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Simple d-transposition of the great arteries
Simple d-transposition of the great arteries Results of early balloon septotomy followed by two-stage surgical correction The follow-up of 44 patients with simple d-transposition of the great arteries is presented. All had balloon atrial septotomy shortly after birth. If an operation was needed at or before one year of age, atrial septectomy was carried out. Correction was done between the ages of 19 and 64 months. Of the 44 patients, 34 (77 per cent) are living and doing well (27 after the Mustard procedure), 3 (7 per cent) are lost to follow-up, and 7 (16 per cent) are dead. Death was due to technical problems during initial catheterization and balloon septotomy in 2 patients, occurred after septectomy in one (3 per cent), and after the Mustard procedure in one (4 per cent). There was one sudden inexplicable death in a 1 Vz-month-old patient, and 2 patients died with pulmonary vascular obstructive disease. Because of the relatively low over-all mortality and morbidity and the low surgical mortality rate for both palliation and correction, we believe that two-stage surgical correction of transposition of the great arteries is preferable to early one-stage correction in the infant.
C. Kratz, A. Davignon, C. Chartrand, and P. Stanley, Montreal,
Quebec,
Canada
A he introduction of balloon atrial septotomy in 1966 by Rashkind and Miller 1 has greatly improved the outlook for children with transposition of the great arteries. This is especially true for children with simple transposition since, in the absence of a significant shunt, palliation is needed at a very young age. Also, if palliation is successful, it is in these patients that surgical correction carries a relatively low risk 2 and offers a good chance of normal postoperative cardiac function. Although balloon atrial septotomy is successful in the majority of patients, its benefits are transitory. In most cases, hypoxia recurs in a matter of weeks or even days or, at best, after several months. There is at present no general agreement as to the best management at this point of the evolution. 3 Correction according to the method developed by Mustard 4 is being done in some centers even during the first months of life, so that the additional risk of a palliative intervention is avoided. Mustard's procedure is then carried out with deep hypothermia and circulatory arrest. Early results reported so far seem encouraging, 12 but not much is
known about the late effects of deep hypothermia or about the relative frequency of pulmonary or caval vein stenosis if correction is done at a very young age. 2 The alternative to early correction is the two-stage operation which had been generally employed before the introduction of balloon septotomy, i.e., atrial septectomy during the first months of life followed by Mustard's procedure, which is done electively after one year of age and which is still performed in large centers. 3 This approach carries the risk of two surgical interventions and of morbidity between the two operations. However, the risk of a corrective operation and extracorporeal circulation seems less in children than in infants, and the problems of hypothermia are avoided. At Sainte-Justine Hospital of Montreal, patients with d-transposition of the great arteries have been treated by two-stage surgical correction with very few exceptions. We are presenting the results of surgical treatment and the clinical follow-up of our patients.
From the Departments of Cardiology and Cardiovascular Surgery, Hopital Ste-Justine pour les Enfants, Universite' de Montreal, Montre'al, Quebec, Canada. Received for publication Sept. 17, 1976. Accepted for publication Dec. 20, 1976.
Since the performance of our first Rashkind atrial septotomy in 1967, 44 patients with d-transposition of the great arteries have been treated at our hospital. None of the patients had a patent ductus or ventricular septal defect at the time of operation. We are present-
Patients
707
The Journal of Thoracic and Cardiovascular Surgery
7 0 8 Kratz et al.
Table I. Rashkind balloon septotomy: Postseptotomy evolution Immediate death Late death Lost to follow-up Follow-up
2 1 2 39
Legend: Forty-four patients are included, ranging in age from birth to 50 days (mean 7 days).
Table II. Surgical septectomy: Postoperative evolution in 36 patients* Immediate death Late death Doing well; awaiting correction After Mustard procedure
1(11 days old) 2 (PVOD) 7 26
Legend: PVOD, Pulmonary vascular obstructive disease. The age at operation was 4 days to 13 months in these patients (mean age 3'A months). They were operated upon 2 days to 13 months after the Rashkind procedure.
