- Email: [email protected]
Surgical treatment of truncus arteriosus, with emphasis on infants and small children
Surgical treatment of truncus arteriosus, with emphasis on infants and small children Two of 10 children with truncus arteriosus. Type I, II, less than 2 years of age, 6 of 7 between 2 and 5 years of age, and all 5 older than 5 years survived after complete repair. Five of the nine hospital deaths are believed related to inadequate intra- and postoperative management. One late death has occurred 42 months postoperatively. The low hospital mortality rates in those 2 years of age and older, good long-term results to date, and the poor prognosis of children not operated upon indicate the advisability of elective repair at age 2 to 3 years. Although results in infants less than 2 years old have not been good, current improvements of intra- and postoperative care and the lack of a better alternative suggest that prompt primary repair is indicated for infants with intractable heart failure or increasing pulmonary vascular resistance.
Azai Appelbaum, M.D., Lionel M. Bargeron, Jr., M.D., Albert D. Pacifico, M.D., and John W. Kirklin, M.D., Birmingham, Ala.
M air and colleagues1 recently analyzed their large experience at the Mayo Clinic with the investigation and surgical treatment of patients with truncus arteriosus Type I, II. Although their group of patients treated surgically included none under one year of age, they alluded specifically to the problems of management of this malformation in infants. Responding to the same problem, Poirier and colleagues2 recently suggested a management program based on pulmonary arterial banding for infants in congestive heart failure and deferral of intracardiac repair until the age of 5 years. Mair and colleagues suggested also that repair be deferred until age 4 years unless there is significant increase in pulmonary vascular resistance before that time. Since January, 1971, we have practiced primary definitive repair for infants and small children less than 5 years of age with truncus arteriosus. Repair was advised electively at age 2 to 3 years but was done early From the Departments of Surgery and Pediatrics, The University of Alabama Medical School and Medical Center, Birmingham, Ala. 35294. Received for publication June 27, 1975. Address for reprints: John W. Kirklin, M.D., Department of Surgery, University Station, Birmingham, Ala. 35294.
436
in infancy when operative intervention seemed urgently indicated because of congestive heart failure, severe growth failure, or increasing pulmonary vascular resistance. This management program was based on the strong likelihood of patients with this malformation dying in infancy; the fact that an unknown but significant number of patients with truncus arteriosus have severe pulmonary vascular disease and are inoperable by the age of 5 years1; and our extrapolation of data3 showing that the long-term results in patients with large ventricular septal defects and severe pulmonary hypertension are best when the child is less than about 2 to 3 years of age at the time of repair. The hospital mortality rate in infants has been high, but the over-all results are probably better than those of banding followed by later repair, and they should improve with the knowledge and experience gained during the past 5 years. The results in children age 2 or more at the time of operation have been satisfactory. Clinical material and methods Twenty-two patients with truncus arteriosus, Type I, II, underwent corrective surgery at The University of Alabama Medical Center between Jan. 1, 1971, and Oct. 1, 1974. Two children over 5 years of age were operated upon before that date. No patients underwent
Volume 71 Number 3 March, 1976
pulmonary arterial banding during that period. No patients were denied operation because of young age, small size, presence of truncal valve incompetence, or severity of congestive heart failure, but a few older patients were advised against surgery because they had severe pulmonary vascular disease. The operation was that described by McGoon, Rastelli, and Ongley.4 Many technical details of the operation varied as experience grew. In the first 3 cases we used an aortic homograft with its retained valve as the valved external conduit. Subsequently, we used a composite valved conduit of Dacron containing within it a homograft valve5 or more recently a heterograft valve.6 In the early cases, in infants and small children, as large a valved external conduit as could be accommodated was selected; however, in the past few years one whose size was harmonious with the heart's size has been used. In the first 2 years of our experience, infants less than 2 years of age were operated upon by the Barratt-Boyes-Kyoto technique7 of profound hypothermia, limited cardiopulmonary bypass, and total circulatory arrest. Subsequently, we8 used profound hypothermia with low-flow perfusion. The postoperative management was less intense during the early part of the experience than it was in the last 24 months of it. In general, blood was infused to maintain left atrial pressure at about 14 mm. Hg. Catecholamines were used where cardiac output seemed less than desired. All patients remained intubated for at least 24 hours postoperatively. Many of the infants and small children breathed spontaneously with continuous positive airway pressure9 during the early years, but in the last 2 years all have been managed initially with controlled ventilation. Autopsy was done in 7 of the 11 patients who died. The cause of death was assigned after retrospective review of the operative note, notes and data from the postoperative period, and the report of the autopsy. Follow-up was by personal examination or correspondence with referring physicians, with the date of inquiry being April 1, 1975. Results Hospital mortality rate. Two infants underwent repair of the truncus arteriosus and replacement of repair of a severely incompetent truncal valve, and both died early postoperatively. Ten additional infants less than 2 years of age had corrective surgery, 8 of whom died in hospital. Both infants less than 6 months (2 and 5 months) old at the time of operation died. Three of 5 operated upon between 6 to 12 months of age died, the surviving infants being 8 and 9 months old when
Truncus arteriosus 4 3 7
Table I. Truncus arteriosus Type I, II treated with valved external conduit (Jan. 1, 1971, to Oct. 1, 1974) Hospital deaths Age (mo.)
