Selective Operative Treatment for Tetralogy of Fallot: Rationale and Results HARVEY W. BENDER, JR., M.D., R. DARRYL FISHER, M.D., DAVIP M. CONKLE, M.D., CHARLES E. MARTIN, M.D., THOMAS P. GRAHAM, M.D.

Eighty-one patients with tetralogy of Fallot malformations evaluated between July 1, 1971 and November, 1975 are presented. Fifty-one patients underwent primary intracardiac repair; three died. Twenty-seven patients were corrected after a previous palliative shunt; there was one death. Three additional patients have been palliated and are awaiting repair. While the overall mortality in these patients was less than 5%, the infants undergoing total correction before the age of two years appeared to be at greater risk (25%). There were no deaths in the group of patients undergoing palliative procedures. Based on these data It appears that a safely performed palliative shunt in the symptomatic small infant is a reasonable first step, particularly if the outflow tract of the right ventricle is diffusely hypoplastic. SURGICAL TREATMENT of the tetralogy of Fallot mal-

From the Department of Cardiac and Thoracic Surgery, Vanderbilt University School of Medicine, Nashville, Tennessee

next two decades uncertainty about the choice and timing of palliative and corrective procedures resulted in many different approaches to the surgical treatment of these children. In recent years, the intracardiac correction of most congenital defects in the small infant has become possible with improvements in surgical techniques. As a result, interest in correction of the tetralogy malformation in the symptomatic infant has been renewed, and there has been a movement toward immediate correction of the tetralogy malformation by most surgeons and cardiologists. Our choice of a palliative or corrective operation has been based primarily on the specific intracardiac anatomy defined by precise angiography. In the past 4 years all patients with the tetralogy malformation, despite age or size, were considered for correction unless the intracardiac anatomy was considered unfavorable for primary correction.

formation had its origin when Dr. Alfred Blalock2 performed the first subclavian-pulmonary artery anastomosis for this condition in 1944. During the next decade operative treatment consisted entirely of palliative procedures designed to increase pulmonary blood flow in the cyanotic child with this malformation. In 1955 the intracardiac correction of tetralogy defects became a reality following development of extracorporeal circulation.57 Many of the early corrective procedures were done on small infants but operative mortality in this group Clinical Material was high. For this reason palliative systemic-pulmonary anastomosis as a preliminary procedure before eventual Eighty-one patients with symptoms of tetralogy of total correction was advocated by many. 10"12'13 During the Fallot have been evaluated at the Vanderbilt University Medical Center since July 1, 1971. There were 45 males and 36 females, whose ages ranged from 10 days to 46 Presented at the Annual Meeting of the Southern Surgical Associa- years. The selective treatment employed in these 81 paThe 1Hot December tion, 8-10, 1975, Homestead, Springs, Virginia. Reprint requests: Dr. Bender, Department of Cardiac and Thoracic Surgery, Vanderbilt University School of Medicine, Nashville,

Tennessee 37232.

tients was based upon consideration of the patient's clinical course and specific intracardiac anatomy. Fifty-one patients underwent total correction as the first

685

FIG. la.

after

a

Postoperative

aortogram in

a

tetralogy of Fallot pulmonary arteries can posterior aortopulmonary

patient

with

Waterston anastomosis. Flow into both

detected

positioned

properly

the

in

anastomosis.

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intracardiac

total

for

pulmonary

The

correction

arteries

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of satisfactory caliber and

.the right ventricular outflow tract

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assessment of

as determined by subjective cardiograms. Although right ventricular volume

plastic ments

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Operative Methods The technical aspects of the total correction as well as the method for creating a systemic-pulmonary anastomosis have been refined by Kirklin,6 McGoon,8 Cooley,3 and others.1'9'11'14 Several technical refinements and improvements in the overall management of the infant undergoing total correction have proved useful for us in recent years. Intraoperative monitoring is identical for patients undergoing either total correction or palliative shunts except for the placement of an intra-arterial catheter in the children undergoing cardiopulmonary bypass. The operating room is previously