Table III. Operative results of Mustard procedure (survival rate 96 per cent) With prior septectomy* With prior septectomy Without prior septectomy
1 (no recent follow-up) 25 (1 postop. deathf) 3 (no deaths)
Legend: This group comprises 29 patients ranging in age from 19 to 64 months (mean 37 months). *Operated upon elsewhere. tRenal insufficiency and pancreatitis.
ing the follow-up of these patients who, with few exceptions, have been treated by two-stage surgical correction following a balloon septotomy performed shortly after birth. Rashkind septotomy. The age at the time of balloon septotomy varied from zero to 50 days, mean age 7 days. Forty-five balloon septotomies were carried out altogether, as the procedure was repeated in one patient at the age of 3'/2 months. In all but one patient who had severe acidosis and cardiac failure, diagnostic procedures preceded the septotomy. The ductus arteriosus was patent in 11 patients aged from birth to 30 days, and it shunted from the aorta to the pulmonary artery. A small ventricular septal defect was present in one patient. All of these lesions closed spontaneously prior to the first surgical intervention, as verified by the changes in auscultation or by exploration during the operation. Following the septotomy, there were two immediate deaths owing to technical problems, one caused by catheterization and the other caused by balloon septotomy. Another patient died suddenly at the age of IVi months of unknown causes. Two patients have been
lost to follow-up and the remaining 39 patients have been seen regularly (Table I). The status of all but 3 of the patients who survived was improved by the procedure. These 3 patients needed a surgical septectomy in the days that followed. The degree and duration of improvement in the others were variable. Recurrence of significant cyanosis and signs of hypoxia were indications for surgical intervention. As the patients usually were very young (average age 3V4 months) at this stage of the disease, we felt that the risk of palliative surgery was less than the risk of a Mustard procedure. Atrial septectomy. Of the 39 patients having Rashkind procedures, 36 have undergone atrial septectomy and 3 patients have had correction of the malformation at the age of 26, 31, and 37 months, respectively, without other palliation. Atrial septectomy was done at ages varying from 4 days to 13 months (mean age 3Vz months). The time lapse between balloon septotomy and surgical septectomy varied from 2 days to 13 months, average 3V£ months. The surgical technique for septectomy is as follows: The heart is approached through a median sternotomy and the septum is resected leisurely with a straight rongeur introduced through the right atrial appendage. This technique has been used since 1960 and has been satisfactory. The atrial septal defect as seen at the time of the Mustard procedure is large, over 2.5 cm., and the septal ridge between the right pulmonary veins and the right atrium is totally absent. Of the 36 patients who underwent surgical resection of the atrial septum, one died 12 hours after the operation. He was a severely hypoxic 11-day-old baby in whom septotomy had not reduced the hypoxia. All patients who survived were in markedly improved condition. There has been no postoperative arrhythmia. Of the 35 patients who survived, 2 developed pulmonary vascular obstructive disease which was discovered at the ages of 18 and 23 months, respectively, during catheterization in preparation for a planned Mustard procedure. One of these 2 patients was later found to have a neuroblastoma which disseminated rapidly. Both patients have died. Autopsy revealed pulmonary vascular obstructive disease of Grade 4 (Heath and Edwards) in both cases. Seven patients are doing well on medical treatment awaiting surgical correction. Twenty-six have already undergone the Mustard operation (Table II). Mustard operation. At present, this corrective operation is performed as soon as symptoms recur or elec-
Volume 73 Number 5 May, 1977
Simple d-transposition of great arteries
709
Table IV. Evaluation of cardiac rhythm after Mustard procedure in relation to surgical management of coronary sinus in 25 patients Rhythm at time of follow-up Group*
No. of patients
Sinus rhythm
I
8
II
9
III
8
3 patients (37%) 4 patients (44%) 6 patients (75%)
Intermittent sinus and nodal rhythm
Nodal rhythm (A V dissociation)
Ectopic atrial rhythm
3 patients
1 patient
1 patient
1 patient
3 patients
1 patient
1 patient
1 patient
Legend: AV, Atrioventricular. *Group I: The coronary sinus was incised from the ostium down along its upper border, with the coronary sinus blood draining into the "new right atrium." Group II: The coronary sinus was incised along its upper border in the left atrium; the ostium was not incised but sutured, and the coronary sinus blood drained into the "new right atrium." Group III: The coronary sinus was left intact, with the coronary sinus blood draining into the "new left atrium."