Total*
No.
Per cent
< 24 24 =s 60 > 60
10 7
8 1
80 14
Totals
22
9
41
5t
-
-
*Exc!usive of 2 nonsurviving patients (12 months and 21 months old) requiring also repair or replacement of severely incompetent truncal valve. tOne was operated upon in 1969 and one in 1970.
Table II. Causes of death after repair of truncus arteriosus (January, 1971, to October, 1974) Year
No.
1974 1973
2 1 1
1972
2 1 1
1971 Total
1 2
Cause of death Acute cardiac failure* Acute cardiac failuret On CPAP with low cardiac output^ On CPAP with low cardiac outputt Contaminated indocyanine green dyet High RV pressure (proximal conduit kinking) Acute cardiac failure On CPAP with poorly treated low Clt
11
Legend: CPAP. Continuous positive airway pressure. RV, Right ventricular. CI. Cardiac index. "One of these patients had aortic valve repair. tThis patient had aortic valve replacement. ^Postoperative management inadequate by present standards.
operation was performed. Twelve patients 2 years of age or over were operated upon, with one (8 per cent) death (Table I). Since the operations were done over a 5 year period during which intraoperative and postoperative techniques for cardiac surgery in infants improved markedly, not surprisingly some of the patients who died (all but one of whom were less than 2 years old) were treated inadequately as judged by present standards. In our early experience we attempted to use as large a valved external conduit as possible. The resulting large aperture in the right ventricle and the mismatch between conduit and cardiac size probably impaired cardiac performance unduly. The right ventricular pressure was high after repair in one patient
The Journal of Thoracic and Cardiovascular Surgery
4 3 8 Appelbaum et al.
Table III. Mortality rate for pulmonary artery banding in patients with truncus arteriosus Hospi tat deaths
Author
Year
Total No.
Heilbrunnetal. 13 Takahashi etal. 14 Stark et al.15 Horsley et al. 16 Bernhard et a l . " Hunt et al.18 Oldhamet al.19 Tatooles and Miller20 Behrendt et al.21
1964 1968 1971 1970 1972 1971 1972 1973
3 12 5 9 9 2 4 2
1 6 4 3 5 0 3 1
33.3 50.0 80.0 33.3 55.6 0.0 75.0 50.0
1974
5
3
80.0
51
26
51.0
Totals
No.
Per cent
because of kinking of the conduit at its junction with the right ventricle. This does not now occur with proper tailoring and positioning of the conduit. Certain inadequacies in postoperative care are also evident (Table II). The 2 patients who died in 1971 were managed with continuous positive airway pressure and spontaneous ventilation beginning immediately after operation, and their low cardiac output was inadequately treated. The same is true of 2 patients operated upon in 1972 and one operated upon early in 1973. One infant who had been doing well died of hyperthermia and convulsions which began shortly after measurement of cardiac output with indocyanine green dye from a batch that subsequently was proved to be contaminated. Late results. The 2 infants (ages 8 months and 9 months at operation) who survived the early postoperative period are growing normally and are in good health 11 months and 43 months after operation. One of the 6 hospital survivors age 24 to 60 months at the time of operation cannot be traced. All the other 5 are growing well and are without symptoms in a mean follow-up period of 21 months (range 11 to 30 months). One of the 5 patients operated on when 5 years of age or older is untraced. Another (age 5 years at operation) continued to have marked cardiomegaly postoperatively. He had no shunt, mild elevation of pulmonary vascular resistance, and a 30 mm. Hg pressure gradient across the valved external conduit at cardiac catheterization 36 months postoperatively. He began about that time to have many episodes of ventricular tachycardia and was placed on quinidine. He died suddenly 42 months postoperatively. Autopsy showed calcification of the homograft conduit, a patent channel and good valve cusps, and intact repair of the
ventricular septal defect. No acute changes were seen. The other 3 patients who were 5 years of age or older at the time of repair have been followed 29, 45, and 72 months and are well without symptoms. Discussion With the development by McGoon and colleagues4 of a corrective operation for truncus arteriosus Type I, II, a group of operable patients ages 5 to 15 years has appeared for surgical treatment. Unfortunately, all available information indicates that they represent only a small fraction of infants born with truncus arteriosus. The autopsy studies of Collett and Edwards10 and the Van Praaghs11 strongly suggest that about 75 per cent of infants born with truncus arteriosus die in the first year of life. A number of these have severe truncal valve incompetence.12 Mair's1 data suggest that at least one third of those who survive the first year of life have severe pulmonary vascular disease and are inoperable by the age of 4 to 5 years. The long-term follow-up studies of children who have undergone repair of a large ventricular septal defect3 