Management of Infants with Large Ventricular Septa1 Defects

THE ANNALS OF THORACIC SURGERY Journal of The Society of Thoracic Surgeons and the Southern Thoracic Surgical Association VOLUME 15 * NUMBER * FEBRUARY 1973 Management of Infants with Large Ventricular Septa1 Defects Results with Two-Stage Surgical Treatment James Henry, M.D., Samuel Kaplan, M.D., James A. Helmsworth, M.D., and J. Tracy Schreiber, M.D. ABSTRACT Forty-two infants with large ventricular septal defects underwent pulmonary arterial banding. There have been 7 postoperative deaths in the entire series but only 1 death in 9 procedures during the last five years, and there has been 1 late death after operation. Another child suffered a severe cerebrovascular accident and hemiparesis 15 months following the banding procedure. Twelve children underwent pulmonary arterial debanding and ventricular septal defect closure. There were postoperative deaths, both related to difficulty in removing the band and in pulmonary artery reconstruction. In this study, the mortality with the debanding procedure was directly related to removal of the band and not to the unusual anatomy of the septal defect. Because of the total morbidity and mortality with the staged surgical approach in infants with large ventricular septal defects, we advocate early primary closure of the defect. nfants with a large ventricular septal defect (VSD) that is unresponsive to vigorous medical treatment continue to present a difficult and controversial problem in management [1, 15, 11. From 1961 to 1970 we employed a staged surgical approach in these patients consisting of pulmonary arterial banding followed by a later corrective procedure. The alternative surgical approach is primary closure of the VSD [l-, 11, 171. The purpose of this study is to evaluate our results with the staged procedure and to provide a basis upon which to compare the results with those of From the Departments of Pediatrics and Surgery, College of Medicine, University of Cincinnati and the Cincinnati Children s Hospital, Cincinnati, Ohio. Supported in part by U.S. Public Health Service Grant No. HE0578-06 and by the Cincinnati Children s Heart Association. Accepted for publication July 18, 197. Address reprint requests to Dr. Kaplan, Division of Cardiology, Children s Hospital, Cincinnati, Ohio 59.

HENRY ET AL. primary closure. For reasons of comparison, the study was limited to infants with isolated VSD or with VSD associated with atrial septal defect or patent ductus arteriosus. In addition, two complications of the staged approach are presented which, to our knowledge, have not previously been reported. These are (1) fatal complications related to debanding and pulmonary artery reconstruction and () late occurrence of a cerebrovascular accident following pulmonary arterial banding. Clinical Material This study included all infants (less than 1 months of age) who underwent pulmonary arterial banding in our institution up to December 31, 1970, and in whom the dominant cardiac lesion was judged to be a VSD. Infants who were banded for lesions other than VSD plus atrial septal defect or patent ductus arteriosus were excluded. Forty-two infants met these criteria, of whom 9 had an associated left-to-right shunt at the atrial level and 9 had an associated patent ductus arteriosus. All patients were evaluated preoperatively by history and physical examination, electrocardiography, chest roentgenogram, and cardiac catheterization. Table 1 summarizes information obtained in the patients prior to banding. Intractable congestive heart failure was present in 38 of the patients and was the major indication for pulmonary arterial banding. Moderate to severe pulmonary hypertension was present in all infants. Secondary considerations for operation were failure to thrive and recurrent severe lower respiratory tract infections. Sixteen infants were less than 3 months of age and 33 of the patients were less than 6 months of age at the time of banding. Twenty-one infants weighed less than kg. The remainder weighed between.0 and 6. kg. The smallest patient weighed 1.8 kg. Debanding and repair of a VSD and repair of an atrial septal defect, when these lesions were indeed present, has been performed on 1 patients to date. Nine are included in the pulmonary arterial banding group. Three are not included because they were banded after 1 months of age. Pulmonary arterial banding was performed by placing a circumferential cloth band (usually Dacron graft material) on the main pulmonary artery. The band was constricted until the mean systemic arterial pressure rose by approximately 10 mm. Hg. This has been the major criterion of optimal pulmonary artery constriction. Other criteria used early in our experience included constriction until the outside diameter of the main pulmonary artery was reduced to approximately one-third its original diameter or constriction sufficient to reduce the mean pulmonary artery pressure distal to the band to approximately one-half the control pressure. In some instances bradycardia followed these procedures. When this occurred, the band was 110 THE ANNALS OF THORACIC SURGERY