procedure after diagnosis because the in this group was judged

and immediate

favorable

1976

complex outflow tract anatomy and had shunt procedures as their primary operative procedure. These patients are presently well and awaiting intracardiac repair. Operative techniques for the creation of the systemicpulmonary shunts have become relatively standardized. The Blalock-Taussig anastomosis2 remains our first choice for the palliative shunt and it was performed in 9 of the 13 children and infants. This shunt was always done if the subclavian artery was found to be of sufficient caliber at the time of thoracotomy for the palliative shunt. On the other hand, if the arteriographic findings suggested a diminutive subclavian artery in the small infant, a systemic-pulmonary shunt as originally described by Waterstont5 was used; 4 of the 13 patients underwent preliminary Waterston anastomoses.

s_

be

Ann. Surg. * June

BENDER AND OTHERS

686

were

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secondary importance

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July 1, 1971

patients we chose to proceed with preliminary systemic-pulmonary anastomosis; in these 10 patients the principal reason for this choice was the configuration of the right ventricular

or

another institution.

In the

other

10

outflow tract. Three additional patients with tetralogy of Fallot were evaluated by the authors and felt to have

FIG. lb. Postoperative aortogram in a patient with tetralogy of Fallot after a Waterston anastomosis. Preferential flow to the right pulmonary artery can be identified because of the creation of the anastomosis on the posterolateral aspect of the ascending aorta.

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warmed to prevent inadvertent cooling of these infants. Each child lies on a warming blanket throughout the operation. Arterial pressure monitoring is achieved in the small infants undergoing shunt procedures by the use of photoelectric pulse sensors. Continuous temperature and electrocardiographic monitoring is employed in all patients. The conduct of anesthesia is a critical consideration in these infants and two experienced anesthesiologists in constant attendance throughout the procedure can be of extreme importance. The same intraoperative monitoring is continued into the recovery period for patients undergoing shunts or total correction. Assisted or controlled ventilation is provided until it has been demonstrated by arterial blood gas determination that the infant or child can be safely extubated. The same technical emphasis is given to the palliative operation as to the total corrective procedures. Head lamp illumination and optical magnification have proved valuable for the small anastomoses. For the shunt procedures, a full posterolateral thoractomy was necessary to provide for excellent exposure for the systemicpulmonary anastomoses. For the Waterston shunt the right pulmonary artery is exposed posterior to the superior vena cava and dissected free well into the mediastinum. In this manner the anastomosis can be placed on the posterior aspect of the aorta. This posterior placement of the Waterston anastomosis will provide perfusion to both pulmonary arteries and will avoid undesirable kinking of the right pulmonary artery (Figs. la and b.) Similarly, for the Blalock-Taussig shunt, the right pulmonary artery is dissected to the main pulmonary artery and the subclavian and innominate artery are mobilized to the aortic arch to avoid undesirable tension and kinking of the anastomosis. The technique for total correction always consisted of cardiopulmonary bypass with moderate general body hypothermia (30 C). Deep hypothermia and circulatory arrest was not used in the series. A vertical right ventriculotomy was used in all but three patients; in those three patients an anomalous left anterior descending coronary artery arising from the right coronary artery required a transverse ventriculotomy. The standardized techniques of the intracardiac repair including infundibular resection and patch closure of the ventricular septal defect were utilized. Outflow tract reconstruction by means of fabric or pericardial patches across the pulmonary annulus was required in nearly one half (13/27) of the patients undergoing total correction after a preliminary systemicpulmonary anastomosis (Fig. 2). This frequency of outflow tract reconstruction in this subgroup of patients reflects the more severe degree of infundibular hypoplasia in those patients selected for a preliminary systemicpulmonary anastomosis. In contrast, 14 of the 51 pa-

