to retain fluid, and frequent diuretic therapy is needed. Digitalis has been added early in the management of all patients with ventricular septal defects, and furosemide is given as indicated by the clinical course in the less complex transpositions of the great vessels. Results
There has been one death in the first 25 infants undergoing total correction of transposition of the great vessels in our series. This death occurred in a 19-monthold boy who had a long history of frequent difficulty with upper respiratory problems and increasing cyanosis. He had a large ventricular septal defect which was closed at the time of operation. His immediate postoperative course was uncomplicated, but subsequently he developed evidence of increasing fluid retention and congestive failure. This was managed successfully, but his continued upper respiratory problems required prolonged hospitalization. Approximately 7 weeks after the operation, he developed abdominal distention and at operation was found to have an infarction of the right colon. This was resected, but the child died of sepsis. At postmortem examination, we found evidence of intravascular coagulopathy which was probably the cause of death. The repair was intact except for some evidence of mural thrombus along the wall of the new left atrium. Using the new technique of exposure of
the ventricular septal defect with the aid of a Bakes common duct probe, we have been able to close all of the ventricular septal defects without a ventriculotomy. Three of the 6 patients had a large ventricular septal defect that required a patch prosthesis; the remaining three defects were located below the tricuspid valve annulus and required only interrupted sutures for closure. Pulmonary obstruction was valvular in I patient and required only a valvulotomy. Another patient had a subvalvular diaphragm which was easily removed at the time of exposure by the method described by Daicoff." In the two instances of subpulmonary muscular obstruction, it was possible to resect enough muscle to relieve the obstruction by using a pituitary rongeur introduced through the pulmonary valve. It is important to cross-clamp the aorta during this maneuver to allow as much exposure as possible during relaxation of the muscular obstruction. Five patients required re-exploration for postoperative bleeding. This complication resulted in no significant morbidity or mortality rates. Three patients with significant supraventricular tachycardia required early digitalis therapy; all responded to medical management. The remainder of the arrhythmias were slow nodal and sinus rhythms requiring elective pacing for an effective cardiac output in these small infants. One patient showed evidence of a cerebral embolus in the late postoperative period with hemiparesis. This patient's defect was repaired with stretch Dacron material. He recovered completely from this embolus and on last examination showed no residual neurological damage. Congestive heart failure with evidence of fluid retention has been a significant complication in this series of patients. We have elected to manage this problem with vigorous diuretic therapy early in the postoperative course along with fluid restriction. Digitalis therapy has been reserved until
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most of the danger of surgically induced atrioventricular block has passed. In most patients, the heart has reduced to normal size by posteroanterior chest film within the first 6 to 8 weeks after the operation. All survivors have been completely rehabilitated, with evidence of normal blood gases and acceleration of growth and development patterns. Discussion
Other authors': 6, 7 have introduced the concept of early total correction of transposition of the great vessels, and we have substantiated this concept with our own results. Waldhausen'> was among the first to note in his series of patients that correction prior to 1 year of age seemed to have the best results. Rashkind,": 10 from the same institution, emphasized that the complication rate during the waiting period between balloon atrial septostomy and total correction was extremely high. Early in the series, total surgical correction was indicated by severe cyanosis, growth retardation, and the inability of the patient to attain a body weight of 10 kilograms or above. It became obvious to us that earlier operation was indicated with any evidence of failure of the balloon atrial septostomy and deterioration of the good clinical course. This method of management became apparent when we had better results with the younger infants no matter how complicated their defect appeared. Therefore, in the latter part of the series elective total correction is done from 6 to 12 months of age or earlier if there is any evidence of failure of the balloon atrial septostomy. (Our youngest patient is 3 months of age and weighed 3 kilograms.) There is certainly no assurance in transposition of the great vessels that an intact ventricular septum, pulmonary stenosis, or banding. of the pulmonary artery will prevent the development of progressive pulmonary vascular changes.r- 14 Our experience in early banding of the pulmonary artery for large ventricular septal defect
