- Email: [email protected]
Intracardiac repair of tetralogy of Fallot
Intracardiac repair of tetralogy of Fallot Five-year review of 403 patients During the 5 year period 1969 to 1973, 403 patients underwent intracardiac repair of tetralogy of Fallot. The patients ranged in age from 3 months to 41 years. Before operation, 86 per cent were cyanotic and 14 per cent were acyanotic. Forty-nine per cent had had at least one palliative procedure before total correction. Major associated anomalies included atrial septal defect in 24 per cent, patent ductus arteriosus in 2.5 per cent, coronary anomalies in 4 per cent, and left superior vena cava in 4.5 per cent. Seven per cent of the patients had pulmonary atresia. Pulmonary valvotomy was performed in 111 patients and complete excision of the pulmonary valve in 151. Patch graft reconstruction of the right ventricular outflow tract was performed in 57 per cent of the patients. In 11 patients a conduit was used to connect the right ventricle to the pulmonary artery. The over-all hospital mortality rate was 9.5 per cent, with the lowest rate (3.5 per cent) in the age group 6 to 10 years and the highest in the infant and adult groups (16.5 and 14.5 per cent, respectively). Three known late deaths occurred. A residual ventricular septal defect (VSD) was found in 3 per cent of the patients and an aneurysm of the pericardial patch in the right ventricular outflow tract developed in 7 patients. Intracardiac repair of tetralogy of Fallot can be performed with reasonable risk and low morbidity. In our experience the optimal age for elective surgery is between 6 and 10 years.
Luigi Chiariello, M.D., Joseph Meyer, M.D., Don C. Wukasch, M.D., Grady L. Hallman, M.D., and Denton A. Cooley, M.D., Houston, Texas
he
first successful intracardiac repair of tetralogy of Fallot was accomplished by Lillehei- in 1955 by means of controlled cross-circulation. Since that time continuous improvement in diagnostic and surgical techniques, quality of cardiopulmonary bypass, and postoperative care have aided in achieving progressively better results.v" Hospital mortality and morbidity have significantly decreased, late deaths are uncommon, and late symptomatic and hemodynamic results generally are excellent. Intracardiac repair From the Division of Surgery of the Texas Heart Institute, St. Luke's Episcopal and Texas Children's Hospital, Houston, Texas 77025. Received for publication April 15, 1975. Address for reprints: Denton A. Cooley, M.D., Texas Heart Institute, P. O. Box 20345, Houston, Texas 77025.
has been successfully extended to include complicated forms of tetralogy of Fallot."?" Until recently, most cardiovascular surgical centers maintained a policy of performing elective total correction in patients at 5 years of age and a palliative operation, if necessary in severely symptomatic children, before that age. However, a more aggressive attitude has been adopted and reported by several authors with excellent results.'>" This report is a review of our experience in intracardiac repair of tetralogy of Fallot during the period 1969 to 1973. Materials and methods
During a 17 year period, 954 patients underwent intracardiac repair of tetralogy of Fallot at the Texas Heart Institute of 529
The Journol of
530
Thoracic and Cardiovascular
Chiariello et al.
Surgery
Table I. Hospital mortality rate in 954 patients who had intracardiac repair of tetralogy of Fallot Per cent
Period Before 1969 1969-1973
551 403
61 38
11.0 9.5
Totals
954
99
10.5
Table II. Age at operation and hospital mortality rate in 403 patients who had intracardiac repair of tetralogy of Fallot from 1969 through 1973 Age
Percent
(yr.) <2 2-5 6-10 11-20 > 20
Totals
12 172 115 62 42
3.0 42.5 28.5 15.5 10.5
2 20 4 6 6
16.5 11.5 3.5 9.5 14.5
403
100.0
38
9.5
Texas Children's and St. Luke's Episcopal Hospitals in Houston, Texas. During the last 5 years of that period, January, 1969, through December, 1973, 403 consecutive intracardiac repairs were performed (Table I). The series included 237 males (59 per cent) and 166 (41 per cent) females whose ages ranged from 3 months to 41 years at operation (Table II). The patients weighed from 5 to 76 kilograms. Preoperative symptoms included cyanosis and clubbing, in various degrees, in 345 (86 per cent) of the patients. Fifty-eight patients (14 per cent) were acyanotic. Severe cyanosis with anoxic spells was present in 28 patients. In accordance with the New York Heart Association Functional Classifications, 76 per cent were in Class II, 23 per cent in Class III and 1 per cent in Class IV. Nine patients had experienced episodes of congestive heart failure. One hundred ninety-eight patients (49 per cent) had had at least one palliative procedure before correction. A single Blalock-Taussig anastomosis was present in 74 patients and a bilateral anastomosis in 6,
an ascending aorta-right pulmonary artery anastomosis in 56, an isolated Potts anastomosis in 44, and a Glenn anastomosis in 2. Multiple shunt procedures had been performed in 7 patients, the Brock procedure in 3, and pulmonary valvotomy in 3. In 2 cases the palliative procedure had been an ascending aorta-main pulmonary artery side-to-side anastomosis (Table III). Preoperative cardiac catheterization and angiocardiography were performed in all patients. The arterial oxygen saturation varied from 45 to 97 per cent. All patients had right ventricular hypertension at the systemic level. The right ventricular outflow tract obstruction was located at the infundibular and valvular level in 73 per cent of the patients; in 26 per cent there was isolated infundibular stenosis; and in 1 per cent, isolated valvular stenosis. Associated anomalies included atrial septal defect in 24 per cent, patent ductus arteriosus in 2.5 per cent, coronary anomalies in 4 per cent, and a left superior vena cava in 4.5 per cent. Pulmonary atresia was discovered in 28 patients (7 per cent) and congenital absence of the pulmonary valve in 6 (1.5 per cent). In 8 patients, overriding of the aorta was so severe that it simulated features of a double-outlet right ventricle. Other major associated anomalies are listed in Table IV. Surgical techniques
All but one of the operations were performed with the patient under total cardiopulmonary bypass. Venous cannulation was done through the right atrium into the superior and inferior venae cavae, with cannulation usually of the ascending aorta for arterial return. Moderate total body hypothermia and topical cooling of the heart with chilled saline was used occasionally, depending upon the anticipated duration of ischemic time. Deep hypothermia to 22 0 C. and complete cardiocirculatory arrest was used in 1 patient, a 3-month-old boy. Functioning shunts were closed immediately after institution of cardiopulmonary bypass. A paracoronary right ventriculotomy inci-
Volume 70
Repair of tetralogy of Fallot
Number 3 September, 1975
531
Table III. Operative history before intracardiac repair in tetralogy of Fallot in 403 patients Operative history
No. ofpatients
Percent
No. ofdeaths
Percent
205 198 74
51.0 49.0 18.5
81 20 5
9.0 10.0 6.5
56 44 2
14.0 11.0 0.5
7 7
12.5 15.5
6 7 3 3
1.5 1.7 0.7 0.7
3
0.5
Nonpalliated Palliated Simple Blalock-Taussig shunt Ascending aorta-right pulmonary artery anastomosis Potts procedure Glenn procedure Bilateral Blalock-Taussig shunt Multiple shunt procedures Brock procedure Pulmonary valvotomy Ascending aorta-main pulmonary artery anastomosis
I.5
Legend: SBE, Subacute bacterial endocarditis. RPA, Right pulmonary artery.