suggest that 30 per cent or more of patients undergoing repair of truncus arteriosus when more than 2 to 3 years of age will have postoperative progression of the pulmonary vascular disease and an unsatisfactory long-term result. Therefore, a policy of deferring repair of truncus arteriosus until age 4 to 5 years has many disadvantages, when viewed from the standpoint of the whole patient population born with truncus arteriosus. An initial banding of the pulmonary artery followed by later repair is theoretically attractive, in view of the present risks of correction in the first few years of life. Unfortunately, the risk of this palliative procedure in infants and young children with truncus arteriosus has been 51 per cent in series reported in the literature13-21 (Table III). This may be less than the true risk. Data from the literature indicate that at least 10 per cent of the patients surviving banding in infancy for truncus arteriosus die between 1 and 5 years of age. A further but unknown number are not protected by the procedure from the development of severe pulmonary vascular disease. McGoon and colleagues (personal communication) had two deaths among the last 22 patients with truncus arteriosus and banded pulmonary arteries upon whom they have operated, certainly an excellent record. We suspect others may have a higher mortality rate in this situation. From a synthesis of these facts and recognizing the pitfalls of such analyses, we estimate that of 100 infants undergoing banding for truncus arteriosus only about 30 will survive the procedure, the interval between the
Volume 71 Number 3 March, 1976
procedure and repair, and correction at age 4 to 5 years. Our small experience to date suggests that a hospital mortality rate of about 10 per cent should be realized when primary definitive repair is done at the age of about 2 to 3 years. The experience with patients with large ventricular septal defects indicates that patients operated upon at that age are unlikely to have progression of pulmonary vascular disease postoperatively. Our patients operated upon between 2 and 5 years of age have done well. Therefore, we believe the optimal age for elective repair in patients with truncus arteriosus is about 2 to 3 years. Unfortunately, the valved external conduit will probably need to be removed and replaced with a larger one in some patients at about 8 to 12 years of age. Our experience (unpublished data) and that of McGoon (unpublished data) indicate that this extracardiac operation can be accomplished safely. Delaying the primary operation until the patient is 4 to 5 years old probably does not obviate the need for later replacement of the conduit. The great problem is the fact that many patients with truncus arteriosus have intractable congestive heart failure or increasing pulmonary vascular resistance before the age of 2 years, and often in the first year of life. Barratt-Boyes 22 has repaired truncus arteriosus in 3 infants less than one year of age, with one death. The combined experience of Barratt-Boyes and ourselves with repair of truncus arteriosus in infants less than one year old in 13 patients with nine deaths, a mortality of 69 per cent. The late results in the 2 infants in our group who survived operation have been excellent to date. We believe that some of the hospital deaths in this group were related to inadequate intra- or postoperative management and that the mortality rate in this age group should be 50 per cent or less in the future. Therefore, we advise elective repair for truncus arteriosus at the age of about 2 to 3 years in patients without severe congestive heart failure, severe growth failure, or increasing pulmonary vascular disease. When these complications are intractable earlier in infancy, before the age of election, prompt primary repair is advised. Although we have had no success in infants with severe truncal valve incompetence associated with truncus arteriosus, the hopeless prognosis without operation indicates the need for continuing surgical efforts. REFERENCES 1 Mair, D. D., Ritter, D. G., Davis, G. D., Wallace, R. B., Danielson, G. K., and McGoon, D. G.: Selection of
Truncus arteriosus
A3 9
Patients With Truncus Arteriosus for Surgical Correction, Circulation 49: 144, 1974. 2 Poirier, R. A., Berman, M. A., and Stansel, H. C , Jr.: Current Status of the Surgical Treatment of Truncus Arteriosus, J. THORAC. CARCIOVASC. SURG. 69: 169,
1975. 3 DuShane, J. W., and Kirklin, J. W.: Late Results of the Repair of Ventricular Septal Defect on Pulmonary Vascular Disease, in Kirklin, J. W., editor: Advances in Cardiovascular Surgery, New York, 1973, Grune & Stratton, Inc., p. 9. 4 McGoon, D. C , Rastelli, G. C , and Ongley, P. A.: An Operation for the Correction of Truncus Arteriosus, J. A. M. A. 205: 69, 1968. 5 Kouchoukos, N. T., Barcia, A., Bargeron, L. M., and Kirklin, J. W.: Surgical Treatment of Congenital Pulmonary Atresia With Ventricular Septal Defect, J. THORAC. CARDIOVASC. SURG. 61: 70, 1971.