Mnnngement of Infants with Large Ventricular Defects TABLE 1. CARDIAC CATHETERIZATION DATA PRIOR TO PULMONARY ARTERY BANDING Pulmonary Pulmonary Artery Artery Mean Pulmonary- Patient Weight Age Pressure Pressure to-systemic Associated Operative No. (kg.) (mo.) (mm. Hg) (mm. Hg) Flow Ratio Defects Mortality 35 10: 1 1 3 5 6 7 8 9 10 11 1 13 1 15 16 17 18 19 0 1 3" 5 6 7 8 9 30 31 3 33 3 35 36 37 38 39 0 1 ' 1.8 3.0 3.1 3.1 3.1 3.3 3. 3. 3.5 3.5 3.5 3.6 3.6 3.6 3.6 3.6 3.7 3.7 3.7 3.8 3.8.0.0.1..3.3.5.7.8.9.9 5.0 5.1 5.1 5. 5.3 5.6 5.8 5.9 6.0 6. 1 1 1.5 5 3.5 1.5 1 6.5 1.5 5 5.5.5 5 7 5.5.5 7.5 7 1 11 10.5 11 10 58/8 91 1" 5/7 76/16 53/6 8010 66/5 1116 6/7 6015 79/13" 86/3 6 / 9 8/3 7918" 116117" 77/5 75/1 51 17 601 11 63/0 86/ 5/ 901 98 / 30 51/1 581 1 90/16 581 13 63/3 61 19 75/5 18150 85/5 701 60115 8013 9018 6519" 8/18 85/19 55/33 3 5 3 53 5 90 1 5 50 7 55 5 5 3 30 5 8 37 5 58 1 31 3 33 37 36 0 68 65 1 33 63 5 0 0 5. : 1.8 : 1 6: 1 1.6: 1 7: 1 7: 1.5 : 1. : 1.6 : 1.5 : 1 b.6: 1 7. : 1 8.8: 1.8 : 1 5.3 : 1 5.8 : 1 :l 5: 1 5.5 : 1 7.5 : 1 3: 1 5:l.5 : 1 3.8 : 1 3. : 1 6: I 3.: 1.5 : 1 5: 1 : 1 3.6 : 1.7 : 1.8 : 1 1.6 : 1.5 : 1 Yes Yes Yes Yes Yes Yes Yes "Right ventricular pressure given because pulmonary artery was not entered. bpulmonary arterial blood sample, 98% oxygen saturation. OVSD closed spontaneously. = atrial septa1 defect; = patent ductus arteriosus. VOL. 15, NO., FEBRUARY, 1973 111