Table IV. Associated lesions in 403 patients who had total repair of tetralogy of Fallot
I
No. of _ _ _ _ _Lesion _ _ _ _ _ _--:p_a_Il_·en_l_s Per cent Atrial septal defect Patent ductus arteriosus Coronary anomalies Left superior vena cava Absent right superior vena cava Congenital pulmonary atresia Absent pulmonary valve Stenosis of pulmonary arteries Absent left pulmonary artery Mirror-image dextrocardia Corrected transposition Double-outlet right ventricle Aortic insufficiency (SBE) Congenital mitral stenosis Cleft mitral valve Divided RPA (after BlalockTaussig shunt) Left ventricular diverticulum
96 10
17 18 I 28 6
4 2 4 3 8
2 I I
24.0 2.5 4.0 4.5 0.25 7.0
I.5 1.0 0.5 1.0 0.75 2.0 0.5 0.25 0.25 0.25 0.25
sion was used. The infundibular stenosis was resected through this incision. The ventricular septal defect (VSD) was almost always closed with a knitted Dacron patch. Primary repair of the VSD was performed in only 3 patients, 1 of whom had a muscular type IV defect associated with a type II VSD, which was patched. In 3 pa-
tients the VSD was repaired through an aortic approach to prevent damage to an anomalous coronary artery. Pulmonary valvular stenosis was managed either through the right ventriculotomy or by extending the same incision through the annulus into the main pulmonary artery. Pulmonary valvotomy was performed in 111 patients (27.5 per cent). Deliberate complete excision of the pulmonary valve was carried out in 151 patients (37.5 per cent) and a partial excision in 5 others. Simple digital dilatation of the pulmonary valve was required in 6 patients (1.5 per cent). One pulmonary cusp was constructed with pericardium on the inner surface of a pericardial outflow patch in 5 patients (1.2 per cent). A narrow hypoplastic annulus was an indication for a patch reconstruction of the right ventricular outflow tract in 230 patients (57 per cent). Up through 1971 a pericardial patch was used in 144 patients; since 1972 woven Dacron has been utilized most frequently. Conduit reconstruction of the right ventricular outflow tract was performed in 11 patients (2.5 per cent). In 8 of the 11 patients the indication for reconstruction was congenital pulmonary atresia with a VSD, an extreme form of tetralogy
532
Chiariello et al.
of Fallot. In 2 patients an anomalous coronary artery crossed the right ventricular outflow tract and prevented performance of the usual technique of a patch graft reconstruction. One of these 2 patients also had transposition of the great arteries; the pulmonary valve was excised and a 12 mm. woven Dacron tube sewn to the ventriculotomy and to the main pulmonary artery to create a "double-outlet" venous ventricle. 'The last patient who underwent conduit reconstruction of the outflow tract of the right ventricle had severe pulmonary hypertension associated with a Potts anastomosis that had been performed many years previously. The usual patch repair resulted in severe pulmonary valvular insufficiency. In order to discontinue cardiopulmonary bypass it was necessary to replace the patch with a conduit containing a valve. Dacron tube conduits containing BjorkShiley prosthetic valves were used in 3 patients. One patient received a Dacron aortic allograft conduit containing a Bjork-Shiley valve. Four patients received aortic allografts containing the aortic valve with an attached mitral leaflet. Commercially available Dacron tube grafts * containing xenograft (porcine) valves were used in 2 patients. One patient, mentioned earlier, had a valveless Dacron conduit inserted to avoid injury to a major coronary artery. In 1 patient, Dacron patch reconstruction of the right ventricular outflow tract was performed in conjunction with insertion of a Bjork-Shiley valve prosthesis at the pulmonary site. One patient required replacement of the aortic valve because of associated aortic insufficiency following subacute bacterial endocarditis. Associated anomalies which required repair, e.g., atrial septal defect, patent ductus arteriosus, left ventricular diverticulum, etc., were also managed at the time of operation. Results
Of the total series of 954 patients who underwent total repair of tetralogy of Fallot "Hancock Laboratories, Inc., Anaheim, Calif.