6 Bowman, F. O., Jr., Hancock, W. D., and Malm, J. R.: A Valve-Containing Dacron Prosthesis, Arch. Surg. 107: 724, 1973. 7 Barratt-Boyes, B. G., Simpson, M., and Neutze, J. M.: Intracardiac Surgery in Neonates and Infants Using Deep Hypothermia, Circulation 43, 44: 25, 1971 (Suppl. I). 8 Kirklin, J. W., Pacifico, A. D., Hannah, H., Ill, and Allarde, R. R.: Primary Definitive Intracardiac Operations in Infants: Intraoperative Support Techniques, in Kirklin, J. W. editor: Advances in Cardiovascular Surgery, New York, 1973, Grune & Stratton, Inc., p. 85. 9 Stewart, S., Ill, Edmunds, L. H., Jr., Kirklin, J. W., and Allarde, R. R.: Spontaneous Breathing With Continuous Positive Airway Pressure After Open Intracardiac Operations in Infants, J. THORAC. CARDIOVASC. SURG. 65: 37,
1973. 10 Collett, R. W., and Edwards, J. E.: Persistent Truncus Arteriosus: A Classification According to Anatomic Types, Surg. Clin. North Am. 29: 1245, 1949. 11 Van Praagh, R., and Van Praagh, S.: The Anatomy of Common Aorticopulmonary Trunk (Truncus Arteriosus Communis) and Its Embryologic Implications: A Study of 57 Necropsy Cases, Am. J. Cardiol. 16: 406, 1965. 12 Gelband, H., Van Meter, S., and Gersony, W. M.: Truncal Valve Abnormalities in Infants With Persistent Truncus Arteriosus: A Clinico-pathologic Study, Circulation 45: 397, 1972. 13 Heilbrunn, A., Kittle, C. F., and Diehl, A. M.: Pulmonary Arterial Banding in the Treatment of Truncus Arteriosus, Circulation 29: 102, 1964 (Suppl.). 14 Takahashi, M., Lurie, P. R., Petry, E. L., and King, H.: Clinical and Hemodynamic Effects of Pulmonary Artery Banding, Am. J. Cardiol. 21: 174, 1968. 15 Stark, J., Hucin, B., Aberdeen, E., and Waterston, D. J.: Cardiac Surgery in the First Year of Life: Experience With 1,049 Operations, Surgery 69: 483, 1971. 16 Horsley, B. L., Zuberbuhler, J. R., and Bahnson, H. T.: Factors Influencing Survival After Banding of the Pulmonary Artery, Arch. Surg. 101: 776, 1970.
44 0
Appelbaum
et al.
17 Bernhard, W. F., Litwin, S. B., Williams, W. W., Jones, J. E., and Gross, R. E.: Recent Results of Cardiovascular Surgery in Infants in the First Year of Life, Am. J. Surg. 123: 451, 1972. 18 Hunt, C. E., Formanek, G., Levine, M. A., Castaneda, A., and Moller, J. H.: Banding of the Pulmonary Artery: Results in 111 Children, Circulation 43: 395, 1971. 19 Oldham, H. N., Jr., Kakos, G. S., Jarmakani, M. M., and Sabiston, D. C , Jr.: Pulmonary Artery Banding in Infants With Complex Congenital Heart Defects, Ann. Thorac. Surg. 13: 342, 1972. 20 Tatooles, C. J., and Miller, R. A.: Palliative Surgery in
The Journal of Thoracic and Cardiovascular Surgery
Infants With Congenital Heart Disease, Progr. Cardiovasc. Dis. 15: 331, 1973. 21 Behrendt, D. M., Kirsh, M. M., Stern, A., Sigmann, J., Perry, B., and Sloan, H.: The Surgical Therapy for Pulmonary Artery-Right Ventricular Discontinuity, Ann. Thorac. Surg. 18: 122, 1974. 22 Barratt-Boyes, B. G.: Complete Correction of Cardiovascular Malformations in the First Two Years of Life Using Profound Hypothermia, in Barratt-Boyes, B. G., Neutze, J. M., and Harris, E. A., editors: Heart Disease in Infancy: Diagnosis and Surgical Treatment, Edinburgh and London, 1973, Churchill Livingstone, p. 25.