HENRY ET AL. released slightly until the vital signs stabilized. When a patent ductus arteriosus was present, it was divided prior to pulmonary arterial banding. Debanding was performed during cardiopulmonary bypass by cutting the band and carefully dissecting it from the pulmonary artery if it could be removed easily. In most patients it was removed completely. Digital dilation was attempted to expand the constricted area. If this was not successful, plastic reconstruction of the pulmonary artery using a pericardial graft was performed. Ventricular septa1 defect closure was accomplished with a Teflon patch using interrupted buttressed sutures. Results PULMONARY ARTERIAL BANDING The immediate postoperative mortality (hospital deaths) is shown in Figure 1. There were 7 deaths in procedures. The total group has been divided into those operated upon in an early period (1961-1965) and those operated upon in a later period (1966-1970). The hospital mortality was 6y0 in the early period and 3y0 in the later period. In this study, age, weight, and associated cardiac defects were not important determinants of immediate postoperative mortality (see Table 1). COURSE FOLLOWING BANDING Thirty-five patients survived the immediate postoperative period (Table ). One was lost to follow-up. One infant died two months postoperatively with overwhelming pneumonia. Two children continued to exhibit signs and symptoms of congestive heart failure that required readmission to the hospital. One child continued to experience recurrent episodes of lower respiratory tract infection. Thirteen patients became cyanotic. One cyanotic child suffered a severe cerebrovascular accident fifteen months following the banding procedure and has a residual right hemiparesis. Twenty-nine patients (including 1 of the 13 who developed cyanosis) exhibited a considerable reduction in morbidity. Signs and symptoms of Number of Patients Hospital Deaths FIG. 1. Hospital deaths following pulmonary arterial banding. Early and late experiences are shown. 1961-1965 1966-1970 11 THE ANNALS OF THORACIC SURGERY

Management of Infants with Large Ventricular Defects TABLE. COURSE OF 35 PATIENTS FOLLOWING PULMONARY ARTERY BANDING Course No. of Patients Lost to follow-up 1 Late death 1 Recurrent CHF Recurrent pneumonia 1 CVA 1 Well 9 Twelve patients have cyanosis. CHF = congestive heart failure; CVA = cerebrovascular accident. congestive heart failure either disappeared following pulmonary arterial banding or were easily controlled on an outpatient basis. The patients no longer experienced recurrences of pneumonia, and they exhibited improved growth and development. Improved growth and development following pulmonary arterial banding has been previously documented by others [S, 01. CORRECTIVE OPERATION Twelve patients were reevaluated by cardiac catheterization and angiocardiography prior to debanding (Table 3). Left-to-right shunting could not be demonstrated by oximetry or by selective left ventriculography in patients. Spontaneous closure of the VSD was confirmed in these patients at the time of debanding by inspection through a right ventriculotomy. Infundibular stenosis was demonstrated by pressure gradient and angiocardiography (Fig. ) in 5 patients. Acquired branch stenosis of the right or both pulmonary arteries secondary to distal migration of the band (Fig. 3) occurred in patients. Corrective operations including debanding, closure of the VSD when present, and closure of an atrial septa1 defect when present have been accomplished in 1 patients to date. There were postoperative deaths. Pulmonary Artery Reconstruction. The major complication of the corrective procedure was related to reconstruction of the main pulmonary artery. In patients the band was removed easily, leaving a systolic gradient of 0 mm. Hg or less. In 8 of the 1 the pulmonary artery was reconstructed using a pericardial patch graft. In 3 of these patients reconstruction was complicated by friability of the pulmonary artery at the level of the band site and distal to it. Two of the 3 died immediately postoperatively. In all 3 patients Dacron graft material had been utilized in the pulmonary arterial banding. Right Ventricular Outflow Tract Obstruction. Infundibular resection was performed in 5 patients. In 1 a pericardial patch was utilized to enlarge the right ventricular outflow tract. Significant obstruction at the pulmonic valve was not recognized in any of our patients. VOL. 15, NO., FEBRUARY, $173 113

cl 3 > z * t: 8 TABLE 3. PULMONARY ARTERY DEBANDING EXPERIENCE IN 1 PATIENTS Banding of Band Patch Infund. Patch Obstruct- Closure RV PA PA PA-RV E E! n s t z Pericar- Spon- Ageat Duration dial Outflow Band taneous Pressures (mm. Hg) Outcome (follow- Patient" (mo.) (yr.) MPA Resect. RV ing PAS VSD Syst. Phasic Mean Gradient up period) 13 1 J J RPA 38 3/10 17 A&W (50mo.) B.D. 6 both PAS 5 318 15 A&W (50 mo.) R.C. 15 5 55 55/ 33 0 A&W (1 mo.) 5 J J /18 0 18 A&W (39mo.) 37 11 5 J 65 5/0 30 0 A&W (3mo.) 8 5 5.5 J 35 b 5/10 16 10 CHB (3mo.) 3 J J...b Died postop. b 10 5.5 J 35 35/15 0 Died postop. S.W. 17 6 J J J RPA 30 0110 15 10 A&W (1 mo.) 1 5.5 J RPA 0 018 15 0 A&W (6mo.) 15 J 30 0/ 10 13 10 A&W (5mo.) 3 5 J J 13 3/10 1 0 A&W (3 mo.) "Patient number corresponds to that in Table 1: patients banded after 1 months (not included in Table 1) are identified by initials. bcondition of patient precluded measurement. MPA = main pulmonary artery: Infund. Resect. = infundibular resection: RV = right ventricle: PA = pulmonary artery: VSD = ventricular septa1 defect: RPA = right pulmonary artery: A&W = alive and well: CHB = complete heart block.