The Journal of Thorocic ond Cordiovoscular Surgery
at the Texas Heart Institute during a 17 year period, 99 died in the hospital, for a hospital mortality rate of 10.5 per cent. Of the 403 patients operated upon during the 5 year period 1969 to 1973, 38 died during the postoperative course, for a hospital mortality rate of 9.5 per cent (Table I). Among the 237 males, there were 25 deaths (l0.5 per cent); among the 166 females, 13 deaths (8 per cent). Thirtyfive of 38 who died were cyanotic patients; thus the mortality rate for the cyanotic group was 10 per cent and that for the acyanotic group, 5 per cent. The highest hospital mortality rate was in the extreme age groups, i.e., below 2 years and above 20 years of age. The lowest mortality rate (3.5 per cent) was found in the age group 6 to 10 years (Table II). Of the two deaths in the infant group, one occurred in a 3-month-old boy who was notably cyanotic. This was the only patient in our series in whom deep hypothermia and complete cardiocirculatory arrest was employed. The patient died shortly after the operation because of cardiac failure. The other death in the infant group occurred in a 6-month-old boy and was caused by cerebral infarction following operative embolism. Four of 6 patients in the adult group (above 20 years of age) who died during the postoperative course had severe pulmonary hypertension (80 to 120 mm. Hg) which was related to a previous systemic-pulmonary shunt. Three of the 4 died from cardiac failure; I died from bronchopneumonia. Of the other two deaths, one was caused by cardiac failure. In this patient postmortem examination revealed severe mitral stenosis undiagnosed before operation. The last death in this group was attributed to technical factors. Correlation between hospital mortality rate and the presence or absence of a previous shunt is reported in Table III. There is no difference between the 2 groups. Cardiac failure and sudden cardiac arrest caused by arrhythmia were the principal causes of early deaths. Other causes of death included broncho-
Volume 70
Repair of tetralogy of Fallot
Number 3
533
September, 1975
Table V. Causes of death _____ _ _ _ _ _ _1 Cause
Cardiac failure Sudden cardiac arrest Bronchopneumonia Cerebral infarction Disseminated intravascular coagulopathy
I
;~~~:s
Percent
29 4 3 1
76.5 10.5 8.0 2.5 2.5
Table VI. Complications Percent
Complication Hemorrhage requiring re-exploration Residual VSD Post pericardiotomy syndrome Aneurysm R VOT pericardial patch Pulmonary infection Cerebral emboli Renal failure Sternal dehiscence Post-transfusion hepatitis
25 13 70 7 4 4 4
2 2
6.0 3.0 17.5 1.7 1.0 1.0 1.0 0.5 0.5
Legend: VSD, Ventricular septal defect. RVOT, Right ventricular outflow tract.
pneumonia (3 patients), cerebral infarction (1 patient), and disseminated intravascular coagulopathy (l patient) (Table V). The mean patient follow-up time was 15 months. Follow-up data were obtained in 26.5 per cent of the patients in this group, 91 per cent of whom had excellent or good clinical results. Three patients are known to have died after discharge. Among the three late deaths, one occurred 8 months after the operation from uncontrollable bleeding at the time of reoperation for repair of a residual VSD. The other two late deaths were caused by progressive heart failure, one in a patient with residual VSD. In 13 patients (3 per cent) repeat cardiac catheterization revealed a residual VSD which, in 4 patients, required surgical repair (one death occurred). Seven patients developed an aneurysm of the right ventricular outflow tract (pericardial patch) post operatively. This was subsequently resected in 6 patients. The other most common nonfatal postoperative complications included hemorrhage, requiring re-exploration in 25
patients, postpericardiotomy syndrome in 70, pulmonary infection in 4, cerebral emboli in 4, renal failure in 4, sternal dehiscence in 2, and post-transfusion hepatitis in 2 (Table VI). Postoperative hemorrhage was frequent in the adult group (17 per cent). Among the 11 patients who underwent a conduit reconstruction of the right ventricular outflow tract, 1 patient required reoperation because of a pressure gradient (85 mm. Hg) across the Bjork valve. Two years after the original correction, the valve was removed and a residual, small VSD was repaired primarily. In another patient a Dacron conduit containing a thrombosed Bjork valve was replaced with a Hancock prosthesis, and a residual VSD also was closed primarily 32 months after the original operation. Further details about the results of our" experience in conduit reconstruction of the right ventricular outflow tract have been reported elsewhere. Discussion
The results obtained in this group of patients indicate intracardiac repair of tetralogy of Fallot is a relatively safe procedure and one of the most satisfactory operations in cardiovascular surgery. Our experience also shows a progressive improvement in results: an 11 per cent mortality rate before 1969 decreased to 9.5 per cent in the 5 year period 1969 to 1973 (Table I). This figure, however, gives an inaccurate estimate of the surgical risk since many complex cases are included in the present series. The hospital mortality rate of 3.5 per cent in the group whose ages were 6 to 10 years indicates that this age range apparently is the most favorable period of life for elective operation. The mortality rate increases if repair is attempted in earlier or later periods of life and is significantly above average for the two age groups (1) below 2 years and (2) above 20 years. However, the only two deaths in the infant group were probably both related to technical factors and, in retrospect, perhaps could have been prevented. Previously we reported our experience in
The Journal of
534
Chiariello et al.
intracardiac repair of tetralogy of Fallot in adult patients" and observed a higher surgical risk in that age group (14.5 per cent) in contrast to the experience of others." 18 The higher mortality rate could be attributed to progressive pulmonary vascular obstructive disease found mostly in patients with large, long-lasting systemicpulmonary shunts. Von Bernuth and associates'" observed moderate-to-severe pul.monary vascular obstructive disease in 13 of 26 patients who were studied 6 to 18 years after creation of a Potts anastomosis. Also, operative results were poor in patients with severe pulmonary hypertension. For patients in our series who had preoperative palliative procedures, the mortality rate of intracardiac repair was slightly higher than that for those who had no palliative surgery (Table III). Patients with Potts anastomosis and, to a minor degree, ascending aortaright pulmonary anastomosis present a higher surgical risk related to the increase in pulmonary resistances. In contrast, the Blalock-Taussig anastomosis appears to be well tolerated. Cyanosis was associated with a higher mortality rate (10 per cent in the group with cyanosis compared to 5 per cent in the acyanotic group). Numerous anomalies of the cardiovascular system are associated with tetralogy of Fallot (Table IV), some of which required treatment at the time of intracardiac repair. Coronary artery anomalies occurred frequently (4 per cent) in our series. The anomalous vessel was usually the left anterior descending coronary artery arising from the right coronary artery. In this case, the left anterior passed across the downstream portion of the infudibulum. Care must be taken to avoid transection of this artery, since this causes left ventricular myocardial infarction. We 2 0 , 21 have reported elsewhere the surgical importance of coronary anomalies in tetralogy of Fallot and techniques which we use to avoid injury to the anomalous vessel. In the present series, 28 cases of congenital pulmonary atresia with VSD were included. This malformation is not actually a true case of tetralogy of Fallot; however, the hemody-