supports this. It is possible that primary pulmonary hypertension may be another defect that can exist in concert with other congenital defects, and its prevention or prophylaxis by various surgical maneuvers is impossible. 11 We feel that exposure of all ventricular defects can be accomplished with our technique and that an incision in the systemic ventricle can be avoided. This improves the prognosis in the more complicated lesions and decreases the occurrence of systemic ventricular failure related to the ventricular incision for exposure of the ventricular septal defect. Exposure and resection of the obstructive muscular hypertrophy below the pulmonary valve has been possible in each of our cases but still remains a technical problem due to the limited accessibility of this area. Calibration of the effectiveness of resection should be done so that at least a 10 mm. Bakes dilator can be passed through this opening. This seems to be adequate for the 10 kilogram infant. It has been demonstrated that subpulmonic stenosis may develop even with an intact ventricular septum; this may be another reason for early total repair of transposition of the great vessels. Pericardium is the easiest material to work with for fashioning the baffle, but the availability of stretch Dacron has certain advantages. We have not had the problem of baffle retraction at this point and therefore have continued to use the autogenous material except in those patients with previous surgical septostomy in whom the quality and quantity of the pericardium was of some question. We have always enlarged the new left atrium with a large triangular pericardial patch so that a generous-sized baffle could be used. We have found no disadvantages with this method except that it adds some time to the total procedure. Perhaps this technique, along with an extra-generous intra-atrial baffle, explains the absence of the problem of graft retraction noted in our small series of patients." We have chosen to use a simple technique
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for cardiopulmonary bypass, emphasizing high-flow normothermia with a standard pediatric bubble oxygenator. We carefully monitor urinary output during bypass and use our findings as a guide to effective flow. Flows have averaged 220 c.c. per kilogram per minute in this series of infants, with flow rates inversely proportionate to the body weights. Special arterial and venous cannulas developed for infant bypass have been used but, other than this, the technique is similar to adult techniques. The only problem we have uniformly noted is venous fluid retention due to the high flow, but this can be handled nicely by the administration of a rapid-acting diuretic such as furosemide immediately before cessation of cardiopulmonary bypass. We feel that both our results with infant bypass and the technical exposure are adequate; therefore, we have had no reason to change to total body hypothermia and circulatory arrest for infants. Infant cardiopulmonary bypass requires more attention to small details but should have good results if done with highflow, normothermic techniques.
Summary Transposition of the great vessels was totally corrected in 25 infants with a mortality rate of 4 per cent. High-flow, normothermic cardiopulmonary bypass was used with minimal complications. Correction of accompanying ventricular septal defects was possible in all eight instances by means of a new technique for exposure without ventriculotomy. The "new" systemic atrium was enlarged with a pericardial patch in each instance, and no retraction of the baffle has been noted. We have found that transposition of the great vessels can be corrected successfully in infancy by means of our techniques; therefore, we advocate elective total repair prior to I year of age and emergency total repair at any time that the primary balloon atrial septostomy seems to be inadequate for good growth and development or that the clinical course deteriorates.
REFERENCES Aberdeen, E., Waterston, D. J., Carr, I., Graham, G., Bonham-Carter, R. E., and Subramanian, S.: Successful Correction of Transposed Great Arteries by Mustard's Operation, Lancet 1: 1233, 1965. 2 Baker, F., Baker, L., Zoltun, R., and Zuberbuhler, J. R.: Effectiveness of the Rashkind Procedure in Transposition of the Great Arteries in Infants. Circulation 43: I, 1971 (Suppl. I) (Discussion by Rashkind). 3 Bonchek, L. I., and Starr, A.: Total Correction of Transposition of the Great Arteries in Infancy as Initial Surgical Management, Ann. Surg. 14: 376, 1972. 4 Ching, E., DuShane, J. W., McGoon, D. C., and Danielson, G. K.: Total Correction of Cardiac Anomalies in Infancy Using Extracorporeal Circulation, J. THORAC. CARDIOVASC. SURG. 62: 117, 1971. 5 Daicoff, G. R., Schiebler, G. L., Elliott, L. P., Van Mierop, L. H. S., Bartley, T. D., Gessner, I. H., and Wheat, M. W.: Surgical Repair of Complete Transposition of the Great Arteries With Pulmonary Stenosis, Ann. Thorac. Surg. 7: 529, 1969. 6 Indeglia, R. A., Moller, J. H., Lucas, R. V., and Castaneda, A. R.: Treatment of Transposition of the Great Vessels With an Intra-atrial Baffle (Mustard Procedure), Arch. Surg. 101: 797, 1970. 