tively at the age of 2 to 3 years. The age at elective operation has changed. In earlier years, our indication for corrective surgery being recurrent symptoms only, 2 of our patients reached the age of 5 years before undergoing the Mustard procedure. Before surgical correction, repeat catheterization is being done in all patients. So far 31 patients have been recatheterized. Right atrial pressures were recorded in all 31 patients. Left atrial pressures were also obtained in 29, and no gradient was noted across the septum. Mean atrial pressures were significantly elevated (above 7 mm. Hg) in 6 patients. Right and left ventricular pressures were recorded in all 31 patients. Pulmonary artery pressures were obtained in 21. There'was no evidence of pulmonary hypertension except in 3. One 6-month-old infant had a pulmonary pressure of 50 mm. Hg. He was in mild heart failure at the time of catheterization, and verification of left ventricular pressure during the corrective procedure showed normal values (25 mm. Hg). Two patients mentioned previously had severe pulmonary hypertension, pulmonary artery pressure being equal to systemic pressure in one and higher than systemic pressure in the other. They were considered to be inoperable. There was a subpulmonary diaphragmatic stenosis with a left ventricle-pulmonary artery pressure gradient of 60 mm. Hg in one patient. Another patient had stenosis of the right branch of the pulmonary artery with a systolic pressure of 67 mm. Hg in the main pulmonary artery and a main pulmonary artery-right pulmonary artery gradient of 37 mm. Hg. To date, 29 of the patients have undergone the Mustard operation at ages varying from 19 months to 64 months, mean age 37 months (Table III). One of the
Table V. Over-all results in 44 patients Result
Cause
No.
Medical deaths Early: During Rashkind and catheterization Late: After Rashkind Late: After septectomy
5 2
Technical problems
1 2
Sudden death (1 Vi months) PVOD* (2 and 3 years)
Surgical deaths Early: After septectomy (3% mortality rate) Early: After Mustard (4% mortality rate)
2 1
Survivors Awaiting Mustard After Mustard Lost to follow-up
1
Poor result from Rashkind (11 days) Renal insufficiency and pancreatitis
34 7 27 3
*PVOD, Pulmonary vascular obstructive disease.
patients had the corrective procedure elsewhere, and we have no recent follow-up. Twenty-five patients were operated upon here following prior septectomy. There was one postoperative death owing to renal failure and pancreatitis. Three patients had the Mustard procedure without septectomy and are doing well. The corrective procedure was done with a pericardial baffle in 26 patients and a Dacron patch in two. The 27 survivors have been observed for periods varying from 3 months to 63 months, mean follow-up 30 months (Table III). Immediate postoperative arrhythmia was common. Only 2 patients continued to have permanent sinus rhythm after the operation. Heart failure of varying degrees occurred in most patients, but it disappeared in all within a few months.
The Journal of Thoracic and Cardiovascular Surgery
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Clinically, all 27 patients are asymptomatic. They are doing well and take part in normal activities. On physical examination, none of them shows signs of heart failure, although 2 patients have distended external jugular veins. The chest x-ray film of all of the patients shows a normal pulmonary vascularity indicating the absence of any significant pulmonary venous obstruction. Some degree of cardiomegaly persists in 17 of the 27 patients, which may well be related to the pericardiectomy. The 2 patients in whom obstruction of the superior vena cava was suspected clinically have been recatheterized. Angiograms showed the superior vena cava to be occluded in one and severely obstructed in the other. Late arrhythmia has been assessed with regard to a possible relationship between the frequency of ectopic rhythm and the surgical management of the coronary sinus as well as the method of cannulating the superior vena cava, these two factors being the only variants in surgical technique. The right atriotomy extended from the right atrial appendage downward to the origin of the right pulmonary veins, and the new left atrium was enlarged with a pericardial patch in all. Rhythms encountered were classified into four types. Type I is permanent sinus rhythm. We consider as sinus any rhythm with a normal PR interval and with normally directed P waves in the majority of the leads. Tracings which showed flattened isoelectric P waves in Lead I in the presence of normally directed P waves in the other standard leads are included. Type II is sinus rhythm alternating with nodal rhythm. Type III is permanent nodal rhythm. Nodal rhythm in all of our cases seems to be due to atrioventricular dissociation. Type IV is ectopic atrial rhythm with a normal, short, or prolonged PR interval and abnormal P wave configuration. In 25 of the 27 patients, the cavae were cannulated indirectly through the right atrial wall just anterior to the origin of the caval vessels. The coronary sinus was managed in various ways. These 25 patients were grouped as follows (Table IV): Group I. In 8 patients, the coronary sinus was incised from the ostium down along its upper border to drain the coronary sinus blood into the "new right atrium." Three patients on their last examination still had sinus rhythm, 3 had alternating sinus and nodal rhythm, one had continuous nodal rhythm, and one patient had an ectopic atrial rhythm with a short PR interval. Group II. In 9 patients, the coronary sinus was incised along its upper border in the left atrium, the ostium not being incised but sutured. Thus coronary sinus