687 60

50

NUMBER OF PATI ENTS

40

3027

20 14

10

13

0-

PRIMARY SECONDARY CORRECTION CORRECTION FIG. 2. Graphic comparison of the necessity of outflow tract reconstruction in 51 patients undergoing total correction without a preliminary systemic-pulmonary shunt and in 27 patients undergoing total correction following a systemic-pulmonary shunt. The hatched areas represent these patients requiring outflow tract reconstruction. The severity of the outflow tract malformation is reflected by the greater proportion of patients requiring outflow reconstruction at the time of secondary correction.

tients undergoing primary correction of tetralogy of Fallot required major outflow tract reconstruction. Results In the 13 patients undergoing palliative systemicpulmonary anastomosis, there have been no deaths. Immediate symptomatic improvement was achieved in all 13 patients. Total correction has been performed in 10 of these 13 patients; one of these 10 patients was a 14-month-old child who died of disseminated intravascular coagulation in the immediate postoperative period. The other 9 patients are asymptomatic. The 3 patients who have not yet been corrected are symptom-free and total correction is planned. There were no operative deaths in the 17 patients who underwent subsequent total correction after undergoing shunt procedures done before 1971 or at another institution, and each of these patients is now asymptomatic. Thus, of the 27 patients undergoing secondary correction, 26 are living and well (Fig. 3). Of the 51 patients who underwent primary intracardiac correction of their defects, there were 3 deaths in the

6050NUMBER OF

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51

40-

PATI ENTS 30-

127 20-

mediate intracardiac correction while 10 of the patients underwent immediate palliative systemic-pulmonary shunt as the first step in the surgical treatment of tetralogy of Fallot. Two of the 10 patients who underwent immediate palliative shunt procedures subsequently have undergone total correction before 2 years of age. The operative mortality for this subgroup of the youngest patients is depicted in Fig. 4. None of the 10 infants undergoing shunting procedures died as a result of the shunt procedure. Three of the 14 patients undergoing primary total correction died; one of the two patients undergoing total correction after a preliminary shunt died. These 4 deaths were the only deaths among the entire group of 78 patients undergoing total correction. Discussion

100

PRI MARY SECONDARY CORRECTION CORRECTION FIG. 3. Graphic comparison of the operative mortality between patients undergoing intracardiac repair of tetralogy of Fallot without previous systemic-pulmonary anastomosis (primary correction) and with a preliminary palliative shunt. There is no significant difference in the mortality experience for the two groups. (chi-square analysis P = 0.46)

postoperative period (Fig. 3). The remaining 48 patients are asymptomatic. The 3 patients who died were among the youngest and smallest infants in this group of 51 patients. Each weighed less than 9 kg and their ages were 2, 10, and 15 months. Two of these 3 deaths resulted from inadequate cardiac output after the procedure and the third resulted from sepsis. Each of the three infants

required outflow patch reconstruction. Twenty-six operations for symptomatic relief of tetralogy of Fallot were performed in 24 patients who were less than two years of age. The distribution of ages is shown in Table 1. The dominant consideration in the choice of palliative or corrective operation for this subset of 24 patients was the subjective assessment of the severity of the right ventricular outflow tract malformation. Fourteen of these 24 patients underwent imTABLE 1. Age Distribution of 24 Patients Less Than Two Years of Age Undergoing Surgical Treatment for the Tetralogy of Fallot 0- 6 7-12 13-18 19-24