Azai Appelbaum, M.D., Lionel M. Bargeron, Jr., M.D., Albert D. Pacifico, M.D., and John W. Kirklin, M.D., Birmingham, Ala.
M air and colleagues1 recently analyzed their large experience at the Mayo Clinic with the investigation and surgical treatment of patients with truncus arteriosus Type I, II. Although their group of patients treated surgically included none under one year of age, they alluded specifically to the problems of management of this malformation in infants. Responding to the same problem, Poirier and colleagues2 recently suggested a management program based on pulmonary arterial banding for infants in congestive heart failure and deferral of intracardiac repair until the age of 5 years. Mair and colleagues suggested also that repair be deferred until age 4 years unless there is significant increase in pulmonary vascular resistance before that time. Since January, 1971, we have practiced primary definitive repair for infants and small children less than 5 years of age with truncus arteriosus. Repair was advised electively at age 2 to 3 years but was done early From the Departments of Surgery and Pediatrics, The University of Alabama Medical School and Medical Center, Birmingham, Ala. 35294. Received for publication June 27, 1975. Address for reprints: John W. Kirklin, M.D., Department of Surgery, University Station, Birmingham, Ala. 35294.
436
in infancy when operative intervention seemed urgently indicated because of congestive heart failure, severe growth failure, or increasing pulmonary vascular resistance. This management program was based on the strong likelihood of patients with this malformation dying in infancy; the fact that an unknown but significant number of patients with truncus arteriosus have severe pulmonary vascular disease and are inoperable by the age of 5 years1; and our extrapolation of data3 showing that the long-term results in patients with large ventricular septal defects and severe pulmonary hypertension are best when the child is less than about 2 to 3 years of age at the time of repair. The hospital mortality rate in infants has been high, but the over-all results are probably better than those of banding followed by later repair, and they should improve with the knowledge and experience gained during the past 5 years. The results in children age 2 or more at the time of operation have been satisfactory. Clinical material and methods Twenty-two patients with truncus arteriosus, Type I, II, underwent corrective surgery at The University of Alabama Medical Center between Jan. 1, 1971, and Oct. 1, 1974. Two children over 5 years of age were operated upon before that date. No patients underwent
Volume 71 Number 3 March, 1976
pulmonary arterial banding during that period. No patients were denied operation because of young age, small size, presence of truncal valve incompetence, or severity of congestive heart failure, but a few older patients were advised against surgery because they had severe pulmonary vascular disease. The operation was that described by McGoon, Rastelli, and Ongley.4 Many technical details of the operation varied as experience grew. In the first 3 cases we used an aortic homograft with its retained valve as the valved external conduit. Subsequently, we used a composite valved conduit of Dacron containing within it a homograft valve5 or more recently a heterograft valve.6 In the early cases, in infants and small children, as large a valved external conduit as could be accommodated was selected; however, in the past few years one whose size was harmonious with the heart's size has been used. In the first 2 years of our experience, infants less than 2 years of age were operated upon by the Barratt-Boyes-Kyoto technique7 of profound hypothermia, limited cardiopulmonary bypass, and total circulatory arrest. Subsequently, we8 used profound hypothermia with low-flow perfusion. The postoperative management was less intense during the early part of the experience than it was in the last 24 months of it. In general, blood was infused to maintain left atrial pressure at about 14 mm. Hg. Catecholamines were used where cardiac output seemed less than desired. All patients remained intubated for at least 24 hours postoperatively. Many of the infants and small children breathed spontaneously with continuous positive airway pressure9 during the early years, but in the last 2 years all have been managed initially with controlled ventilation. Autopsy was done in 7 of the 11 patients who died. The cause of death was assigned after retrospective review of the operative note, notes and data from the postoperative period, and the report of the autopsy. Follow-up was by personal examination or correspondence with referring physicians, with the date of inquiry being April 1, 1975. Results Hospital mortality rate. Two infants underwent repair of the truncus arteriosus and replacement of repair of a severely incompetent truncal valve, and both died early postoperatively. Ten additional infants less than 2 years of age had corrective surgery, 8 of whom died in hospital. Both infants less than 6 months (2 and 5 months) old at the time of operation died. Three of 5 operated upon between 6 to 12 months of age died, the surviving infants being 8 and 9 months old when
Truncus arteriosus 4 3 7
Table I. Truncus arteriosus Type I, II treated with valved external conduit (Jan. 1, 1971, to Oct. 1, 1974) Hospital deaths Age (mo.)