blood again drained into the "new right atrium." Four patients when last seen were in sinus rhythm, one had alternating nodal and sinus rhythm, 3 had permanent nodal rhythm, and one had an ectopic atrial rhythm with a prolonged PR interval. Group III. In 8 patients, the coronary sinus was left intact, coronary sinus blood thus draining into the "new left atrium." Six of these 8 patients when last seen were in sinus rhythm, one had alternating nodal and sinus rhythm, and one had an ectopic atrial rhythm with a normal PR interval. Thus the incidence of ectopic rhythm is least frequent in the patients of the third group, in whom the coronary sinus was left intact. However, follow-up of this group has not been long enough to allow definitive conclusions, as ectopic rhythm has appeared as late as 3 years postoperatively in Groups I and II. In the last 2 patients operated upon, the superior vena cava was cannulated directly in its intrapericardial segment above the right atrium in order to avoid trauma to the sinus node, and the coronary sinus was untouched. Both have remained in sinus rhythm. Discussion The results show an over-all mortality rate of 16 per cent for the entire group of 44 patients with simple transposition of the great vessels treated in our hospital since 1967 (Table V). The surgical mortality rate is low both for surgical resection of the interatrial septum (3 per cent) and for total correction (4 per cent). The development of pulmonary vascular obstructive disease in 2 patients at the ages of 18 and 23 months, following the Rashkind procedure and the interatrial septectomy, is troublesome and raises the question of its prevention by early corrective surgery. The given data on this disease do not allow for any clear conclusion on this subject at present. Pulmonary vascular obstructive disease has, on rare occasions, appeared in transposition of the great arteries with intact ventricular septum after the Mustard procedure5-7 as well as before the operation.8-10 However it seems that the possibility of its development would be less likely if transposition were corrected before the child was 12 months of age. The low incidence of death and morbidity between the Rashkind procedure and total correction is most likely due to our liberal indications for surgical septal resection. Increasing cyanosis and hematocrit values rising above 60 per cent are indications for intervention even in the presence of good clinical status. After Mustard's procedure, all of our surviving patients are clinically well. However, postoperative ar-
Volume 73 Number 5 May, 1977
rhythmia is of concern. This seems related partly to surgical technique, and improvement in this line should help decrease the incidence of this complication. Avoidance of trauma to the coronary sinus has in our small group of patients diminished arrhythmias notably. Reducing trauma to the sinus node by direct cannulation of the superior vena cava above the right atrium should also reduce the chances of atrial arrhythmia. The 2 patients in whom the superior vena cava was cannulated directly would seem to bear out this point. However, it is not possible to draw conclusions on results obtained in 2 patients alone. The other postoperative problem is that of severe stenosis of the superior intra-auricular caval tunnel, which was encountered in 2 patients. This problem is related to surgical technique, and its occurrence should decrease with better evaluation of the surgical details involved in the construction of the superior auricular caval tunnel. The absence of pulmonary vein obstruction is most likely related to the orientation of the atriotomy, which in all of our patients extended downward from the atrial appendage to the right pulmonary veins, and to the enlargement of the new left atrium with a wide pericardial patch. Conclusion Because of the low mortality rate in these patients, we believe that two-stage surgical correction of transposition of the great arteries is preferable to one-stage surgical correction in the infants, as the risk of openheart surgery is greater in the patient under one year of age than in the young child.
Simple d-transposition
of great arteries
71 1
REFERENCES 1 Rashkind, W. J., and Miller, W. W.: Creation of an Atrial Septal Defect Without Thoracotomy: A Palliative Approach to Complete Transposition of the Great Arteries, J. A. M. A. 196: 991, 1966. 2 Stark, J., de Leval, M. R., Waterston, D. J., Graham, G. R., and Bonham-Carter, R. E.: Corrective Surgery of Transposition of the Great Arteries in the First Year of Life, J. THORAC. CARDIOVASC. SURG. 67: 673,
1974.
3 Gutgesell, H. P., and McNamara, D. G.: Transposition of the Great Arteries: Results of Treatment With Early Palliative and Late Intracardiac Repair, Circulation 51: 32, 1975. 4 Mustard, W. T., Keith, J. D., Trusler, G. A., Fowler, R. and Kidd, L.: The Surgical Management of Transposition of the Great Vessels, J. THORAC. CARDIOVASC. SURG. 48:953, 1964.
5 Mair, D. D., Danielson, G. K., Wallace, R. B., and McGoon, D. C : Long Term Follow-up of Mustard Operation Survivors, Circulation 50: 46, 1974 (Suppl. II). 6 Newfeld, E. A., Paul, M. H., Muster, A. J., and Idriss, F. S.: Pulmonary Vascular Disease in Complete Transposition of the Great Arteries: A Study of 200 Patients, Am. J. Cardiol. 34: 75, 1974. 7 Newfeld, E. A.: Pulmonary Thrombosis and Vascular Disease After Mustard Operation, Am. J. Cardiol. 37: 1115, 1975. 8 Clarkson, P. M., Neutze, J. M., Wardill, J. C , and Barratt-Boyes, B. C : The Pulmonary Vascular Bed in Patients With Complete Transposition of the Great Arteries, Circulation 53: 539, 1976. 9 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. 10 Ferencz, C : Transposition of the Great Vessels: Pathophysiologic Considerations Based Upon a Study of the Lungs, Circulation 33: 232, 1966.