Management of Znfants with Large Ventricular Defects FIG.. Lateral view of a right ventriculogram in a patient with VSD following pulmonary arterial banding. The top arrows indicate the area of constriction of the main pulmonary artery. The bottom arrows indicate infundibular obstruction. This patient required infundibular resection. (PA = pulmonary artery; RV = right ventricle.) Ventricular Septa1 Defect Closure. The VSD was closed with a Teflon patch in 10 patients. Five were closed through the right atrium and 5 through the right ventricle. The locations of the VSD were not unusual. Neither muscular nor multiple VSDs were encountered. One patient FIG. 3. Pulmonary arteriogram illustrating acquired branch stenosis of the right pllmonary artery in a patient with distal migration of the pulmonary arterial band. Arrows indicate the site of stenosis. (PA = pulmonary artery; RV = right ventricle.) VOL. 15, NO., FEBRUARY, 1973 115

HENRY ET AL. developed complete heart block and has a permanently implanted pacemaker. There was no other episode of heart block, temporary or permanent. All patients have complete right bundle-branch block postoperatively whether or not they had a ventriculotomy. None have abnormal left axis deviation. Comment Pulmonary arterial banding is an effective method for temporary treatment of infants with congestive heart failure and pulmonary hypertension secondary to large VSD [8, 9, 1, 01. Recent reports of operative mortality from pulmonary arterial banding range from 8 to 6y0 [5, 8, 9, 1, 18, 01. High mortality figures (15 to 6Y0) were generally associated with additional complicated cardiovascular defects [9, 1, 181, young patients [1, 01, and early surgical experience [8, 01. Our recent mortality rate (3y0 in the period 1965-1970; Fig. 1) undoubtedly reflects the selection of patients to include only those with relatively minor associated defects. Due to the severe disability of the infants who are candidates for pulmonary artery banding and to the often urgent circumstances of the operative procedure, it is unlikely that operative mortality can be lowered further. Late deaths, continuing congestive heart failure, and continuing recurrent pneumonia following pulmonary artery banding are not unique to this study [5, 8, 1, 181. We are unaware of a previous report of a cerebrovascular accident following pulmonary artery banding, although its occurrence in a patient with VSD, pulmonary stenosis, and right-to-left shunting is not unexpected. Combined reports of mortality with debanding compiled by Stark and his associates [19] plus the published series by Stark [19] and Hunt [1] and their co-workers are shown in Table and indicate that the mortality rate for closure of VSD, when present, and debanding is 0ojb. The mortality reported by these investigators was attributed to difficulty with repair of multiple VSDs and to postoperative complete heart block. Stark and associates [19] note that no deaths were attributed to pulmonary artery TABLE. POSTOPERATIVE MORTALITY WITH PULMONARY ARTERY DEBANDINC" Authors No. of Patients No. of Deaths Stark et al. [19] 8 7 Compiled by Stark et al. [19] 71 I5 Hunt et al. [1] 3 Henry et al. [present report] 1 Total 135 7 "Overall mortality rate, 0'70. 116 THE ANNALS OF THORACIC SURGERY

