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Repair of hypoplastic or interrupted aortic arch via sternotomy
Surgery for Congenital Hearl Disease
Repair of hypoplastic or interrupted aortic arch via sternotomy Herein we describe our experience with repair of interrupted aortic arch and coarctation plus hypoplastic aortic arch in 55 consecutive infants (1984 to 1990). Median age at operation was 6 days and median weight 3.1 kg. Associated severe intracardiac anomalies were the rule. All patients had significant congestive cardiac failure, and the majority required prostaglandin El resuscitation and inotropic support (with or without ventilation) before the operation. All operations were performed via sternotomy with core cooling and circulatory arrest. Isolated myocardial perfusion was used in 13 patients during arch repair. A complete intracardiac (biventricular) repair was performed except in patients expected to require a Fontan operation as definitive treatment. The operative mortality overall was 14.5 % (confidence limits 10% to 22%). For arch repair plus biventricular intracardiac repair, the operative mortality was 9% (confidence limits 5% to 15%), and for arch repair plus palliative intracardiac repair, 40 % (confidence limits 22 % to 60 %). The mortality in the isolated myocardial perfusion group was 0 % (confidence limits 0 % to 14 %), which may be related to reduced myocardial ischemic time (p < 0.05). Actuarial survival was 75% (confidence limits 65% to 83%) at 12 months, with no subsequent deaths over 1294 patient-months (mean 28 months) of follow-up. Actuarial freedom from recurrent arch obstruction was 69% (confidence limits 48% to 85%) at 46 months' follow-up. Primary repair of interrupted aortic arch and coarctation plus hypoplastic arch compares favorably with a staged approach and is recommended even when complex intracardiac anatomy is present. (J THORAe CARDIOVASC SURG 1992;104:688-95)
Tom R. Karl, MS, MD, Shunji Sano, MD, PhD,* William Brawn, FRCS,** and Roger B. B. Mee, FRACS, Melbourne. Australia
h e management of interrupted aortic arch and complex coarctation of the aorta with severe arch hypoplasia presents a medical and surgical challenge. Although the From Victorian Paediatriac Cardiac Surgical Unit, Royal Children's Hospital, Melbourne, Australia Received for publication April 6, 1990. Accepted for publication Aug. 27, 1991. Address for reprints: T. R. Karl, Royal Children's Hospital, Flemington Road, Parkville, Victoria, 3052, Australia. *Current address: University of Okayama Medical Center, Okayama, Japan. **Current address: Children's Hospital, Ladywood, Middleway Birmingham, B16AIT, England.
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use of prostaglandin E, resuscitation to maintain ductal patency and improve systemic circulation has dramatically improved the preoperative condition of these infants, significant operative mortality is still a problem." 2 Furthermore, the relative benefit of one- versus two-stage repair for some of these lesions remains controversial.v 4 The purpose of this paper is to review our experience with repair of interrupted aortic arch and coarctation with severe arch hypoplasia and concomitant repair of intracardiac lesions via midline sternotomy. Herein we present our surgical indications, techniques, and results for all patients treated since 1984 (the year in which we opted for one-stage repair of these lesions). We have considered interrupted and severely hypoplastic arch together, because of the similarities in physiologic characteristics
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Table I. Anatomic diagnosis in 55 infants with interrupted aortic arch or coarctation of the aorta with hypoplastic transverse arch Anatomic diagnosis (in addition to arch obstruction)
VSD Truncus arteriosus OILV + TGA + SAS VSD+SAS Tricuspid atresia + TGA + SAS APW Multiple VSDs VSD + anomalous RSCA VSD + anomalous RSCA + SAS VSD+ AS +SAS AVSD Taussig-Bing anomaly Taussig-Bing anomaly + SAS Taussig-Bing anomaly + SAS + AS TGA+ VSD TGA + VSD + SAS AS Mitral atresia + TGA + SAS AS +SAS PAPVD + ASD + VSD AV-VA discordance + VSD + Ebstein's anomaly VSD + RVOTO
No.