Thoracic and Cardiovascular Surgery
namic features and surgical problems are so similar to those presented by the patient with tetralogy of Fallot that we believe inclusion in this report is justified. In patients in whom the operation could not be performed in a manner similar to that for patients with simple tetralogy of Fallot (opening the atretic pulmonary valve and/ or performing a plastic revision of the main pulmonary artery), a conduit was used for reconstruction of the right ventricular outflow tract. 1 0 Results demonstrate that this technique may be used with reasonable risk and satisfactory clinical results. Postoperatively, 7 of 144 patients (5 per cent) developed true aneurysm of the pericardial patch used in the reconstruction of the right ventricular outflow tract. Reoperation was necessary in 6 of the 7 because of rapid and progressive enlargement. Rosenthal and co-workers" reported that this complication occurred in 6 per cent of their series. Since 1972 we have mainly used Dacron patches for reconstruction of the right ventricular outflow tract and have observed no aneurysms. In our series the incidence of known residual VSD was 3 per cent; there was a significant left-toright shunt which required reoperation in 4 of these cases (l per cent), with 1 death. No instances of complete heart block were observed after surgery. The pulmonary valve was completely excised in more than one third of the patients, and the right ventricular outflow tract was reconstructed with a patch in more than one half of the cases. This surgical technique results in a high incidence of pulmonary regurgitation; however, regurgitation is preferable to residual stenosis and has been reported well tolerated up to 10 years postoperatively. 23 Results of the present series suggest intracardiac repair of tetralogy of Fallot can be performed with reasonable risk and low morbidity. The optimal age for elective repair is from 6 to 10 years. However, progress in surgical technique, cardiopulmonary bypass, and postoperative care have made this operation feasible also in the younger age groups and even in infants. The increased
Volume 70 Number 3
Repair of tetralogy of Fallot
535
September, 1975
surgical risk found in the adult age group suggests that an earlier intracardiac repair in asymptomatic patients may be beneficial by preventing irreversible pulmonary vascular complications. REFERENCES Lillehei, C. W., Cohen, M., Warden, H. E., Reed, R. C., Aust, J. B., Dewall, R. A, and Varco, R. L.: Direct Vision Intracardiac Surgical Correction of the Tetralogy of Fallot, Pentalogy of Fallot and Pulmonary Atresia Defects: Report of First Ten Cases, Ann. Surg. 142: 418, 1955. 2 Bristow, J. D., Menashe, V. D., Griswold, H. E., and Starr, A: Total Correction of Tetralogy of Fallot: Complications and Results, Am. J. Cardio!. 8: 358, 1961. 3 Bristow, J. D., Adrouny, Z. A., Porter, G. A., Menashe, V. D., Starr, A, and Griswold, H. E.: Hemodynamic Studies After Total Correction of Tetralogy of Fallot, Am. J. Cardio!. 9: 924, 1962. 4 Kirklin, J. W., Wallace, R. B., McGoon, D. c., and DuShane, J. W.: Early and Late Results After Intracardiac Repair of Tetralogy of Fallot: Five-Year Review of 337 Patients, Ann. Surg. 162: 578, 1965. 5 Maim, J. R., Blumenthal, S., Bowman, F. 0., Ellis, K., Jameson, A G., Jesse, M. J., and Yeoh, C. B.: Factors That Modify Hemodynamic Results in Total Correction of Tetralogy of Fallot, J. THORAC. CARDIOVASC. SURG. 52: 502, 1966. 6 Zerbini, E. J.: The Surgical Treatment of the Complex of Fallot: Late Results, J. THORAC. CARDJOVASC. SURG. 58: 158, 1969. 7 Kirklin, J. W., and Karp, R. B.: The Tetralogy of Fallot From a Surgical Viewpoint, Philadelphia, 1970, W. B. Saunders Company. 8 Beach, P. M., Jr., Bowman, F. 0., Jr., Kaiser, G. A., and Maim, J. R.: Total Correction of Tetralogy of Fallot in Adolescents and Adults, Circulation 43, 44: 37, 1971 (Supp!. I). 9 Gersony, W. M., and Maim, J. R.: Late Results of Repair of Tetralogy of Fallot, in Kirklin, J. W., editor: Advances in Cardiovascular Surgery, New York, 1973, Grone & Stratton, Inc., p. 17. 10 Kyger, E. R., III, Chiariello, L., Hallman, G. L., and Cooley, D. A: Conduit Reconstruction of the Right Ventricular Outflow Tract: Experience With 17 Patients, Ann. Thorac. Surg. 19: 277, 1975. 11 Dobell, A R. C., Charrette, E. P., and Chughtai, M. S.: Correction of Tetralogy of Fallot in the Young Child, J. THORAC. CARDIOVASC. SURG. 55: 70, 1968.
12 Woodson, R. D., Burnell, R. H., Herr, R. H., Lees, M. H., and Starr, A.: Surgical Management of Tetralogy of Fallot in Children Under Four, Ann. Surg. 169: 257, 1969. 13 Starr, A, Bonchek, L. I., and Sunderland, C. 0.: Total Correction of Tetralogy of Fallot in Infancy, J. THORAC. CARDIOVASC. SURG. 65: 45, 1973. 14 Barratt-Boyes, B. G., and Neutze, J. M.: Primary Repair of Tetralogy of Fallot in Infancy Using Profound Hypothermia With Circulatory Arrest and Limited Cardiopulmonary Bypass: A Comparison With Conventional Two Stage Management, Ann. Surg. 178: 406, 1973. 15 Pacifico, A D., Bargeron, L. M., and Kirklin, J. W.: Primary Total Correction of Tetralogy of Fallot in Children Less Than Four Years of Age, Circulation 48: 1085, 1973. 16 Venugopal, P., and Subramanian, S.: Intracardiac Repair of Tetralogy of Fallot in Patients Under 5 Years of Age, Ann. Thorac. Surg. 18: 228, 1974. 17 Chiariello, L., Wukasch, D. C., Meyer, J., Sandiford, F. M., and Cooley, D. A: La correzione totale della tetralogia di Fallot negli adulti, Venice, 1974, Proceedings of the Fourteenth Meeting of the Italian Society for Thoracic Surgery, p. 151. 18 Bender, H. W., Jr., Haller, J. A., Jr., Brawley, R. K., Humphries, 0., Neill, C. A, and Gott, V. L.: Experience in Repair of Tetralogy of Fallot Malformation in Adults, Ann. Thorac. Surg. 11: 508, 1971. 19 Von Bernuth, G., Ritter, D. G., Grye, R. L., Weidman, W. H., David, G. D., and Me Goon, D. C.: Evaluation of Patients With Tetralogy of Fallot and Potts Anastomosis, Am. J. Cardio!. 27: 259, 1971. 20 Meyer, J., Chiariello, L., Hallman, G. L., and Cooley, D. A.: Coronary Artery Anomalies in Patients With Tetralogy of Fallot, J. THORAC. CARDIOVASC. SURG. 69: 373, 1974. 21 Meyer, J., Reul, G. J., Jr., Chiariello, L., Hallman, G. L., and Cooley, D. A: Anomalous Origin and Distribution of Coronary Arteries: Review of 38 Patients Who Underwent Operation, J. Cardiovasc. Surg. (Torino). In press. 22 Rosenthal, A., Gross, R. E., and Pasternac, A: Aneurysm of Right Ventricular Outflow Patches, J. THORAC. CARDIOVASC. SURG. 63: 735, 1972. 23 Bristow, J. D., Kloster, F. E., Lees, M. H., Menashe, V. D., Griswold, H. E., and Starr, A: Serial Cardiac Catheterizations and Exercise Hemodynamics After Correction of Tetralogy of Fallot, Circulation 41: 1057, 1970.