C. Kratz, A. Davignon, C. Chartrand, and P. Stanley, Montreal,
Quebec,
Canada
A he introduction of balloon atrial septotomy in 1966 by Rashkind and Miller 1 has greatly improved the outlook for children with transposition of the great arteries. This is especially true for children with simple transposition since, in the absence of a significant shunt, palliation is needed at a very young age. Also, if palliation is successful, it is in these patients that surgical correction carries a relatively low risk 2 and offers a good chance of normal postoperative cardiac function. Although balloon atrial septotomy is successful in the majority of patients, its benefits are transitory. In most cases, hypoxia recurs in a matter of weeks or even days or, at best, after several months. There is at present no general agreement as to the best management at this point of the evolution. 3 Correction according to the method developed by Mustard 4 is being done in some centers even during the first months of life, so that the additional risk of a palliative intervention is avoided. Mustard's procedure is then carried out with deep hypothermia and circulatory arrest. Early results reported so far seem encouraging, 12 but not much is
known about the late effects of deep hypothermia or about the relative frequency of pulmonary or caval vein stenosis if correction is done at a very young age. 2 The alternative to early correction is the two-stage operation which had been generally employed before the introduction of balloon septotomy, i.e., atrial septectomy during the first months of life followed by Mustard's procedure, which is done electively after one year of age and which is still performed in large centers. 3 This approach carries the risk of two surgical interventions and of morbidity between the two operations. However, the risk of a corrective operation and extracorporeal circulation seems less in children than in infants, and the problems of hypothermia are avoided. At Sainte-Justine Hospital of Montreal, patients with d-transposition of the great arteries have been treated by two-stage surgical correction with very few exceptions. We are presenting the results of surgical treatment and the clinical follow-up of our patients.
From the Departments of Cardiology and Cardiovascular Surgery, Hopital Ste-Justine pour les Enfants, Universite' de Montreal, Montre'al, Quebec, Canada. Received for publication Sept. 17, 1976. Accepted for publication Dec. 20, 1976.
Since the performance of our first Rashkind atrial septotomy in 1967, 44 patients with d-transposition of the great arteries have been treated at our hospital. None of the patients had a patent ductus or ventricular septal defect at the time of operation. We are present-
Patients
707
The Journal of Thoracic and Cardiovascular Surgery
7 0 8 Kratz et al.
Table I. Rashkind balloon septotomy: Postseptotomy evolution Immediate death Late death Lost to follow-up Follow-up
2 1 2 39
Legend: Forty-four patients are included, ranging in age from birth to 50 days (mean 7 days).
Table II. Surgical septectomy: Postoperative evolution in 36 patients* Immediate death Late death Doing well; awaiting correction After Mustard procedure
1(11 days old) 2 (PVOD) 7 26
Legend: PVOD, Pulmonary vascular obstructive disease. The age at operation was 4 days to 13 months in these patients (mean age 3'A months). They were operated upon 2 days to 13 months after the Rashkind procedure.
Table III. Operative results of Mustard procedure (survival rate 96 per cent) With prior septectomy* With prior septectomy Without prior septectomy
1 (no recent follow-up) 25 (1 postop. deathf) 3 (no deaths)
Legend: This group comprises 29 patients ranging in age from 19 to 64 months (mean 37 months). *Operated upon elsewhere. tRenal insufficiency and pancreatitis.