10 8 5
5 3
3 2 2
2 2 2 I
1 1 I I I I
1 I I
VSD, Ventricular septal defect; DILV, double-inlet left ventricle; TGA, transposition of the great arteries; SAS, subaortic stenosis; APW, aortopulmonary window; RSCA, right subclavian artery; AS, aortic stenosis; A VSD, atrioventricular septal defect; PAPVD, partial anomalous pulmonary venous drainage; AV- VA, atrioventricular-ventriculoarterial; RVOTO, right ventricular outflow tract obstruction.
(ductal dependency, high pulmonary blood flow), presentation (severe congestive cardiac failure, low cardiac output in the newborn period), and preferred surgical technique (arch and intracardiac repair via sternotomy).
Patients and methods Since July 1984,55 patients have undergone operations for repair of intracardiac defects complicating coarctation with severe arch hypoplasia or interrupted aortic arch. Excepting patients expected to require a Fontan operation as future correction, all patients had a definitive intracardiac repair. Age range was 2 days to 8 months (median 6 days), and weight range was 1.4 to 7.3 kg (median 3.1 kg). We considered the aortic arch to be hypoplastic if the transverse arch diameter was less than the patient's weight in kilograms plus one (i.e., 4 mm for a 3 kg baby). Patients with such anatomy were considered unsuitable candidates for coarctation repair alone as their initial procedure. In general, these patients were duct dependent and had significant congestive cardiac failure. Twenty-two patients had coarctation plus hypoplastic arch and 33 had complete interruption, either type A (n = 6) or type
Fig. 1. Cannulation technique for neonatal arch repair; Angled cannulas, size 10F, are inserted directly into the ductus and ascending aorta. The ductus is ligated proximal to the cannula after the start of CPB. LCA, Left carotid artery; LSCA, left subclavian artery; lA, innominate artery; AO, aorta; PA, pulmonary artery; LA, left atrium; RA, right atrium; RV, right ventricle; L V, left ventricle. B (n = 27). The presence of one or more associated cardiac anomalies was the rule, and specific lesions are summarized in Table I. In addition, seven patients, all with type B interruption of the aortic arch, had DiGeorge syndrome, diagnosed by preoperative T-Iymphocyte studies. For the purposes of this study, and for comparison with other reports, patients were considered in three groups (Table II). All patients had arch repair via sternotomy. Group I consisted of 30 patients with interrupted aortic arch having complete (biventricular) intracardiac repair. Group 2 consisted of 15 patients with complex coarctation of the aorta and hypoplastic arch having complete (biventricular) repair. Group 3 consisted of 10 patients with arch obstruction and complex univentricular heart having open palliation (without biventricular repair). Nine had double-inlet ventricle or atrioventricular valve atresia with transposition of the great arteries (TGA) and subaortic stenosis due to a restrictive bulboventricular foramen. The tenth had multiple ventricular septal defects (VSDs) and a hypoplastic left ventricle. Preoperatively, all the patients were in congestive heart failure and 10 were critically ill with hypotension, renal failure, and metabolic acidosis. Forty-one of the 55 patients received prostaglandin E l infusion preoperatively to improve perfusion of the distal aorta via the ductus arteriosus. Cardiac catheterization was performed in 12 patients, seven of whom had been referred from other units. In the remainder, the diagnosis was made
The Journal of Thoracic and Cardiovascular
6 9 0 Karl et al.
Surgery
Table II. Operations performed concomitant to
transsternal aortic arch repair in 55 patients with interrupted aortic arch or coarctation with hypoplastic arch
Fig. 2. Isolated myocardial perfusion technique for arch repair: The ascending aorta is clamped distal to the aortic cannula, and pump flow is reduced to 10% of full flow. The anastomosis is performed with the arch branches snared and the heart beating.