Luigi Chiariello, M.D., Joseph Meyer, M.D., Don C. Wukasch, M.D., Grady L. Hallman, M.D., and Denton A. Cooley, M.D., Houston, Texas
he
first successful intracardiac repair of tetralogy of Fallot was accomplished by Lillehei- in 1955 by means of controlled cross-circulation. Since that time continuous improvement in diagnostic and surgical techniques, quality of cardiopulmonary bypass, and postoperative care have aided in achieving progressively better results.v" Hospital mortality and morbidity have significantly decreased, late deaths are uncommon, and late symptomatic and hemodynamic results generally are excellent. Intracardiac repair From the Division of Surgery of the Texas Heart Institute, St. Luke's Episcopal and Texas Children's Hospital, Houston, Texas 77025. Received for publication April 15, 1975. Address for reprints: Denton A. Cooley, M.D., Texas Heart Institute, P. O. Box 20345, Houston, Texas 77025.
has been successfully extended to include complicated forms of tetralogy of Fallot."?" Until recently, most cardiovascular surgical centers maintained a policy of performing elective total correction in patients at 5 years of age and a palliative operation, if necessary in severely symptomatic children, before that age. However, a more aggressive attitude has been adopted and reported by several authors with excellent results.'>" This report is a review of our experience in intracardiac repair of tetralogy of Fallot during the period 1969 to 1973. Materials and methods
During a 17 year period, 954 patients underwent intracardiac repair of tetralogy of Fallot at the Texas Heart Institute of 529
The Journol of
530
Thoracic and Cardiovascular
Chiariello et al.
Surgery
Table I. Hospital mortality rate in 954 patients who had intracardiac repair of tetralogy of Fallot Per cent
Period Before 1969 1969-1973
551 403
61 38
11.0 9.5
Totals
954
99
10.5
Table II. Age at operation and hospital mortality rate in 403 patients who had intracardiac repair of tetralogy of Fallot from 1969 through 1973 Age
Percent
(yr.) <2 2-5 6-10 11-20 > 20
Totals
12 172 115 62 42
3.0 42.5 28.5 15.5 10.5
2 20 4 6 6
16.5 11.5 3.5 9.5 14.5
403
100.0
38
9.5
Texas Children's and St. Luke's Episcopal Hospitals in Houston, Texas. During the last 5 years of that period, January, 1969, through December, 1973, 403 consecutive intracardiac repairs were performed (Table I). The series included 237 males (59 per cent) and 166 (41 per cent) females whose ages ranged from 3 months to 41 years at operation (Table II). The patients weighed from 5 to 76 kilograms. Preoperative symptoms included cyanosis and clubbing, in various degrees, in 345 (86 per cent) of the patients. Fifty-eight patients (14 per cent) were acyanotic. Severe cyanosis with anoxic spells was present in 28 patients. In accordance with the New York Heart Association Functional Classifications, 76 per cent were in Class II, 23 per cent in Class III and 1 per cent in Class IV. Nine patients had experienced episodes of congestive heart failure. One hundred ninety-eight patients (49 per cent) had had at least one palliative procedure before correction. A single Blalock-Taussig anastomosis was present in 74 patients and a bilateral anastomosis in 6,
an ascending aorta-right pulmonary artery anastomosis in 56, an isolated Potts anastomosis in 44, and a Glenn anastomosis in 2. Multiple shunt procedures had been performed in 7 patients, the Brock procedure in 3, and pulmonary valvotomy in 3. In 2 cases the palliative procedure had been an ascending aorta-main pulmonary artery side-to-side anastomosis (Table III). Preoperative cardiac catheterization and angiocardiography were performed in all patients. The arterial oxygen saturation varied from 45 to 97 per cent. All patients had right ventricular hypertension at the systemic level. The right ventricular outflow tract obstruction was located at the infundibular and valvular level in 73 per cent of the patients; in 26 per cent there was isolated infundibular stenosis; and in 1 per cent, isolated valvular stenosis. Associated anomalies included atrial septal defect in 24 per cent, patent ductus arteriosus in 2.5 per cent, coronary anomalies in 4 per cent, and a left superior vena cava in 4.5 per cent. Pulmonary atresia was discovered in 28 patients (7 per cent) and congenital absence of the pulmonary valve in 6 (1.5 per cent). In 8 patients, overriding of the aorta was so severe that it simulated features of a double-outlet right ventricle. Other major associated anomalies are listed in Table IV. Surgical techniques
All but one of the operations were performed with the patient under total cardiopulmonary bypass. Venous cannulation was done through the right atrium into the superior and inferior venae cavae, with cannulation usually of the ascending aorta for arterial return. Moderate total body hypothermia and topical cooling of the heart with chilled saline was used occasionally, depending upon the anticipated duration of ischemic time. Deep hypothermia to 22 0 C. and complete cardiocirculatory arrest was used in 1 patient, a 3-month-old boy. Functioning shunts were closed immediately after institution of cardiopulmonary bypass. A paracoronary right ventriculotomy inci-
Volume 70
Repair of tetralogy of Fallot
Number 3 September, 1975
531
Table III. Operative history before intracardiac repair in tetralogy of Fallot in 403 patients Operative history
No. ofpatients
Percent
No. ofdeaths
Percent
205 198 74
51.0 49.0 18.5
81 20 5
9.0 10.0 6.5
56 44 2
14.0 11.0 0.5
7 7
12.5 15.5
6 7 3 3
1.5 1.7 0.7 0.7
3
0.5
Nonpalliated Palliated Simple Blalock-Taussig shunt Ascending aorta-right pulmonary artery anastomosis Potts procedure Glenn procedure Bilateral Blalock-Taussig shunt Multiple shunt procedures Brock procedure Pulmonary valvotomy Ascending aorta-main pulmonary artery anastomosis
I.5
Legend: SBE, Subacute bacterial endocarditis. RPA, Right pulmonary artery.