ing the follow-up of these patients who, with few exceptions, have been treated by two-stage surgical correction following a balloon septotomy performed shortly after birth. Rashkind septotomy. The age at the time of balloon septotomy varied from zero to 50 days, mean age 7 days. Forty-five balloon septotomies were carried out altogether, as the procedure was repeated in one patient at the age of 3'/2 months. In all but one patient who had severe acidosis and cardiac failure, diagnostic procedures preceded the septotomy. The ductus arteriosus was patent in 11 patients aged from birth to 30 days, and it shunted from the aorta to the pulmonary artery. A small ventricular septal defect was present in one patient. All of these lesions closed spontaneously prior to the first surgical intervention, as verified by the changes in auscultation or by exploration during the operation. Following the septotomy, there were two immediate deaths owing to technical problems, one caused by catheterization and the other caused by balloon septotomy. Another patient died suddenly at the age of IVi months of unknown causes. Two patients have been
lost to follow-up and the remaining 39 patients have been seen regularly (Table I). The status of all but 3 of the patients who survived was improved by the procedure. These 3 patients needed a surgical septectomy in the days that followed. The degree and duration of improvement in the others were variable. Recurrence of significant cyanosis and signs of hypoxia were indications for surgical intervention. As the patients usually were very young (average age 3V4 months) at this stage of the disease, we felt that the risk of palliative surgery was less than the risk of a Mustard procedure. Atrial septectomy. Of the 39 patients having Rashkind procedures, 36 have undergone atrial septectomy and 3 patients have had correction of the malformation at the age of 26, 31, and 37 months, respectively, without other palliation. Atrial septectomy was done at ages varying from 4 days to 13 months (mean age 3Vz months). The time lapse between balloon septotomy and surgical septectomy varied from 2 days to 13 months, average 3V£ months. The surgical technique for septectomy is as follows: The heart is approached through a median sternotomy and the septum is resected leisurely with a straight rongeur introduced through the right atrial appendage. This technique has been used since 1960 and has been satisfactory. The atrial septal defect as seen at the time of the Mustard procedure is large, over 2.5 cm., and the septal ridge between the right pulmonary veins and the right atrium is totally absent. Of the 36 patients who underwent surgical resection of the atrial septum, one died 12 hours after the operation. He was a severely hypoxic 11-day-old baby in whom septotomy had not reduced the hypoxia. All patients who survived were in markedly improved condition. There has been no postoperative arrhythmia. Of the 35 patients who survived, 2 developed pulmonary vascular obstructive disease which was discovered at the ages of 18 and 23 months, respectively, during catheterization in preparation for a planned Mustard procedure. One of these 2 patients was later found to have a neuroblastoma which disseminated rapidly. Both patients have died. Autopsy revealed pulmonary vascular obstructive disease of Grade 4 (Heath and Edwards) in both cases. Seven patients are doing well on medical treatment awaiting surgical correction. Twenty-six have already undergone the Mustard operation (Table II). Mustard operation. At present, this corrective operation is performed as soon as symptoms recur or elec-
Volume 73 Number 5 May, 1977
Simple d-transposition of great arteries
709
Table IV. Evaluation of cardiac rhythm after Mustard procedure in relation to surgical management of coronary sinus in 25 patients Rhythm at time of follow-up Group*
No. of patients
Sinus rhythm
I
8
II
9
III
8
3 patients (37%) 4 patients (44%) 6 patients (75%)
Intermittent sinus and nodal rhythm
Nodal rhythm (A V dissociation)
Ectopic atrial rhythm
3 patients
1 patient
1 patient
1 patient
3 patients
1 patient
1 patient
1 patient
Legend: AV, Atrioventricular. *Group I: The coronary sinus was incised from the ostium down along its upper border, with the coronary sinus blood draining into the "new right atrium." Group II: The coronary sinus was incised along its upper border in the left atrium; the ostium was not incised but sutured, and the coronary sinus blood drained into the "new right atrium." Group III: The coronary sinus was left intact, with the coronary sinus blood draining into the "new left atrium."
tively at the age of 2 to 3 years. The age at elective operation has changed. In earlier years, our indication for corrective surgery being recurrent symptoms only, 2 of our patients reached the age of 5 years before undergoing the Mustard procedure. Before surgical correction, repeat catheterization is being done in all patients. So far 31 patients have been recatheterized. Right atrial pressures were recorded in all 31 patients. Left atrial pressures were also obtained in 29, and no gradient was noted across the septum. Mean atrial pressures were significantly elevated (above 7 mm. Hg) in 6 patients. Right and left ventricular pressures were recorded in all 31 patients. Pulmonary artery pressures were obtained in 21. There'was no evidence of pulmonary hypertension except in 3. One 6-month-old infant had a pulmonary pressure of 50 mm. Hg. He was in mild heart failure at the time of catheterization, and verification of left ventricular pressure during the corrective procedure showed normal values (25 mm. Hg). Two patients mentioned previously had severe pulmonary hypertension, pulmonary artery pressure being equal to systemic pressure in one and higher than systemic pressure in the other. They were considered to be inoperable. There was a subpulmonary diaphragmatic stenosis with a left ventricle-pulmonary artery pressure gradient of 60 mm. Hg in one patient. Another patient had stenosis of the right branch of the pulmonary artery with a systolic pressure of 67 mm. Hg in the main pulmonary artery and a main pulmonary artery-right pulmonary artery gradient of 37 mm. Hg. To date, 29 of the patients have undergone the Mustard operation at ages varying from 19 months to 64 months, mean age 37 months (Table III). One of the
Table V. Over-all results in 44 patients Result
Cause
No.