clinically and confirmed with two-dimensional echocardiography. Operative technique. All patients were operated on using cardiopulmonary bypass (CPB), core cooling, profound hypothermia, and circulatory arrest. Median sternotomy was performed and the aortic arch and branches were extensively dissected. Arterial cannulas were inserted into the ascending aorta and the ductus arteriosus or main pulmonary artery and connected to the CPB circuit with a Y-connector (Fig. 1). In patients with interrupted aortic arch and truncus arteriosus or aortopulmonary window, a single arterial cannula was used. A single venous cannula was placed in the right atrial appendage (Fig. 1). CPB was instituted at 150 ml/kg per minute and the patients were core-cooled to a nasopharyngeal temperature of 18 0 C. Pulmonary blood flow on CPB was controlled either by ligating the ductus proximal to the arterial cannula or by snaring branch pulmonary arteries. During cooling the upper descending aorta was mobilized as far distally as possible, well beyond the ductus insertion. Aortic arch reconstruction was performed with profound hypothermia and total circulatory arrest in 42 of 55 patients. With clamps on the ascending and descending aorta and the head vessels snared, all ductal tissue was excised. The descending aorta was anastomosed to the underside of the aortic arch at the level of the innominate and left carotid arteries in an end-to-side fashion with 7-0 polypropylene continuous suture. In three of the patients with interrupted aortic arch and truncus arteriosus, the descending aorta was anastomosed more proximally on the ascending aorta. After completion of arch repair, the distal aortic clamp and head vessel snares were
Operation
No.
VSD closure Truncusrepair Switch + septectomy Switch + VSD closure VSD closure + resection SAS Repair APW Damus + RV-PA conduit Repair AVSD Switch + VSD + resection SAS + aortic valvotomy Aorticvalvotomy VSD + RVOTO resection Aorticvalvotomy + SAS resection PA band ASD closure Switch + Senning + VSD closure CreationAPW + septectomy + BTS CreationAPW + septectomy + PA band
14 8 7
5 5 3 2 2 I
VSD, Ventricular septal defect; APW, aortopulmonary window; SAS, subaortic stenosis; RV-PA, right ventricular-pulmonary arterial; AVSD, atrioventricular septal defect; RVOTO, right ventricular outflow tract obstruction; PA, pulmonary artery; ASD, atrial septal defect; BTS, Blalock-Taussig shunt.
removed and low-flow CPB was resumed. After deairing, flow was returned to normal and intracardiac repair followed. Mean CPB time was 93 minutes (37 to 240 minutes), mean cardiac ischemic time was 69 minutes (16 to 122 minutes), and mean circulatory arrest time was 53 minutes (17 to 105 minutes). The various operations performed concomitant to arch repair are listed in Table II. Recently, a new technique of isolated myocardial perfusion was used in 12 patients during arch reconstruction' After aortic mobilization and core cooling to 180 C, CPB flow was reduced to 10% of normal. A clamp was then placed on the ascending aorta just distal to the aortic cannula (Fig. 3). During arch reconstruction, the heart was allowed to beat slowly at low temperature, at a coronary perfusion pressure of 35 to 45 mm Hg. After completion of arch repair, the descending aortic clamp was removed and the ascending aortic clamp repositioned to allow cardioplegic arrest at full CPB flow (for intracardiac repair). By means of isolated myocardial perfusion, mean myocardial ischemic time was reduced from 69 to 55 minutes (p < 0.05). Left ventricular outflow tract obstruction was present in both group 1 (n = 8) and group 2 (n = 5) patients. The obstruction was usually at the subvalvular level and was treated by resection through the right ventricle (n = 1), through the pulmonary artery (n = I), or through the aorta (n = 5). Two patients had Damus aortopulmonary connections combined with insertion of a right ventricular-pulmonary arterial conduit. In group 3, nine patients had subaortic stenosis resulting from a restrictive bulboventricular foramen. The obstruction was treated by creation of an aortopulmonary window plus atrial
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PROBABILITY (70% confidence limits)
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Fig. 3. Actuarial survival (Kaplan-Meier) for 55 infants after arch repair, including operative mortality. The last death occurred at 12 months' follow-up. Two patients have been lost to follow-up.
septectomy plus Blalock-Taussig shunt or pulmonary artery band (n = 2) or by arterial switch plus atrial septectomy (n = 7).