7 Lindesmith, G. G., Stiles, Q. R., Tucker, B. L., GaIlaher, M. E., Stanton, R. E., and Meyer, B. W.: The Mustard Operation as a Palliative Procedure, J. THORAC. CARDIOVASC. SURG. 63: 75, 1972. 8 Morgan, J. R., Miller, B. L., Daicoff, G. R, and Andrews, E. J.: Hemodynamic and Angiocardiographic Evaluation After Mustard Procedure for Transposition of the Great Arteries, J. THORAC. CARDIOVASC. SURG. 64: 878, 1972. 9 Mustard, W. T.: Successful Two-Stage Correction of Transposition of the Great Vessels, Surgery 55: 469, 1964. 10 Parsons, C. G., Astley, R., Burrows, F. G. 0., and Sigh, S. P.: Transposition of the Great Arteries: A Study of 65 Infants Followed for I to 4 Years After Balloon Septostomy, Br. Heart J. 33: 725, 1971. II Plauth, W. H., Nadas, A. S., Bernhard, W. F., and Fyler, D. C.: Changing Hemodynamics in Patients With Transposition of the Great Arteries, Circulation 42: 131, 1970. 12 Stark, J., Tynan, M. J., Ashcraft, K. W., Aberdeen, E., and Waterston, D. J.: Obstruction of Pulmonary Veins and Superior Vena Cava After Mustard Operation for Transposition of the Great Arteries (Abstr.) Circulation 43, 44: 91, 1971 (Suppl. 11).
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I3 Tynan, M., and Aberdeen, E.: Tricuspid Incompetence Following the Mustard Operation for Transposition of the Great Arteries (Abstr.) Circulation 43, 44: 92, 1971 (Suppl, 11). 14 Viles, P. H., Ongley, P. A., and Titus, J. L.: The Spectrum of Pulmonary Vascular Disease in Transposition of the Great Arteries, Circulation 40: 31, 1969. 15 Waldhausen, J. A., Pierce, W. A., Park, C. D., Rashkind, W. J., and Friedman, S.: Physiologic Correction of Transposition of the Great Arteries, Circulation 43: 738, 1971.
Discussion DR. J. ALEX HALLER Baltimore, Md.
I rise to ask two questions of Dr. Kilman regarding patient management. We have seen a distressingly frequent problem of right-to-left shunting in the immediate postoperative period in children with Mustard repairs. They often have considerable cyanosis and unsaturation for several days. We have been unable to ascertain whether this represents pulmonary shunting or intracardiac shunting. I wonder if you have seen this problem and, if so, if you have done anything specific to identify the level of the shunt? Most of the shunting disappeared over the next 3 or 4 days with positive-pressure respirator support. My second question has to do with your handling of the coronary sinus. I am not sure whether your modification of the Mustard repair means that you leave the sinus in the pulmonary venous atrium rather than transposing it? If you do transpose it, how are you currently doing this, and do you feel that this is an important part of the repair? DR. JAMES R. MALM New York, N. Y.
I rise to comment not specifically on the management of transposition but rather on Dr. Kilman's comment about the complication of seizure activity in these patients. We have been particularly interested in his group because he used a high-flow normothermia
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technique which was the one we utilized at Columbia for the past 4 years. In a group of 50 infants under 6 months of age who have been perfused by this technique, only I has experienced seizure activity. This specifically was a patient who had a cardiac arrest and was intubated before being transferred to our hospital. The fact that, in Dr. Kilman's series, the incidence of seizure was high with the high-flow technique may not be specifically related to the technique but rather to some other factor-something in the perfusion, the lack of an adequate arterial filter, or some problem with air embolus. It is common knowledge that air embolus in transposition can occur quite easily unless that anterior ventricle is vented throughout the procedure until all the air is evacuated from the newly reconstructed atrium. DR. KILMAN (Closing) We have seen cases in which cyanosis persisted in the immediate postoperative period but disappeared after management on the ventilator for 3 or 4 days. Most patients with simple transposition of the great vessels have excellent oxygen tensions and can be weaned from the ventilators by the next morning. In the past few years, we have followed a very vigorous policy in removing the endotracheal tube from infants, electing to take them off the ventilators almost immediately the next morning. Their oxygen tensions always seem to go up immediately after this, and their status continues to improve. We have used the Aberdeen flap technique for the coronary sinus in our patients and have modified it at times, especially in patients whose pulmonary venous return is to the coronary sinus. In general, we try to keep the flap in the venous side. In regard to central nervous system symptoms, I remember that the time our problems with convulsions in the postoperative period ceased was about the time we started using the new arterial filters. Prior to that time we had no arterial filters in our lines because that had been our procedure with adults. When the new filters became available, we did take advantage of them and perhaps it was this rather than the administration of drugs that cured our problems.