Table IV. Associated lesions in 403 patients who had total repair of tetralogy of Fallot
I
No. of _ _ _ _ _Lesion _ _ _ _ _ _--:p_a_Il_·en_l_s Per cent Atrial septal defect Patent ductus arteriosus Coronary anomalies Left superior vena cava Absent right superior vena cava Congenital pulmonary atresia Absent pulmonary valve Stenosis of pulmonary arteries Absent left pulmonary artery Mirror-image dextrocardia Corrected transposition Double-outlet right ventricle Aortic insufficiency (SBE) Congenital mitral stenosis Cleft mitral valve Divided RPA (after BlalockTaussig shunt) Left ventricular diverticulum
96 10
17 18 I 28 6
4 2 4 3 8
2 I I
24.0 2.5 4.0 4.5 0.25 7.0
I.5 1.0 0.5 1.0 0.75 2.0 0.5 0.25 0.25 0.25 0.25
sion was used. The infundibular stenosis was resected through this incision. The ventricular septal defect (VSD) was almost always closed with a knitted Dacron patch. Primary repair of the VSD was performed in only 3 patients, 1 of whom had a muscular type IV defect associated with a type II VSD, which was patched. In 3 pa-
tients the VSD was repaired through an aortic approach to prevent damage to an anomalous coronary artery. Pulmonary valvular stenosis was managed either through the right ventriculotomy or by extending the same incision through the annulus into the main pulmonary artery. Pulmonary valvotomy was performed in 111 patients (27.5 per cent). Deliberate complete excision of the pulmonary valve was carried out in 151 patients (37.5 per cent) and a partial excision in 5 others. Simple digital dilatation of the pulmonary valve was required in 6 patients (1.5 per cent). One pulmonary cusp was constructed with pericardium on the inner surface of a pericardial outflow patch in 5 patients (1.2 per cent). A narrow hypoplastic annulus was an indication for a patch reconstruction of the right ventricular outflow tract in 230 patients (57 per cent). Up through 1971 a pericardial patch was used in 144 patients; since 1972 woven Dacron has been utilized most frequently. Conduit reconstruction of the right ventricular outflow tract was performed in 11 patients (2.5 per cent). In 8 of the 11 patients the indication for reconstruction was congenital pulmonary atresia with a VSD, an extreme form of tetralogy
532
Chiariello et al.
of Fallot. In 2 patients an anomalous coronary artery crossed the right ventricular outflow tract and prevented performance of the usual technique of a patch graft reconstruction. One of these 2 patients also had transposition of the great arteries; the pulmonary valve was excised and a 12 mm. woven Dacron tube sewn to the ventriculotomy and to the main pulmonary artery to create a "double-outlet" venous ventricle. 'The last patient who underwent conduit reconstruction of the outflow tract of the right ventricle had severe pulmonary hypertension associated with a Potts anastomosis that had been performed many years previously. The usual patch repair resulted in severe pulmonary valvular insufficiency. In order to discontinue cardiopulmonary bypass it was necessary to replace the patch with a conduit containing a valve. Dacron tube conduits containing BjorkShiley prosthetic valves were used in 3 patients. One patient received a Dacron aortic allograft conduit containing a Bjork-Shiley valve. Four patients received aortic allografts containing the aortic valve with an attached mitral leaflet. Commercially available Dacron tube grafts * containing xenograft (porcine) valves were used in 2 patients. One patient, mentioned earlier, had a valveless Dacron conduit inserted to avoid injury to a major coronary artery. In 1 patient, Dacron patch reconstruction of the right ventricular outflow tract was performed in conjunction with insertion of a Bjork-Shiley valve prosthesis at the pulmonary site. One patient required replacement of the aortic valve because of associated aortic insufficiency following subacute bacterial endocarditis. Associated anomalies which required repair, e.g., atrial septal defect, patent ductus arteriosus, left ventricular diverticulum, etc., were also managed at the time of operation. Results
Of the total series of 954 patients who underwent total repair of tetralogy of Fallot "Hancock Laboratories, Inc., Anaheim, Calif.
The Journal of Thorocic ond Cordiovoscular Surgery
at the Texas Heart Institute during a 17 year period, 99 died in the hospital, for a hospital mortality rate of 10.5 per cent. Of the 403 patients operated upon during the 5 year period 1969 to 1973, 38 died during the postoperative course, for a hospital mortality rate of 9.5 per cent (Table I). Among the 237 males, there were 25 deaths (l0.5 per cent); among the 166 females, 13 deaths (8 per cent). Thirtyfive of 38 who died were cyanotic patients; thus the mortality rate for the cyanotic group was 10 per cent and that for the acyanotic group, 5 per cent. The highest hospital mortality rate was in the extreme age groups, i.e., below 2 years and above 20 years of age. The lowest mortality rate (3.5 per cent) was found in the age group 6 to 10 years (Table II). Of the two deaths in the infant group, one occurred in a 3-month-old boy who was notably cyanotic. This was the only patient in our series in whom deep hypothermia and complete cardiocirculatory arrest was employed. The patient died shortly after the operation because of cardiac failure. The other death in the infant group occurred in a 6-month-old boy and was caused by cerebral infarction following operative embolism. Four of 6 patients in the adult group (above 20 years of age) who died during the postoperative course had severe pulmonary hypertension (80 to 120 mm. Hg) which was related to a previous systemic-pulmonary shunt. Three of the 4 died from cardiac failure; I died from bronchopneumonia. Of the other two deaths, one was caused by cardiac failure. In this patient postmortem examination revealed severe mitral stenosis undiagnosed before operation. The last death in this group was attributed to technical factors. Correlation between hospital mortality rate and the presence or absence of a previous shunt is reported in Table III. There is no difference between the 2 groups. Cardiac failure and sudden cardiac arrest caused by arrhythmia were the principal causes of early deaths. Other causes of death included broncho-
Volume 70
Repair of tetralogy of Fallot
Number 3
533
September, 1975
Table V. Causes of death _____ _ _ _ _ _ _1 Cause
Cardiac failure Sudden cardiac arrest Bronchopneumonia Cerebral infarction Disseminated intravascular coagulopathy
I
;~~~:s
Percent
29 4 3 1
76.5 10.5 8.0 2.5 2.5
Table VI. Complications Percent
Complication Hemorrhage requiring re-exploration Residual VSD Post pericardiotomy syndrome Aneurysm R VOT pericardial patch Pulmonary infection Cerebral emboli Renal failure Sternal dehiscence Post-transfusion hepatitis
25 13 70 7 4 4 4
2 2
6.0 3.0 17.5 1.7 1.0 1.0 1.0 0.5 0.5
Legend: VSD, Ventricular septal defect. RVOT, Right ventricular outflow tract.