Total*
No.
Per cent
< 24 24 =s 60 > 60
10 7
8 1
80 14
Totals
22
9
41
5t
-
-
*Exc!usive of 2 nonsurviving patients (12 months and 21 months old) requiring also repair or replacement of severely incompetent truncal valve. tOne was operated upon in 1969 and one in 1970.
Table II. Causes of death after repair of truncus arteriosus (January, 1971, to October, 1974) Year
No.
1974 1973
2 1 1
1972
2 1 1
1971 Total
1 2
Cause of death Acute cardiac failure* Acute cardiac failuret On CPAP with low cardiac output^ On CPAP with low cardiac outputt Contaminated indocyanine green dyet High RV pressure (proximal conduit kinking) Acute cardiac failure On CPAP with poorly treated low Clt
11
Legend: CPAP. Continuous positive airway pressure. RV, Right ventricular. CI. Cardiac index. "One of these patients had aortic valve repair. tThis patient had aortic valve replacement. ^Postoperative management inadequate by present standards.
operation was performed. Twelve patients 2 years of age or over were operated upon, with one (8 per cent) death (Table I). Since the operations were done over a 5 year period during which intraoperative and postoperative techniques for cardiac surgery in infants improved markedly, not surprisingly some of the patients who died (all but one of whom were less than 2 years old) were treated inadequately as judged by present standards. In our early experience we attempted to use as large a valved external conduit as possible. The resulting large aperture in the right ventricle and the mismatch between conduit and cardiac size probably impaired cardiac performance unduly. The right ventricular pressure was high after repair in one patient
The Journal of Thoracic and Cardiovascular Surgery
4 3 8 Appelbaum et al.
Table III. Mortality rate for pulmonary artery banding in patients with truncus arteriosus Hospi tat deaths
Author
Year
Total No.
Heilbrunnetal. 13 Takahashi etal. 14 Stark et al.15 Horsley et al. 16 Bernhard et a l . " Hunt et al.18 Oldhamet al.19 Tatooles and Miller20 Behrendt et al.21
1964 1968 1971 1970 1972 1971 1972 1973
3 12 5 9 9 2 4 2
1 6 4 3 5 0 3 1
33.3 50.0 80.0 33.3 55.6 0.0 75.0 50.0
1974
5
3
80.0
51
26
51.0
Totals
No.
Per cent
because of kinking of the conduit at its junction with the right ventricle. This does not now occur with proper tailoring and positioning of the conduit. Certain inadequacies in postoperative care are also evident (Table II). The 2 patients who died in 1971 were managed with continuous positive airway pressure and spontaneous ventilation beginning immediately after operation, and their low cardiac output was inadequately treated. The same is true of 2 patients operated upon in 1972 and one operated upon early in 1973. One infant who had been doing well died of hyperthermia and convulsions which began shortly after measurement of cardiac output with indocyanine green dye from a batch that subsequently was proved to be contaminated. Late results. The 2 infants (ages 8 months and 9 months at operation) who survived the early postoperative period are growing normally and are in good health 11 months and 43 months after operation. One of the 6 hospital survivors age 24 to 60 months at the time of operation cannot be traced. All the other 5 are growing well and are without symptoms in a mean follow-up period of 21 months (range 11 to 30 months). One of the 5 patients operated on when 5 years of age or older is untraced. Another (age 5 years at operation) continued to have marked cardiomegaly postoperatively. He had no shunt, mild elevation of pulmonary vascular resistance, and a 30 mm. Hg pressure gradient across the valved external conduit at cardiac catheterization 36 months postoperatively. He began about that time to have many episodes of ventricular tachycardia and was placed on quinidine. He died suddenly 42 months postoperatively. Autopsy showed calcification of the homograft conduit, a patent channel and good valve cusps, and intact repair of the
ventricular septal defect. No acute changes were seen. The other 3 patients who were 5 years of age or older at the time of repair have been followed 29, 45, and 72 months and are well without symptoms. Discussion With the development by McGoon and colleagues4 of a corrective operation for truncus arteriosus Type I, II, a group of operable patients ages 5 to 15 years has appeared for surgical treatment. Unfortunately, all available information indicates that they represent only a small fraction of infants born with truncus arteriosus. The autopsy studies of Collett and Edwards10 and the Van Praaghs11 strongly suggest that about 75 per cent of infants born with truncus arteriosus die in the first year of life. A number of these have severe truncal valve incompetence.12 Mair's1 data suggest that at least one third of those who survive the first year of life have severe pulmonary vascular disease and are inoperable by the age of 4 to 5 years. The long-term follow-up studies of children who have undergone repair of a large ventricular septal defect3 