and Don M. Hosier, M.D. ** (by invitation), Columbus, Ohio
The procedure for transposition of the great vessels proposed by Mustard" in 1964 has proved to be a good hemodynamic operation for this' defect and can be done with a reasonable mortality rate. However, there are some problems concerning this anomaly which are still not resolved: (1) At what age should the corrective procedure be done? (2) How should the correction of accompanying defects be handled? (3) Should pericardium or artificial material be used for the baffle? (4) Should the "new" left atrium be enlarged? (5) If the operation is done in infancy, should high-flow normothermia bypass be used or should hypothermia and circulatory arrest be used? At The Columbus Children's Hospital, the Ohio State University College of Medicine, we have attempted to formulate our own answers to some of these questions during From The Division of Thoracic Surgery, Department of Surgery, and The Division of Pediatric Cardiology, Department of Pediatrics, Ohio State University College of Medicine, 410 West 10th Ave., Columbus, Ohio 43210. Read at the Fifty-third Annual Meeting of The American Association for Thoracic Surgery, Dallas, Texas, April 16, 17, and 18, 1973. Address for reprints: James W. Kilman, M.D., 410 West 10th Ave., Columbus, Ohio 43210. 'Division of Thoracic Surgery, Department of Surgery. "Division of Pediatric Cardiology, Department of Pediatrics.
the course of the total correction in our first 25 infants with transposition of the great vessels. Clinical material
Of the 25 patients in this series, the youngest was 3 months and the oldest was 24 months of age; 8 patients were younger than I year of age. Fifteen of the patients were boys and 10 were girls. The body weights ranged from 3 to 13 kilograms; 23 patients weighed 10 kilograms or below. The average body weight was 8.0 kilograms (Fig. I). Most of the children had a balloon atrial septostomy at I to 3 days of age (22 of 25), but 3 had required an atrial septostomy with inflow occlusion as neonates. Repeat balloon atrial septostomy was done for 4 patients. All of these secondary procedures were failures, and emergency total correction was then done at that time. Sixteen had uncomplicated transposition of the great vessels. Six patients had an additional ventricular septal defect. Two patients had pulmonary stenosis without ventricular septal defect, and 2 had the combination of ventricular septal defect and pulmonary stenosis in addition to transposition of the great vessels. Two patients had a small patent ductus arteriosus
387
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Fig. 1. Distribution of body weights shows that the majority of the patients in this series were 10 kilograms or under.
in addition to their primary anomaly (I of these patients also was among those with a small ventricular septal defect). Methods Anesthesia. Anesthesia is induced and maintained with halothane and succinylcholine. A Portex nasotracheal tube is put in place for intra- and postoperative ventilation. Small catheters are placed by cutdown into the left femoral artery and vein, and these are maintained during the postoperative period with a pressurized mini-drip system. Anesthesia is supervised by a constant group specializing in infant cardiac anesthesia. Operative techniques. Cardiopulmonary bypass at normothermia, a standard disposable infant oxygenator, * and high flow rates (average 220 c.c. per kilogram per minute) were used for all patients (Fig. 2). Fresh heparinized blood diluted by one third with lactated Ringer's solution is used for the prime. The ascending aorta is cannulated for arterial input and the cavae are cannulated at their junction with the right atrium. A sump catheter is placed in the anterior ventricle to decompress the heart and lessen the chance of air embolus. The heart is allowed to beat during the entire procedure except for periods during which the aorta is cross-clamped for exposure of ventricular septal defects or pulmonary stenosis. AT-shaped incision is *Temptrol, Bentley Laboratories, Inc., Santa Ana, Calif.