Medical deaths Early: During Rashkind and catheterization Late: After Rashkind Late: After septectomy
5 2
Technical problems
1 2
Sudden death (1 Vi months) PVOD* (2 and 3 years)
Surgical deaths Early: After septectomy (3% mortality rate) Early: After Mustard (4% mortality rate)
2 1
Survivors Awaiting Mustard After Mustard Lost to follow-up
1
Poor result from Rashkind (11 days) Renal insufficiency and pancreatitis
34 7 27 3
*PVOD, Pulmonary vascular obstructive disease.
patients had the corrective procedure elsewhere, and we have no recent follow-up. Twenty-five patients were operated upon here following prior septectomy. There was one postoperative death owing to renal failure and pancreatitis. Three patients had the Mustard procedure without septectomy and are doing well. The corrective procedure was done with a pericardial baffle in 26 patients and a Dacron patch in two. The 27 survivors have been observed for periods varying from 3 months to 63 months, mean follow-up 30 months (Table III). Immediate postoperative arrhythmia was common. Only 2 patients continued to have permanent sinus rhythm after the operation. Heart failure of varying degrees occurred in most patients, but it disappeared in all within a few months.
The Journal of Thoracic and Cardiovascular Surgery
7 1 0 Kratz et al.
Clinically, all 27 patients are asymptomatic. They are doing well and take part in normal activities. On physical examination, none of them shows signs of heart failure, although 2 patients have distended external jugular veins. The chest x-ray film of all of the patients shows a normal pulmonary vascularity indicating the absence of any significant pulmonary venous obstruction. Some degree of cardiomegaly persists in 17 of the 27 patients, which may well be related to the pericardiectomy. The 2 patients in whom obstruction of the superior vena cava was suspected clinically have been recatheterized. Angiograms showed the superior vena cava to be occluded in one and severely obstructed in the other. Late arrhythmia has been assessed with regard to a possible relationship between the frequency of ectopic rhythm and the surgical management of the coronary sinus as well as the method of cannulating the superior vena cava, these two factors being the only variants in surgical technique. The right atriotomy extended from the right atrial appendage downward to the origin of the right pulmonary veins, and the new left atrium was enlarged with a pericardial patch in all. Rhythms encountered were classified into four types. Type I is permanent sinus rhythm. We consider as sinus any rhythm with a normal PR interval and with normally directed P waves in the majority of the leads. Tracings which showed flattened isoelectric P waves in Lead I in the presence of normally directed P waves in the other standard leads are included. Type II is sinus rhythm alternating with nodal rhythm. Type III is permanent nodal rhythm. Nodal rhythm in all of our cases seems to be due to atrioventricular dissociation. Type IV is ectopic atrial rhythm with a normal, short, or prolonged PR interval and abnormal P wave configuration. In 25 of the 27 patients, the cavae were cannulated indirectly through the right atrial wall just anterior to the origin of the caval vessels. The coronary sinus was managed in various ways. These 25 patients were grouped as follows (Table IV): Group I. In 8 patients, the coronary sinus was incised from the ostium down along its upper border to drain the coronary sinus blood into the "new right atrium." Three patients on their last examination still had sinus rhythm, 3 had alternating sinus and nodal rhythm, one had continuous nodal rhythm, and one patient had an ectopic atrial rhythm with a short PR interval. Group II. In 9 patients, the coronary sinus was incised along its upper border in the left atrium, the ostium not being incised but sutured. Thus coronary sinus
blood again drained into the "new right atrium." Four patients when last seen were in sinus rhythm, one had alternating nodal and sinus rhythm, 3 had permanent nodal rhythm, and one had an ectopic atrial rhythm with a prolonged PR interval. Group III. In 8 patients, the coronary sinus was left intact, coronary sinus blood thus draining into the "new left atrium." Six of these 8 patients when last seen were in sinus rhythm, one had alternating nodal and sinus rhythm, and one had an ectopic atrial rhythm with a normal PR interval. Thus the incidence of ectopic rhythm is least frequent in the patients of the third group, in whom the coronary sinus was left intact. However, follow-up of this group has not been long enough to allow definitive conclusions, as ectopic rhythm has appeared as late as 3 years postoperatively in Groups I and II. In the last 2 patients operated upon, the superior vena cava was cannulated directly in its intrapericardial segment above