pneumonia (3 patients), cerebral infarction (1 patient), and disseminated intravascular coagulopathy (l patient) (Table V). The mean patient follow-up time was 15 months. Follow-up data were obtained in 26.5 per cent of the patients in this group, 91 per cent of whom had excellent or good clinical results. Three patients are known to have died after discharge. Among the three late deaths, one occurred 8 months after the operation from uncontrollable bleeding at the time of reoperation for repair of a residual VSD. The other two late deaths were caused by progressive heart failure, one in a patient with residual VSD. In 13 patients (3 per cent) repeat cardiac catheterization revealed a residual VSD which, in 4 patients, required surgical repair (one death occurred). Seven patients developed an aneurysm of the right ventricular outflow tract (pericardial patch) post operatively. This was subsequently resected in 6 patients. The other most common nonfatal postoperative complications included hemorrhage, requiring re-exploration in 25
patients, postpericardiotomy syndrome in 70, pulmonary infection in 4, cerebral emboli in 4, renal failure in 4, sternal dehiscence in 2, and post-transfusion hepatitis in 2 (Table VI). Postoperative hemorrhage was frequent in the adult group (17 per cent). Among the 11 patients who underwent a conduit reconstruction of the right ventricular outflow tract, 1 patient required reoperation because of a pressure gradient (85 mm. Hg) across the Bjork valve. Two years after the original correction, the valve was removed and a residual, small VSD was repaired primarily. In another patient a Dacron conduit containing a thrombosed Bjork valve was replaced with a Hancock prosthesis, and a residual VSD also was closed primarily 32 months after the original operation. Further details about the results of our" experience in conduit reconstruction of the right ventricular outflow tract have been reported elsewhere. Discussion
The results obtained in this group of patients indicate intracardiac repair of tetralogy of Fallot is a relatively safe procedure and one of the most satisfactory operations in cardiovascular surgery. Our experience also shows a progressive improvement in results: an 11 per cent mortality rate before 1969 decreased to 9.5 per cent in the 5 year period 1969 to 1973 (Table I). This figure, however, gives an inaccurate estimate of the surgical risk since many complex cases are included in the present series. The hospital mortality rate of 3.5 per cent in the group whose ages were 6 to 10 years indicates that this age range apparently is the most favorable period of life for elective operation. The mortality rate increases if repair is attempted in earlier or later periods of life and is significantly above average for the two age groups (1) below 2 years and (2) above 20 years. However, the only two deaths in the infant group were probably both related to technical factors and, in retrospect, perhaps could have been prevented. Previously we reported our experience in
The Journal of
534
Chiariello et al.
intracardiac repair of tetralogy of Fallot in adult patients" and observed a higher surgical risk in that age group (14.5 per cent) in contrast to the experience of others." 18 The higher mortality rate could be attributed to progressive pulmonary vascular obstructive disease found mostly in patients with large, long-lasting systemicpulmonary shunts. Von Bernuth and associates'" observed moderate-to-severe pul.monary vascular obstructive disease in 13 of 26 patients who were studied 6 to 18 years after creation of a Potts anastomosis. Also, operative results were poor in patients with severe pulmonary hypertension. For patients in our series who had preoperative palliative procedures, the mortality rate of intracardiac repair was slightly higher than that for those who had no palliative surgery (Table III). Patients with Potts anastomosis and, to a minor degree, ascending aortaright pulmonary anastomosis present a higher surgical risk related to the increase in pulmonary resistances. In contrast, the Blalock-Taussig anastomosis appears to be well tolerated. Cyanosis was associated with a higher mortality rate (10 per cent in the group with cyanosis compared to 5 per cent in the acyanotic group). Numerous anomalies of the cardiovascular system are associated with tetralogy of Fallot (Table IV), some of which required treatment at the time of intracardiac repair. Coronary artery anomalies occurred frequently (4 per cent) in our series. The anomalous vessel was usually the left anterior descending coronary artery arising from the right coronary artery. In this case, the left anterior passed across the downstream portion of the infudibulum. Care must be taken to avoid transection of this artery, since this causes left ventricular myocardial infarction. We 2 0 , 21 have reported elsewhere the surgical importance of coronary anomalies in tetralogy of Fallot and techniques which we use to avoid injury to the anomalous vessel. In the present series, 28 cases of congenital pulmonary atresia with VSD were included. This malformation is not actually a true case of tetralogy of Fallot; however, the hemody-
Thoracic and Cardiovascular Surgery