BODY
4 WEIG~T
8 12 16 IN KILOGRAMS
Fig. 2. The perfusion technique used in this series of infants consisted of normothermic high-flow cardiopulmonary bypass as indicated in this chart of flow rates.
made in the right atrium and is extended down between the right pulmonary veins. The interatrial septum is then excised. We are especially thorough in removing the upper half, at times cutting tissue out of the atrium and repairing it with interrupted sutures as needed. At this point the pulmonary artery is opened and any stenotic areas are removed. The exposure is facilitated by the use of a small nasal speculum and by cross-clamping the aorta. A pituitary rongeur is used to resect subvalvular muscular obstruction until an adequate outflow tract is produced. After any obstruction at the pulmonary valve level has been relieved, a 4 to 5 mm. Bakes dilator is passed into the posterior ventricle through the pulmonary arteriotomy and, with the aorta cross-clamped and the septal leaflet of the tricuspid valve retracted with a nerve hook, any ventricular septal defect can be located easily, prolapsed forward, and sutured in the anterior edge with ease. It must be emphasized that, in this series, no ventriculotomy was needed in any patient treated by means of the above technique (Fig. 3). The atrial baffle was fashioned from pericardium in all except 2 cases (both had previous open septostomies and the amount and quality of the available pericardium was thought to be inadequate). In both of these cases, stretch Dacron material was used. The baffle was sewn in place in the manner described by Bonchek" and Aberdeen,' except that no effort to sculpture
Volume 66 Numbe r 3
Correction of transposition complex
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the baffle was made. The "Aberdeen flap" was used in all cases to incorporate the coronary sinus into the new "right atrium.?" The new left atrium was enlarged in all cases with a large triangle of pericardium as described by Waldhausen' " (Fig. 4). One of the problems noted with highflow cardiopulmonary bypass in infants is abdominal distention (evidence of hepatomegaly and ascites) and facial edema (evidence of obstructive type cerebral edema). This is managed by giving furosemide as soon as cardiopulmonary bypass is slowed. This usually results in a prompt diuresis and relief of the edema. Postoperative management. The patients are ail supported with a Bennett MA-l ventilator for the first 12 to 18 hours. Those who do not bleed excessively are taken off the ventilator the next morning. With evidence of a good P0 2 and adequate tidal volume, the endotracheal tube is immediately removed and the patients are given vigorous pulmonary care by specially trained nurses in an infant cardiopulmonary intensive care unit. Removal of the endotracheal tube is emphasized since an endotracheal tube without positive ventilation in a small infant can be detrimental due to the high resistance of the tube, which leads to the development of microatelectasis. Tracheostomy has not been required in any of these patients. One problem noted early in the series was the increased frequency of convulsive episodes in these small infants following cardiopulmonary bypass. These generalized convulsions seemed to result in no evidence of permanent residual effects but were bothersome due to their frequency. These have been treated by the administration of diphenylhydantoin and a pharmacologic dose of steroid (Solu-Medrol*) immediately after bypass. Since the institution of this therapy, convulsive episodes in the infants have not been noted frequently . Temporary pacing wires are put in ail patients; these have been used mainly for 'The Upjohn Co .• Kal amazoo, M ich .
Fig. 3. A drawing of the method of exposure of the ventricular septal defect without ventriculotomy. A Bakes dil ator is introduced through the pulmonary artery to aid in placement of the sutures.
Fig. 4. A drawing demonstrating the method of enlargement of the new "left atrium" by a large triangular patch of pericardium.
slow nodal rhythms. Supraventricular arrhythmias have not been a major problem; most patients have been discharged with a normal sinus rhythm or well-controlled nodal rhythms. No instances of atrioventricular block have occurred in this series of patients. Elective pacing has been used to maintain the heart rate at a desirable rate, and these
The Journal of
390
Kilman et at.