mon mon mon mon

2 patients 4 patients

10 patients 8 patients

The selective operative treatment of tetralogy of Fallot has developed from previous surgical failures and surgical successes with the operative management of various subsets of patients with the tetralogy of Fallot. We originally thought that a significantly higher operative mortality might be found in those patients with the most severe intracardiac malformations. It was in this subset of patients with hypoplastic pulmonary arteries and diminutive outflows tracts that we initially considered to be the greatest operative risks for total correction and therefore advised palliative operations as their first operative procedure. When our results were analyzed, it became evident that there was no significant difference in the operative mortality between the subgroups of patients with localized outflow tract obstruction and those with diffuse infundibular obstruction and a small pulmonary annulus. The lack of correlation of operative mortality in these two subsets could likely be explained by the selective approach to their management. In those infants and children with hypoplastic outflow tracts, a temporizing palliative shunt provided time for growth so that total correction proved easier at a later date. On the other hand, it could be argued that in reality there is no essential difference in the operative mortality in these patients when contrasted on an anatomic basis. Certainly, if an acceptable overall mortality is to be achieved in the patients selected to undergo the two stage approach to total correction, the operative mortality for the palliative operation must be low and there must be no attrition among those patients awaiting total correction. Both these requirements have been satisfied in our group of patients undergoing palliative procedures. We continue to view the palliative systemicpulmonary shunt as an important surgical adjunct to the total correction of tetralogy of Fallot. Despite the high operative mortality4'10'1' which has been reported following shunt procedures and used as a reason to advocate

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immediate total correction in patients with tetralogy of Fallot, we feel that this operation can be performed safely even in the smallest infants with symptomatic tetralogy. For the operative mortality to be acceptably low, the same careful attention to detail must be used as that employed for the total corrective procedure. Similar monitoring and supportive measures are necessary to provide an acceptable low operative risk for the infant undergoing a palliative shunt. Similarly, the operation must be performed or immediately supervised by an experienced cardiac surgeon. Because there was no difference in the operative mortality when compared on an anatomic basis, we looked further at various subsets within the group of patients undergoing total correction. No correlation could be identified with a previous palliative shunt, with the degree of hypoxemia, with the use of patch reconstruction of the outflow tract, or with severity of preoperative symptoms. However, a definite trend was identified when the analysis was made solely on the basis of body weight and age. It became apparent that the greatest operative risk was age irrespective of the severity of his intracardiac malformation. In the subset. of infants less than two years of age the mortality for total correction was 25%. In contrast, no deaths were encountered in the 10 patients undergoing shunt procedures who were less than two years of age; moreover, the severity of the tetralogy malformation was generally greater in this group. In the remaining 62 patients who were two years of age or older, there were no deaths following intracardiac repair regardless of the presence or absence of a systemic pulmonary shunt. As a result of our continuing reassessment of the surgical results in the treatment of tetralogy of Fallot, we have developed guidelines we are presently using in the selective management of these patients. We select a palliative systemic-pulmonary shunt as the first and immediate form of surgical treatment in the small infants, usually less than 10 kg, who is experiencing symptoms. At the present time we would recommend palliative shunts for virtually all these small symptomatic infants. Even in the larger infant under two years of age whose right ventricular outflow tract is narrowed by more than simple localized infundibular stenosis, we would recommend an initial palliative systemic-pulmonary anastomosis. Furthermore, if complicating anatomic configurations e.g. anomalous coronary circulation, are identified by angiography in the infant, we prefer to delay total correction. In those patients who have undergone an initial palliative procedure, the timing of subsequent total correction should not be unduly delayed. Once reasonable body size has been attained, usually in the immediate preschool years, we recommend total correction. In the rare child who develops recurrent symptoms

689 15 14

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SHUNT STAGED PRIMARY CORRECTION CORRECTION FIG. 4. Graphic representation of the results of surgical treatment of 24 infants under two years of age. Three postoperative deaths (hatched areas) occurred in the 14 patients undergoing total correction without a previous systemic pulmonary shunt. One of the two patients undergoing secondary correction died postoperatively. There were no deaths in the infants undergoing palliative shunts.