suggest that 30 per cent or more of patients undergoing repair of truncus arteriosus when more than 2 to 3 years of age will have postoperative progression of the pulmonary vascular disease and an unsatisfactory long-term result. Therefore, a policy of deferring repair of truncus arteriosus until age 4 to 5 years has many disadvantages, when viewed from the standpoint of the whole patient population born with truncus arteriosus. An initial banding of the pulmonary artery followed by later repair is theoretically attractive, in view of the present risks of correction in the first few years of life. Unfortunately, the risk of this palliative procedure in infants and young children with truncus arteriosus has been 51 per cent in series reported in the literature13-21 (Table III). This may be less than the true risk. Data from the literature indicate that at least 10 per cent of the patients surviving banding in infancy for truncus arteriosus die between 1 and 5 years of age. A further but unknown number are not protected by the procedure from the development of severe pulmonary vascular disease. McGoon and colleagues (personal communication) had two deaths among the last 22 patients with truncus arteriosus and banded pulmonary arteries upon whom they have operated, certainly an excellent record. We suspect others may have a higher mortality rate in this situation. From a synthesis of these facts and recognizing the pitfalls of such analyses, we estimate that of 100 infants undergoing banding for truncus arteriosus only about 30 will survive the procedure, the interval between the
Volume 71 Number 3 March, 1976
procedure and repair, and correction at age 4 to 5 years. Our small experience to date suggests that a hospital mortality rate of about 10 per cent should be realized when primary definitive repair is done at the age of about 2 to 3 years. The experience with patients with large ventricular septal defects indicates that patients operated upon at that age are unlikely to have progression of pulmonary vascular disease postoperatively. Our patients operated upon between 2 and 5 years of age have done well. Therefore, we believe the optimal age for elective repair in patients with truncus arteriosus is about 2 to 3 years. Unfortunately, the valved external conduit will probably need to be removed and replaced with a larger one in some patients at about 8 to 12 years of age. Our experience (unpublished data) and that of McGoon (unpublished data) indicate that this extracardiac operation can be accomplished safely. Delaying the primary operation until the patient is 4 to 5 years old probably does not obviate the need for later replacement of the conduit. The great problem is the fact that many patients with truncus arteriosus have intractable congestive heart failure or increasing pulmonary vascular resistance before the age of 2 years, and often in the first year of life. Barratt-Boyes 22 has repaired truncus arteriosus in 3 infants less than one year of age, with one death. The combined experience of Barratt-Boyes and ourselves with repair of truncus arteriosus in infants less than one year old in 13 patients with nine deaths, a mortality of 69 per cent. The late results in the 2 infants in our group who survived operation have been excellent to date. We believe that some of the hospital deaths in this group were related to inadequate intra- or postoperative management and that the mortality rate in this age group should be 50 per cent or less in the future. Therefore, we advise elective repair for truncus arteriosus at the age of about 2 to 3 years in patients without severe congestive heart failure, severe growth failure, or increasing pulmonary vascular disease. When these complications are intractable earlier in infancy, before the age of election, prompt primary repair is advised. Although we have had no success in infants with severe truncal valve incompetence associated with truncus arteriosus, the hopeless prognosis without operation indicates the need for continuing surgical efforts. REFERENCES 1 Mair, D. D., Ritter, D. G., Davis, G. D., Wallace, R. B., Danielson, G. K., and McGoon, D. G.: Selection of
Truncus arteriosus
A3 9
Patients With Truncus Arteriosus for Surgical Correction, Circulation 49: 144, 1974. 2 Poirier, R. A., Berman, M. A., and Stansel, H. C , Jr.: Current Status of the Surgical Treatment of Truncus Arteriosus, J. THORAC. CARCIOVASC. SURG. 69: 169,
1975. 3 DuShane, J. W., and Kirklin, J. W.: Late Results of the Repair of Ventricular Septal Defect on Pulmonary Vascular Disease, in Kirklin, J. W., editor: Advances in Cardiovascular Surgery, New York, 1973, Grune & Stratton, Inc., p. 9. 4 McGoon, D. C , Rastelli, G. C , and Ongley, P. A.: An Operation for the Correction of Truncus Arteriosus, J. A. M. A. 205: 69, 1968. 5 Kouchoukos, N. T., Barcia, A., Bargeron, L. M., and Kirklin, J. W.: Surgical Treatment of Congenital Pulmonary Atresia With Ventricular Septal Defect, J. THORAC. CARDIOVASC. SURG. 61: 70, 1971.