namic features and surgical problems are so similar to those presented by the patient with tetralogy of Fallot that we believe inclusion in this report is justified. In patients in whom the operation could not be performed in a manner similar to that for patients with simple tetralogy of Fallot (opening the atretic pulmonary valve and/ or performing a plastic revision of the main pulmonary artery), a conduit was used for reconstruction of the right ventricular outflow tract. 1 0 Results demonstrate that this technique may be used with reasonable risk and satisfactory clinical results. Postoperatively, 7 of 144 patients (5 per cent) developed true aneurysm of the pericardial patch used in the reconstruction of the right ventricular outflow tract. Reoperation was necessary in 6 of the 7 because of rapid and progressive enlargement. Rosenthal and co-workers" reported that this complication occurred in 6 per cent of their series. Since 1972 we have mainly used Dacron patches for reconstruction of the right ventricular outflow tract and have observed no aneurysms. In our series the incidence of known residual VSD was 3 per cent; there was a significant left-toright shunt which required reoperation in 4 of these cases (l per cent), with 1 death. No instances of complete heart block were observed after surgery. The pulmonary valve was completely excised in more than one third of the patients, and the right ventricular outflow tract was reconstructed with a patch in more than one half of the cases. This surgical technique results in a high incidence of pulmonary regurgitation; however, regurgitation is preferable to residual stenosis and has been reported well tolerated up to 10 years postoperatively. 23 Results of the present series suggest intracardiac repair of tetralogy of Fallot can be performed with reasonable risk and low morbidity. The optimal age for elective repair is from 6 to 10 years. However, progress in surgical technique, cardiopulmonary bypass, and postoperative care have made this operation feasible also in the younger age groups and even in infants. The increased
Volume 70 Number 3
Repair of tetralogy of Fallot
535
September, 1975
surgical risk found in the adult age group suggests that an earlier intracardiac repair in asymptomatic patients may be beneficial by preventing irreversible pulmonary vascular complications. REFERENCES Lillehei, C. W., Cohen, M., Warden, H. E., Reed, R. C., Aust, J. B., Dewall, R. A, and Varco, R. L.: Direct Vision Intracardiac Surgical Correction of the Tetralogy of Fallot, Pentalogy of Fallot and Pulmonary Atresia Defects: Report of First Ten Cases, Ann. Surg. 142: 418, 1955. 2 Bristow, J. D., Menashe, V. D., Griswold, H. E., and Starr, A: Total Correction of Tetralogy of Fallot: Complications and Results, Am. J. Cardio!. 8: 358, 1961. 3 Bristow, J. D., Adrouny, Z. A., Porter, G. A., Menashe, V. D., Starr, A, and Griswold, H. E.: Hemodynamic Studies After Total Correction of Tetralogy of Fallot, Am. J. Cardio!. 9: 924, 1962. 4 Kirklin, J. W., Wallace, R. B., McGoon, D. c., and DuShane, J. W.: Early and Late Results After Intracardiac Repair of Tetralogy of Fallot: Five-Year Review of 337 Patients, Ann. Surg. 162: 578, 1965. 5 Maim, J. R., Blumenthal, S., Bowman, F. 0., Ellis, K., Jameson, A G., Jesse, M. J., and Yeoh, C. B.: Factors That Modify Hemodynamic Results in Total Correction of Tetralogy of Fallot, J. THORAC. CARDIOVASC. SURG. 52: 502, 1966. 6 Zerbini, E. J.: The Surgical Treatment of the Complex of Fallot: Late Results, J. THORAC. CARDJOVASC. SURG. 58: 158, 1969. 7 Kirklin, J. W., and Karp, R. B.: The Tetralogy of Fallot From a Surgical Viewpoint, Philadelphia, 1970, W. B. Saunders Company. 8 Beach, P. M., Jr., Bowman, F. 0., Jr., Kaiser, G. A., and Maim, J. R.: Total Correction of Tetralogy of Fallot in Adolescents and Adults, Circulation 43, 44: 37, 1971 (Supp!. I). 9 Gersony, W. M., and Maim, J. R.: Late Results of Repair of Tetralogy of Fallot, in Kirklin, J. W., editor: Advances in Cardiovascular Surgery, New York, 1973, Grone & Stratton, Inc., p. 17. 10 Kyger, E. R., III, Chiariello, L., Hallman, G. L., and Cooley, D. A: Conduit Reconstruction of the Right Ventricular Outflow Tract: Experience With 17 Patients, Ann. Thorac. Surg. 19: 277, 1975. 11 Dobell, A R. C., Charrette, E. P., and Chughtai, M. S.: Correction of Tetralogy of Fallot in the Young Child, J. THORAC. CARDIOVASC. SURG. 55: 70, 1968.
12 Woodson, R. D., Burnell, R. H., Herr, R. H., Lees, M. H., and Starr, A.: Surgical Management of Tetralogy of Fallot in Children Under Four, Ann. Surg. 169: 257, 1969. 13 Starr, A, Bonchek, L. I., and Sunderland, C. 0.: Total Correction of Tetralogy of Fallot in Infancy, J. THORAC. CARDIOVASC. SURG. 65: 45, 1973. 14 Barratt-Boyes, B. G., and Neutze, J. M.: Primary Repair of Tetralogy of Fallot in Infancy Using Profound Hypothermia With Circulatory Arrest and Limited Cardiopulmonary Bypass: A Comparison With Conventional Two Stage Management, Ann. Surg. 178: 406, 1973. 15 Pacifico, A D., Bargeron, L. M., and Kirklin, J. W.: Primary Total Correction of Tetralogy of Fallot in Children Less Than Four Years of Age, Circulation 48: 1085, 1973. 16 Venugopal, P., and Subramanian, S.: Intracardiac Repair of Tetralogy of Fallot in Patients Under 5 Years of Age, Ann. Thorac. Surg. 18: 228, 1974. 17 Chiariello, L., Wukasch, D. C., Meyer, J., Sandiford, F. M., and Cooley, D. A: La correzione totale della tetralogia di Fallot negli adulti, Venice, 1974, Proceedings of the Fourteenth Meeting of the Italian Society for Thoracic Surgery, p. 151. 18 Bender, H. W., Jr., Haller, J. A., Jr., Brawley, R. K., Humphries, 0., Neill, C. A, and Gott, V. L.: Experience in Repair of Tetralogy of Fallot Malformation in Adults, Ann. Thorac. Surg. 11: 508, 1971. 19 Von Bernuth, G., Ritter, D. G., Grye, R. L., Weidman, W. H., David, G. D., and Me Goon, D. C.: Evaluation of Patients With Tetralogy of Fallot and Potts Anastomosis, Am. J. Cardio!. 27: 259, 1971. 20 Meyer, J., Chiariello, L., Hallman, G. L., and Cooley, D. A.: Coronary Artery Anomalies in Patients With Tetralogy of Fallot, J. THORAC. CARDIOVASC. SURG. 69: 373, 1974. 21 Meyer, J., Reul, G. J., Jr., Chiariello, L., Hallman, G. L., and Cooley, D. A: Anomalous Origin and Distribution of Coronary Arteries: Review of 38 Patients Who Underwent Operation, J. Cardiovasc. Surg. (Torino). In press. 22 Rosenthal, A., Gross, R. E., and Pasternac, A: Aneurysm of Right Ventricular Outflow Patches, J. THORAC. CARDIOVASC. SURG. 63: 735, 1972. 23 Bristow, J. D., Kloster, F. E., Lees, M. H., Menashe, V. D., Griswold, H. E., and Starr, A: Serial Cardiac Catheterizations and Exercise Hemodynamics After Correction of Tetralogy of Fallot, Circulation 41: 1057, 1970.