or signs of arterial hypoxemia with a previous palliative shunt, we recommend total correction. A second palliatve systemic-pulmonary anastomosis in a symptomatic child with tetralogy of Fallot is a poor alternate selection. Total correction as the primary step is recommended for the majority of patients. Most children with tetralogy of Fallot malformations do not experience life threatening symptoms in early infancy and for this large subgroup of patients, the choice of a palliative or corrective procedure is never a real consideration. For the larger child, usually larger than 12-15 kg, we recommend intracardiac repair even in the presence of complicating anatomical features such as anomalous coronary circula-

tion. References 1. Barratt-Boyes, B. G., Simpsom, M. and Nuetze, J. M.: Intracardiac Surgery in Neonates and Infants Using Deep Hypothermia with Surface Cooling and Limited Cardiopulmonary Bypass. Circulation, 43:Suppl. 1:25, 1971. 2. Blalok, A. and Taussig, H. B.: The Surgical Treatment of Malformations of the Heart in Which There is Pulmonary Stenosis or Pulmonary Atresia. JAMA, 128:189, 1945. 3. Cooley, D. A. and Hallman, G. L.: Surgical Treatment of Congenital Heart Disease. Philadelphia. Lea and Febiger, 1966. 4. Hallman, G. L. and Cooley, D. A.: Cardiovascular Surgery in Newborn Infants: Results in 1050 Patients Less Than Two Years Old. Ann. Surg., 173:1007, 1971. 5. Kirklin, J. W., DuShane, J. W., Patrick, R. T., et al.: Intracardiac Surgery With the Aid of a Mechanical Pump-oxygenator System (Gibbon Type). Report of Eight Cases. Proc Staff Meet., Mayo Clinic, 30:201, 1955. 6. Kirklin, J. W. and Karp, R. B.: The Tetralogy of Fallot from a Surgical Viewpoint. Philadelphia, W. B. Saunders, 1970. 7. Lillehei, C. W., Cohen, M., Warden, H. E., et al.: Direct

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9. 10. 11.

Vision of Intracardiac Surgical Correction of Tetralogy of Fallot and Pulmonary Atresia Defect. Ann. Surg., 142:418, 1955. McGoon, D. C.: Techniques of Open Heart Surgery for Congenital Heart Disease. Curr. Probls. Surg., April, 1968. Maim, J. R., Bowman, F. V., Jr., Jameson, A. G., et al.: An Evaluation of Total Correction of Tetralogy of Fallot. Circulation, 27:805, 1963. Puga, F. J., DuShane, J. W. and McGoon, D. C.: Treatment of Tetralogy of Fallot in Children Less Than Four Years of Age. J. Thorac. Cardiovasc. Surg., 64:247, 1972. Starr, A., Bonchek, L. 1. and Sunderland, C. O.: Total

DISCUSSION DR. CLARENCE S. WELDON (St. Louis, Missouri): We looked at our data, and came up with a slightly different conclusion. We handled in our institution about 20 tetralogies a year, and this slide records data back to 1969. It's incomplete for the current year as it was done about six months ago. The operations are color coded, and arranged by year. Yellow is a total correction, and blue, green, and red are various kinds of shunts. Hospital mortality, where it occurred, is marked with an X, and an asterisk indicates an infant under one year of age done with deep hypothermia and circulatory arrest. The yearly mortality is indicated above each yearly group. There is a clear evolution shown in the way we managed tetralogy. In this early period the corrective operation was reserved for children over five. Children under five had palliative shunts done. in the middle period we extended the reparative operation down to about age two, and reserved palliative shunts only for those children younger than two. In the recent period we have abandoned the use of palliative shunts, except for a few children who had pulmonary arterial atresia in conjunction with tetralogy anatomy. It can be seen that despite the decreased use of shunts, there was an improvement in our overall mortality figures. If you look at this mortality more closely, most of the shunt mortality was some time ago. There was one shunt failure, with persistent cyanosis and death. There were two infants who had operations which were thought to be unsatisfactory for the relief of cyanosis. They then had secondary shunt operations, and developed congestive heart failure and death. There was one tragic pneumonia in a child who had a shunt; and then there was an infant who developed necrosis of the bowel. So there was a 13% mortality in this series of shunts when we were doing them earlier. Considering the mortality for total correction, there have been six of 87 (7.5%) children in this series who died following total correction. Two of them were early in the series. Both died of hemorrhagic atelectasis in the postoperative period. I think this is a preventable lesion. We don't see it anymore, and I think it was a consequence of excessive blood destruction, when we had a lot of suction during these operations, and when we primed the heart-lung machine with whole blood. We did have one uncontrolled intraoperative hemorrhage from an aorta which tore while we were taking down a Potts anastomosis. We had one patient who was a misdiagnosis. This child was thought to have shunt failure, but actually had an Eisenmenger reaction with far advanced pulmonary hypertension. We recognized this at the time of the operation, and unwisely went ahead with the correction anyway. We, of course, ended up with supersystemic pressure in the right ventricle and an intraoperative death. So that, really, is a misdiagnosis. We have one patient where we couldn't relieve the obstruction because of peripheral pulmonary artery coarctations, and ended up