Management of Infants with Large Ventricular Defects reconstruction. In our patients, problems arising from reconstruction of the pulmonary artery were considered to be responsible for the postoperative deaths. Difficulty in reconstruction' of the pulmonary artery was encountered in 3 patients and was caused by friability of the pulmonary artery at the site of the band. Hunt and co-workers [1] noted persistent narrowing of the pulmonary artery with a significant gradient in children and significant valvular pulmonic stenosis in another. Valvular pulmonic stenosis was attributed to trauma caused by the band being placed close to the pulmonary valve. Necrosis of the trunk of the pulmonary artery (at the band site) has been reported [16] and was attributed to arterial wall ischemia secondary to occlusion of the vasovasorum of the main pulmonary artery. Spontaneous closure of the VSD [6, 10, 131 and development of infundibular stenosis [7] have been well documented in the natural history of VSD and cannot be considered complications of pulmonary artery banding. However, it is possible that pulmonary arterial banding influences the frequency of infundibular stenosis and spontaneous closure. Acquired branch stenosis of the right or both pulmonary arteries secondary to distal migration of the band is a complication of pulmonary arterial banding but in this study was not a factor in the deaths that followed the debanding procedure (see Table 3). In addition, those patients with acquired branch stenosis did not have consistently greater gradients after debanding (see Table 3). Mortality rates associated with primary repair of VSD in infancy were reported by Sigmann, Stein, and Sloan [17] and by Ching, DuShane, Mc- Goon, and Danielson [3]. The mortality in these reports for infants less than 1 months of age was 18 and yo, respectively. These reported mortality rates for primary repair in infancy are similar to the reported rate for debanding and VSD repair (Table ). However, infants weighing less than 5 kg. present a more difficult problem, with a correspondingly higher mortality rate associated with primary repair [3, 171 An exception is the report by Barratt-Boyes and his colleagues [Z], which cites successful repair in 7 infants weighing less than 5 kg. ( of whom had partial atrioventricular canal) using the technique of surface cooling, deep hypothermia, and circulatory arrest. Based upon this study and the review of the literature cited above, we have drawn the following conclusions. 1. Pulmonary arterial banding provides effective palliation for infants with large VSD.. In our experience, small infants weighing less than kg. experienced no greater mortality from pulmonary arterial banding than did those weighing more than kg. 3. The staged procedure is accompanied by significant morbidity during the interim between surgical procedures. This morbidity can be related to pulmonary arterial bands that are too loose or too tight.