Thoracic and Cardiovascular Surgery
temporary wires have been removed about 6 to 7 days postoperatively. No complications of the pacing wires have been seen. We have elected to manage these patients in the immediate postoperative period by maintaining a high right atrial pressure (16 to 18 ern. H 2 0 ) . Blood is infused at the rate of 2 c.c. per kilogram per hour, and any chest tube output is replaced as well. This method of management is maintained for the first 24 hours or until bleeding ceases to be a problem. Fluid management has consisted of electrolyte solution at a rate of 800 c.c. per square meter. In general these patients tend to retain fluid, and frequent diuretic therapy is needed. Digitalis has been added early in the management of all patients with ventricular septal defects, and furosemide is given as indicated by the clinical course in the less complex transpositions of the great vessels. Results
There has been one death in the first 25 infants undergoing total correction of transposition of the great vessels in our series. This death occurred in a 19-monthold boy who had a long history of frequent difficulty with upper respiratory problems and increasing cyanosis. He had a large ventricular septal defect which was closed at the time of operation. His immediate postoperative course was uncomplicated, but subsequently he developed evidence of increasing fluid retention and congestive failure. This was managed successfully, but his continued upper respiratory problems required prolonged hospitalization. Approximately 7 weeks after the operation, he developed abdominal distention and at operation was found to have an infarction of the right colon. This was resected, but the child died of sepsis. At postmortem examination, we found evidence of intravascular coagulopathy which was probably the cause of death. The repair was intact except for some evidence of mural thrombus along the wall of the new left atrium. Using the new technique of exposure of
the ventricular septal defect with the aid of a Bakes common duct probe, we have been able to close all of the ventricular septal defects without a ventriculotomy. Three of the 6 patients had a large ventricular septal defect that required a patch prosthesis; the remaining three defects were located below the tricuspid valve annulus and required only interrupted sutures for closure. Pulmonary obstruction was valvular in I patient and required only a valvulotomy. Another patient had a subvalvular diaphragm which was easily removed at the time of exposure by the method described by Daicoff." In the two instances of subpulmonary muscular obstruction, it was possible to resect enough muscle to relieve the obstruction by using a pituitary rongeur introduced through the pulmonary valve. It is important to cross-clamp the aorta during this maneuver to allow as much exposure as possible during relaxation of the muscular obstruction. Five patients required re-exploration for postoperative bleeding. This complication resulted in no significant morbidity or mortality rates. Three patients with significant supraventricular tachycardia required early digitalis therapy; all responded to medical management. The remainder of the arrhythmias were slow nodal and sinus rhythms requiring elective pacing for an effective cardiac output in these small infants. One patient showed evidence of a cerebral embolus in the late postoperative period with hemiparesis. This patient's defect was repaired with stretch Dacron material. He recovered completely from this embolus and on last examination showed no residual neurological damage. Congestive heart failure with evidence of fluid retention has been a significant complication in this series of patients. We have elected to manage this problem with vigorous diuretic therapy early in the postoperative course along with fluid restriction. Digitalis therapy has been reserved until
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most of the danger of surgically induced atrioventricular block has passed. In most patients, the heart has reduced to normal size by posteroanterior chest film within the first 6 to 8 weeks after the operation. All survivors have been completely rehabilitated, with evidence of normal blood gases and acceleration of growth and development patterns. Discussion
Other authors': 6, 7 have introduced the concept of early total correction of transposition of the great vessels, and we have substantiated this concept with our own results. Waldhausen'> was among the first to note in his series of patients that correction prior to 1 year of age seemed to have the best results. Rashkind,": 10 from the same institution, emphasized that the complication rate during the waiting period between balloon atrial septostomy and total correction was extremely high. Early in the series, total surgical correction was indicated by severe cyanosis, growth retardation, and the inability of the patient to attain a body weight of 10 kilograms or above. It became obvious to us that earlier operation was indicated with any evidence of failure of the balloon atrial septostomy and deterioration of the good clinical course. This method of management became apparent when we had better results with the younger infants no matter how complicated their defect appeared. Therefore, in the latter part of the series elective total correction is done from 6 to 12 months of age or earlier if there is any evidence of failure of the balloon atrial septostomy. (Our youngest patient is 3 months of age and weighed 3 kilograms.) There is certainly no assurance in transposition of the great vessels that an intact ventricular septum, pulmonary stenosis, or banding. of the pulmonary artery will prevent the development of progressive pulmonary vascular changes.r- 14 Our experience in early banding of the pulmonary artery for large ventricular septal defect