Ann. Surg. i June 1976

Correction of Tetralogy of Fallot in Infancy. J. Thorac. Cardiovasc. Surg., 18:228, 1974. 12. Sterno, L. P., Bitash, A. F. and Lillehei, C. W.: Cardiovascular Surgery in Infancy. Am. J. Cardiol., 13:153, 1964. 13. Taussig, H. B.: Tetralogy of Fallot-Indications for Operation. Am. J. Cardiol., 12:90, 1963. 14. Venugopal, P. and Subramaman, S.: Intracardiac Repair of Tetralogy of Fallot in Patients Under Five Years of Age. Ann. Thorac. Surg., 18:228, 1974. 15. Waterston, D. J.: Treatment of Fallot's Tetralogy in Children Under One Year of Age. Rozhl. Chir., 41:181, 1962.

with a very high pressure in the right ventricle, and postoperative low output failure. And then we had a recent unexplained and unanticipated death in a child with Down's syndrome, who was extubated the next day, and suddenly died either from an arrhythmia or possibly from some form of respiratory arrest. If we look at these deaths according to risk factor, we then have only one patient under two. There were eight patients under two, and that was the only death. No patients over twenty died. And the shunts didn't seem to be related, except for this one who had the Potts tear. There were eight children in whom we took down Waterston shunts and repaired the pulmonary artery. There were no deaths in that group. So the way we came up with it was that an early correction is a good operation. It looked as though we were improving our results by doing early corrections. None of the five children who had repairs done under deep hypothermia and circulatory arrest, within the first year of life, died. My thought this morning would be, therefore, that Dr. Bender and his colleagues deserve an enormous commendation, and perhaps the operation of repair in early life does not yet deserve a severe condemnation. DR. JAMES B. DONAHOO (Baltimore): Our experience has been similar to theirs, and yet I would agree with Dr. Weldon on the fact that we would not completely condemn the early intraoperative correction in a child if the anatomy is favorable. While Dr. Bender's experience clearly indicates that, at least in Nashville, a preliminary shunt followed by correction is the operation of choice, I think other people's experience would indicate that there is at least some initial mortality in the small children that have undergone systemic pulmonary artery shunts; and coupled with the mortality of the operative correction itself, I think there is room for some primary intraoperative correction in these small children. DR. HARVEY W. BENDER (Closing discussion): I don't mean to condemn the immediate primary correction in this data. Our bias was certainly that we had the feeling that the primary correction in these small infants was going very well, but I think we tended to include those patients with VSD's, transpositions, and other things; so that the results in the first year have been quite good. But when we looked carefully at our tetralogy data, there was a very distinct break at two years of age, with those patients having their operations over two all living, and a significantly high mortality in those patients under two. And yet, the overall mortality for the entire group was less than 5%. In answer to Dr. Donahoo, I think you are absolutely correct. If there is a significant mortality with the shunt, and you couple this with the mortality of a subsequent procedure, it can creep up on you. I think the surgeon must keep shunt mortality very low, if you are going to do a two-stage procedure; but I believe that this can be done.

Selective operative treatment for tetraology of Fallot: rationale and results.

Selective Operative Treatment for Tetralogy of Fallot: Rationale and Results HARVEY W. BENDER, JR., M.D., R. DARRYL FISHER, M.D., DAVIP M. CONKLE, M.D...
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