6 Bowman, F. O., Jr., Hancock, W. D., and Malm, J. R.: A Valve-Containing Dacron Prosthesis, Arch. Surg. 107: 724, 1973. 7 Barratt-Boyes, B. G., Simpson, M., and Neutze, J. M.: Intracardiac Surgery in Neonates and Infants Using Deep Hypothermia, Circulation 43, 44: 25, 1971 (Suppl. I). 8 Kirklin, J. W., Pacifico, A. D., Hannah, H., Ill, and Allarde, R. R.: Primary Definitive Intracardiac Operations in Infants: Intraoperative Support Techniques, in Kirklin, J. W. editor: Advances in Cardiovascular Surgery, New York, 1973, Grune & Stratton, Inc., p. 85. 9 Stewart, S., Ill, Edmunds, L. H., Jr., Kirklin, J. W., and Allarde, R. R.: Spontaneous Breathing With Continuous Positive Airway Pressure After Open Intracardiac Operations in Infants, J. THORAC. CARDIOVASC. SURG. 65: 37,
1973. 10 Collett, R. W., and Edwards, J. E.: Persistent Truncus Arteriosus: A Classification According to Anatomic Types, Surg. Clin. North Am. 29: 1245, 1949. 11 Van Praagh, R., and Van Praagh, S.: The Anatomy of Common Aorticopulmonary Trunk (Truncus Arteriosus Communis) and Its Embryologic Implications: A Study of 57 Necropsy Cases, Am. J. Cardiol. 16: 406, 1965. 12 Gelband, H., Van Meter, S., and Gersony, W. M.: Truncal Valve Abnormalities in Infants With Persistent Truncus Arteriosus: A Clinico-pathologic Study, Circulation 45: 397, 1972. 13 Heilbrunn, A., Kittle, C. F., and Diehl, A. M.: Pulmonary Arterial Banding in the Treatment of Truncus Arteriosus, Circulation 29: 102, 1964 (Suppl.). 14 Takahashi, M., Lurie, P. R., Petry, E. L., and King, H.: Clinical and Hemodynamic Effects of Pulmonary Artery Banding, Am. J. Cardiol. 21: 174, 1968. 15 Stark, J., Hucin, B., Aberdeen, E., and Waterston, D. J.: Cardiac Surgery in the First Year of Life: Experience With 1,049 Operations, Surgery 69: 483, 1971. 16 Horsley, B. L., Zuberbuhler, J. R., and Bahnson, H. T.: Factors Influencing Survival After Banding of the Pulmonary Artery, Arch. Surg. 101: 776, 1970.
44 0
Appelbaum
et al.
17 Bernhard, W. F., Litwin, S. B., Williams, W. W., Jones, J. E., and Gross, R. E.: Recent Results of Cardiovascular Surgery in Infants in the First Year of Life, Am. J. Surg. 123: 451, 1972. 18 Hunt, C. E., Formanek, G., Levine, M. A., Castaneda, A., and Moller, J. H.: Banding of the Pulmonary Artery: Results in 111 Children, Circulation 43: 395, 1971. 19 Oldham, H. N., Jr., Kakos, G. S., Jarmakani, M. M., and Sabiston, D. C , Jr.: Pulmonary Artery Banding in Infants With Complex Congenital Heart Defects, Ann. Thorac. Surg. 13: 342, 1972. 20 Tatooles, C. J., and Miller, R. A.: Palliative Surgery in
The Journal of Thoracic and Cardiovascular Surgery
Infants With Congenital Heart Disease, Progr. Cardiovasc. Dis. 15: 331, 1973. 21 Behrendt, D. M., Kirsh, M. M., Stern, A., Sigmann, J., Perry, B., and Sloan, H.: The Surgical Therapy for Pulmonary Artery-Right Ventricular Discontinuity, Ann. Thorac. Surg. 18: 122, 1974. 22 Barratt-Boyes, B. G.: Complete Correction of Cardiovascular Malformations in the First Two Years of Life Using Profound Hypothermia, in Barratt-Boyes, B. G., Neutze, J. M., and Harris, E. A., editors: Heart Disease in Infancy: Diagnosis and Surgical Treatment, Edinburgh and London, 1973, Churchill Livingstone, p. 25.