HENRY ET AL.. Pulmonary arterial banding plus the later corrective procedure has an appreciable mortality rate, estimated to be approximately 5% in our institution. 5. Complications of debanding and pulmonary artery reconstruction resulted in the death of of the 1 patients who underwent debanding and VSD closure at this institution. 6. Pulmonary artery branch stenosis and pulmonary valvular stenosis may persist after debanding. The significance of this observation awaits longterm follow-up. 7. Pulmonary arterial banding should be reserved for those infants who are judged to be high-risk candidates for primary closure because of either their extremely small size or an unfavorable anatomy. It is recognized that isolated large VSDs are being closed successfully in small babies in some centers, and results in large series of patients and long-term follow-up will be awaited with interest. References 1. Baffes, T. G. Total body perfusion in infants and small children for open heart surgery. J. Pediatr. Surg. 3:551, 1968.. Barratt-Boyes, B. G., Simpson, M., and Neutze, J. M. Intracardiac surgery in neonates and infants using deep hypothermia with surface cooling and limited cardiopulmonary bypass. Circulation 3 (Suppl. I):5, 1971. 3. Ching, E., DuShane, J. W., McGoon, D. C., and Danielson, G. K. Total correction of ventricular septal defect in infancy using extracorporeal circulation. Ann. Thorac. Surg. 1:1, 1971.. Dillard, D. H., Mohri, H., and Merendino, K. A. Correction of heart disease in infancy utilizing deep hypothermia and total circulatory arrest. J. Thorac. Cardiovasc. Surg. 61:6, 1971. 5. Dobell, A. R. C., Murphy, D. R., and Gibbons, J. E. Pulmonary artery debanding. Ann. Thorac. Surg. 5:35, 1968. 6. Evans, J. R., Rowe, R. D., and Keith, J. D. Spontaneous closure of ventricular septal defects. Circulation : 10, 1960. 7. Gasul, B. M., Dillon, R. F., Vrla, V., and Hait, G. Ventricular septal defects: Their natural transformation into those with infundibular stenosis or into the cyanotic or noncyanotic type of tetralogy of Fallot. J.A.M.A. 16:87, 1957. 8. Goldblatt, A., Bernhard, W. F., Nadas, A. S., and Gross, R. E. Pulmonary artery banding: Indications and results in infants and children. Circulation 3: 17, 1965. 9. Hallman, G. L., Cooley, D. A., and Bloodwell, R. D. Two-stage surgical treatment of ventricular septal defect: Results of pulmonary artery banding in infants and subsequent open-heart repair. J. Thorac. Cardio-r/asc. Surg. 5:67, 1966. 10. Hoffman, J. I. E., and Rudolph, A. M. The natural history of ventricular septal defects in infancy. Am. J. Cardiol. 16:63, 1965. 11. Horiuchi, T., Koyamada, K., Ishitoya, T., Honda, T., Abe, T., and Sagawa, Y. Radical operation under hypothermia for ventricular septal defect in infancy: A report of 6 consecutive cases. J. Cardiovasc. Surg. (Torino) 8:85, 1967. 1. Hunt, C. E., Formanek, G., Levine, M. A., Castaneda, A., and Moller, J. H. Banding of the pulmonary artery: Results in 111 children. Circulation 3:395, 1971. 118 THE ANNALS OF THORACIC SURGERY

Management of Znfants with Large Ventricular Defects 13. Kaplan, S., Daoud, G. I., Benzing, G., Devine, F. J., Glass, I. H., and McGuire, J. Natural history of ventricular septal defect. Am. J. Dis. Child. 105:581, 1963. 1. Kirklin, J. W. Pulmonary arterial banding in babies with large ventricular septal defects (Editorial). Circulation 3:31, 1971. 15. Nadas, A. S. Management of infants with ventricular septal defect, a controversy (Editorial). Pediatrics 39: 1, 1967. 16. Osborn, J. R., Hall, R. J., Winn, D. F., Capper, R. S., and Blake, H. A. An unusual late complication of pulmonary artery banding. Circulation 3:61, 1966. 17. Sigmann, J. M., Stein, A. M., and Sloan, H. E. Early surgical correction of large ventricular septal defects. Pediatrics 39:, 1967. 18. Stark, J., Aberdeen, E., Waterston, D. J., Bonham-Carter, R. E., and Tynan, M. Pulmonary artery constriction (banding): A report of 16 cases. Surgery 65:808, 1969. 19. Stark, J., Tynan, M., Aberdeen, E., Waterston, D. J., Bonham-Carter, R. E., Graham, G. R., and Somerville, J. Repair of intracardiac defects after previous constriction (banding) of the pulmonary artery. Surgery 67:536, 1970. 0. Takahasi, M., Lurie, P. R., Petry, E. L., and King, H. Clinical and hemodynamic effects of pulmonary arterial banding. Am. J. Cardiol. 1: 17, 1968. 1. Talner, N. S. Pulmonary arterial banding (Editorial). Am. J. Cardiol. 6:3, 1970. NOTICE FROM THE AMERICAN BOARD OF THORACIC SURGERY The American Board of Thoracic Surgery will not give an examination in 1973. The next examination (written and oral) will be given in January, 197, and annually thereafter. The deadline for registration for the 197 examination is June 1, 1973. Please address a11 communications to the American Board of Thoracic Surgery, Inc., 16 E. Seven Mile Rd., Detroit, Mich. 805. VOL. 15, NO., FEBRUARY, 1973 119