supports this. It is possible that primary pulmonary hypertension may be another defect that can exist in concert with other congenital defects, and its prevention or prophylaxis by various surgical maneuvers is impossible. 11 We feel that exposure of all ventricular defects can be accomplished with our technique and that an incision in the systemic ventricle can be avoided. This improves the prognosis in the more complicated lesions and decreases the occurrence of systemic ventricular failure related to the ventricular incision for exposure of the ventricular septal defect. Exposure and resection of the obstructive muscular hypertrophy below the pulmonary valve has been possible in each of our cases but still remains a technical problem due to the limited accessibility of this area. Calibration of the effectiveness of resection should be done so that at least a 10 mm. Bakes dilator can be passed through this opening. This seems to be adequate for the 10 kilogram infant. It has been demonstrated that subpulmonic stenosis may develop even with an intact ventricular septum; this may be another reason for early total repair of transposition of the great vessels. Pericardium is the easiest material to work with for fashioning the baffle, but the availability of stretch Dacron has certain advantages. We have not had the problem of baffle retraction at this point and therefore have continued to use the autogenous material except in those patients with previous surgical septostomy in whom the quality and quantity of the pericardium was of some question. We have always enlarged the new left atrium with a large triangular pericardial patch so that a generous-sized baffle could be used. We have found no disadvantages with this method except that it adds some time to the total procedure. Perhaps this technique, along with an extra-generous intra-atrial baffle, explains the absence of the problem of graft retraction noted in our small series of patients." We have chosen to use a simple technique
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for cardiopulmonary bypass, emphasizing high-flow normothermia with a standard pediatric bubble oxygenator. We carefully monitor urinary output during bypass and use our findings as a guide to effective flow. Flows have averaged 220 c.c. per kilogram per minute in this series of infants, with flow rates inversely proportionate to the body weights. Special arterial and venous cannulas developed for infant bypass have been used but, other than this, the technique is similar to adult techniques. The only problem we have uniformly noted is venous fluid retention due to the high flow, but this can be handled nicely by the administration of a rapid-acting diuretic such as furosemide immediately before cessation of cardiopulmonary bypass. We feel that both our results with infant bypass and the technical exposure are adequate; therefore, we have had no reason to change to total body hypothermia and circulatory arrest for infants. Infant cardiopulmonary bypass requires more attention to small details but should have good results if done with highflow, normothermic techniques.
Summary Transposition of the great vessels was totally corrected in 25 infants with a mortality rate of 4 per cent. High-flow, normothermic cardiopulmonary bypass was used with minimal complications. Correction of accompanying ventricular septal defects was possible in all eight instances by means of a new technique for exposure without ventriculotomy. The "new" systemic atrium was enlarged with a pericardial patch in each instance, and no retraction of the baffle has been noted. We have found that transposition of the great vessels can be corrected successfully in infancy by means of our techniques; therefore, we advocate elective total repair prior to I year of age and emergency total repair at any time that the primary balloon atrial septostomy seems to be inadequate for good growth and development or that the clinical course deteriorates.
REFERENCES Aberdeen, E., Waterston, D. J., Carr, I., Graham, G., Bonham-Carter, R. E., and Subramanian, S.: Successful Correction of Transposed Great Arteries by Mustard's Operation, Lancet 1: 1233, 1965. 2 Baker, F., Baker, L., Zoltun, R., and Zuberbuhler, J. R.: Effectiveness of the Rashkind Procedure in Transposition of the Great Arteries in Infants. Circulation 43: I, 1971 (Suppl. I) (Discussion by Rashkind). 3 Bonchek, L. I., and Starr, A.: Total Correction of Transposition of the Great Arteries in Infancy as Initial Surgical Management, Ann. Surg. 14: 376, 1972. 4 Ching, E., DuShane, J. W., McGoon, D. C., and Danielson, G. K.: Total Correction of Cardiac Anomalies in Infancy Using Extracorporeal Circulation, J. THORAC. CARDIOVASC. SURG. 62: 117, 1971. 5 Daicoff, G. R., Schiebler, G. L., Elliott, L. P., Van Mierop, L. H. S., Bartley, T. D., Gessner, I. H., and Wheat, M. W.: Surgical Repair of Complete Transposition of the Great Arteries With Pulmonary Stenosis, Ann. Thorac. Surg. 7: 529, 1969. 6 Indeglia, R. A., Moller, J. H., Lucas, R. V., and Castaneda, A. R.: Treatment of Transposition of the Great Vessels With an Intra-atrial Baffle (Mustard Procedure), Arch. Surg. 101: 797, 1970. 7 Lindesmith, G. G., Stiles, Q. R., Tucker, B. L., GaIlaher, M. E., Stanton, R. E., and Meyer, B. W.: The Mustard Operation as a Palliative Procedure, J. THORAC. CARDIOVASC. SURG. 63: 75, 1972. 8 Morgan, J. R., Miller, B. L., Daicoff, G. R, and Andrews, E. J.: Hemodynamic and Angiocardiographic Evaluation After Mustard Procedure for Transposition of the Great Arteries, J. THORAC. CARDIOVASC. SURG. 64: 878, 1972. 9 Mustard, W. T.: Successful Two-Stage Correction of Transposition of the Great Vessels, Surgery 55: 469, 1964. 10 Parsons, C. G., Astley, R., Burrows, F. G. 0., and Sigh, S. P.: Transposition of the Great Arteries: A Study of 65 Infants Followed for I to 4 Years After Balloon Septostomy, Br. Heart J. 33: 725, 1971. II Plauth, W. H., Nadas, A. S., Bernhard, W. F., and Fyler, D. C.: Changing Hemodynamics in Patients With Transposition of the Great Arteries, Circulation 42: 131, 1970. 12 Stark, J., Tynan, M. J., Ashcraft, K. W., Aberdeen, E., and Waterston, D. J.: Obstruction of Pulmonary Veins and Superior Vena Cava After Mustard Operation for Transposition of the Great Arteries (Abstr.) Circulation 43, 44: 91, 1971 (Suppl. 11).