the right atrium in order to avoid trauma to the sinus node, and the coronary sinus was untouched. Both have remained in sinus rhythm. Discussion The results show an over-all mortality rate of 16 per cent for the entire group of 44 patients with simple transposition of the great vessels treated in our hospital since 1967 (Table V). The surgical mortality rate is low both for surgical resection of the interatrial septum (3 per cent) and for total correction (4 per cent). The development of pulmonary vascular obstructive disease in 2 patients at the ages of 18 and 23 months, following the Rashkind procedure and the interatrial septectomy, is troublesome and raises the question of its prevention by early corrective surgery. The given data on this disease do not allow for any clear conclusion on this subject at present. Pulmonary vascular obstructive disease has, on rare occasions, appeared in transposition of the great arteries with intact ventricular septum after the Mustard procedure5-7 as well as before the operation.8-10 However it seems that the possibility of its development would be less likely if transposition were corrected before the child was 12 months of age. The low incidence of death and morbidity between the Rashkind procedure and total correction is most likely due to our liberal indications for surgical septal resection. Increasing cyanosis and hematocrit values rising above 60 per cent are indications for intervention even in the presence of good clinical status. After Mustard's procedure, all of our surviving patients are clinically well. However, postoperative ar-
Volume 73 Number 5 May, 1977
rhythmia is of concern. This seems related partly to surgical technique, and improvement in this line should help decrease the incidence of this complication. Avoidance of trauma to the coronary sinus has in our small group of patients diminished arrhythmias notably. Reducing trauma to the sinus node by direct cannulation of the superior vena cava above the right atrium should also reduce the chances of atrial arrhythmia. The 2 patients in whom the superior vena cava was cannulated directly would seem to bear out this point. However, it is not possible to draw conclusions on results obtained in 2 patients alone. The other postoperative problem is that of severe stenosis of the superior intra-auricular caval tunnel, which was encountered in 2 patients. This problem is related to surgical technique, and its occurrence should decrease with better evaluation of the surgical details involved in the construction of the superior auricular caval tunnel. The absence of pulmonary vein obstruction is most likely related to the orientation of the atriotomy, which in all of our patients extended downward from the atrial appendage to the right pulmonary veins, and to the enlargement of the new left atrium with a wide pericardial patch. Conclusion Because of the low mortality rate in these patients, we believe that two-stage surgical correction of transposition of the great arteries is preferable to one-stage surgical correction in the infants, as the risk of openheart surgery is greater in the patient under one year of age than in the young child.
Simple d-transposition
of great arteries
71 1
REFERENCES 1 Rashkind, W. J., and Miller, W. W.: Creation of an Atrial Septal Defect Without Thoracotomy: A Palliative Approach to Complete Transposition of the Great Arteries, J. A. M. A. 196: 991, 1966. 2 Stark, J., de Leval, M. R., Waterston, D. J., Graham, G. R., and Bonham-Carter, R. E.: Corrective Surgery of Transposition of the Great Arteries in the First Year of Life, J. THORAC. CARDIOVASC. SURG. 67: 673,
1974.
3 Gutgesell, H. P., and McNamara, D. G.: Transposition of the Great Arteries: Results of Treatment With Early Palliative and Late Intracardiac Repair, Circulation 51: 32, 1975. 4 Mustard, W. T., Keith, J. D., Trusler, G. A., Fowler, R. and Kidd, L.: The Surgical Management of Transposition of the Great Vessels, J. THORAC. CARDIOVASC. SURG. 48:953, 1964.
5 Mair, D. D., Danielson, G. K., Wallace, R. B., and McGoon, D. C : Long Term Follow-up of Mustard Operation Survivors, Circulation 50: 46, 1974 (Suppl. II). 6 Newfeld, E. A., Paul, M. H., Muster, A. J., and Idriss, F. S.: Pulmonary Vascular Disease in Complete Transposition of the Great Arteries: A Study of 200 Patients, Am. J. Cardiol. 34: 75, 1974. 7 Newfeld, E. A.: Pulmonary Thrombosis and Vascular Disease After Mustard Operation, Am. J. Cardiol. 37: 1115, 1975. 8 Clarkson, P. M., Neutze, J. M., Wardill, J. C , and Barratt-Boyes, B. C : The Pulmonary Vascular Bed in Patients With Complete Transposition of the Great Arteries, Circulation 53: 539, 1976. 9 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. 10 Ferencz, C : Transposition of the Great Vessels: Pathophysiologic Considerations Based Upon a Study of the Lungs, Circulation 33: 232, 1966.