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I3 Tynan, M., and Aberdeen, E.: Tricuspid Incompetence Following the Mustard Operation for Transposition of the Great Arteries (Abstr.) Circulation 43, 44: 92, 1971 (Suppl, 11). 14 Viles, P. H., Ongley, P. A., and Titus, J. L.: The Spectrum of Pulmonary Vascular Disease in Transposition of the Great Arteries, Circulation 40: 31, 1969. 15 Waldhausen, J. A., Pierce, W. A., Park, C. D., Rashkind, W. J., and Friedman, S.: Physiologic Correction of Transposition of the Great Arteries, Circulation 43: 738, 1971.
Discussion DR. J. ALEX HALLER Baltimore, Md.
I rise to ask two questions of Dr. Kilman regarding patient management. We have seen a distressingly frequent problem of right-to-left shunting in the immediate postoperative period in children with Mustard repairs. They often have considerable cyanosis and unsaturation for several days. We have been unable to ascertain whether this represents pulmonary shunting or intracardiac shunting. I wonder if you have seen this problem and, if so, if you have done anything specific to identify the level of the shunt? Most of the shunting disappeared over the next 3 or 4 days with positive-pressure respirator support. My second question has to do with your handling of the coronary sinus. I am not sure whether your modification of the Mustard repair means that you leave the sinus in the pulmonary venous atrium rather than transposing it? If you do transpose it, how are you currently doing this, and do you feel that this is an important part of the repair? DR. JAMES R. MALM New York, N. Y.
I rise to comment not specifically on the management of transposition but rather on Dr. Kilman's comment about the complication of seizure activity in these patients. We have been particularly interested in his group because he used a high-flow normothermia
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technique which was the one we utilized at Columbia for the past 4 years. In a group of 50 infants under 6 months of age who have been perfused by this technique, only I has experienced seizure activity. This specifically was a patient who had a cardiac arrest and was intubated before being transferred to our hospital. The fact that, in Dr. Kilman's series, the incidence of seizure was high with the high-flow technique may not be specifically related to the technique but rather to some other factor-something in the perfusion, the lack of an adequate arterial filter, or some problem with air embolus. It is common knowledge that air embolus in transposition can occur quite easily unless that anterior ventricle is vented throughout the procedure until all the air is evacuated from the newly reconstructed atrium. DR. KILMAN (Closing) We have seen cases in which cyanosis persisted in the immediate postoperative period but disappeared after management on the ventilator for 3 or 4 days. Most patients with simple transposition of the great vessels have excellent oxygen tensions and can be weaned from the ventilators by the next morning. In the past few years, we have followed a very vigorous policy in removing the endotracheal tube from infants, electing to take them off the ventilators almost immediately the next morning. Their oxygen tensions always seem to go up immediately after this, and their status continues to improve. We have used the Aberdeen flap technique for the coronary sinus in our patients and have modified it at times, especially in patients whose pulmonary venous return is to the coronary sinus. In general, we try to keep the flap in the venous side. In regard to central nervous system symptoms, I remember that the time our problems with convulsions in the postoperative period ceased was about the time we started using the new arterial filters. Prior to that time we had no arterial filters in our lines because that had been our procedure with adults. When the new filters became available, we did take advantage of them and perhaps it was this rather than the administration of drugs that cured our problems.