Results Within group 1 there were two hospital deaths (7%, CL * 2% to 15%). The first occurred in a patient with type B interrupted aortic arch and VSD in whom support was terminated after postoperative diagnosis of a chromosomal defect incompatible with long-term survival. The second death occurred in a 2.1 kg premature baby, chronically ventilated, with hyaline membrane disease and Klippel-Feil syndrome. He died 24 hours postoperativelyof a sudden cardiac arrest. Autopsy revealed severe bronchopulmonary dysplasia. Ingroup 2 there were two hospital deaths (13%, CL 5% to 28%). The first occurred in a 2 kg baby with TGA, VSD, coarctation, and hypoplastic arch. He died of overwhelming sepsis 3 days after arch repair, arterial switch, and VSD closure. The second patient had coarctation, *CL
= 70% confidence limits.
hypoplastic arch, atrial septal defect, VSD, and partial anomalous pulmonary venous drainage. He died 3 months after arch repair because of severe tracheobronchial stenosis. Multiple attempts to relieve compression of the left bronchus by the aortic arch, including interposition graft placement in the upper descending aorta, were unsuccessful. The immediate cause of death was erosion of an intraluminal bronchial stent into the left pulmonary artery. Group 3 patients had more complex anatomic and physiologic problems. There were four hospital deaths (40%, CL 22% to 60%). Two occurred in patients having arch reconstruction, proximal aortopulmonary anastomoses, and regulation of pulmonary blood flow by either a modified Blalock-Taussig shunt (n = 1) or pulmonary artery band (n = 1). Both deaths were related to low cardiac output in the early postoperative period. A third patient, treated with arch reconstruction, arterial switch, and atrial septectomy, died of neoaortic valve incompetence. The fourth baby, who had a similar operation, died of hemorrhage and low cardiac output on the day of the operation.
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Table III. Operative (hospital) mortality for 55 infants after repair of interrupted aortic arch or coarctation with hypoplastic arch plus various intracardiac lesions Patient category IAA, biventricular repair IAA, all patients IAA + truncus arteriosus CoA/hypoplastic arch, biventricular repair Arch repair + palliation Isolated myocardial perfusion Overall operative mortality
Mortality
CL
No.
(%)
(%)
30 33 8 15
7 6 0 13
2-15 2-14 0-21 5-28
10 13
40 0
22-60 0-13
55
14.5
10-22
fAA, Interrupted aortic arch; CoA, coarctation of the aorta.
The overall operative mortality rate was 14.5% (CL 10% to 22%) (Table III). For patients undergoing arch reconstruction plus definitive (biventricular) repair, the operative mortality rate was9% (CL 5% to 15%). The operative mortality rate for all patients having repair of interrupted aortic arch, irrespective of intracardiac defect, was 6% (CL 2% to 14%). Operative mortality was not significantly different in groups 1 and 2 (p < 0.41). The subset having arch reconstruction concomitant to open palliation had the highest mortality (40%, CL 22% to 60%, p < 0.03). For concomitant repair of truncus arteriosus and interrupted aortic arch, the operative mortality rate was 0% (CL 0% to 21%). Likewise, there were no operative deaths in the group having repair with isolated myocardial perfusion (0%, CL 0% to 13%, p < 0.09). Of the 47 early survivors, 45 have been followed up clinically for a total of 1294 patient-months (mean 28 months) after the operation. Two patients have been lost to follow-up. There have been three late deaths in group 1. The first occurred in a 1.4 kg premature baby 7 months after repair oftype B interrupted aortic arch plus VSD. The cause was chronic respiratory insufficiency resulting from tracheobronchial malacia. The second occurred 2 months after the operation in a term baby, also with type B interrupted aortic arch plus VSD, in whom left ventricular outflow tract obstruction developed after the initial operation. She died after attempted balloon catheter dilation of the aorta and subaortic stenosis in another hospital. The third patient died 12 months postoperatively of cardiac failure, cause unknown, in another hospital. No postmortem examination was performed. There was one late death in group 2, occurring 12
months postoperatively. This patient had been operated on at 5 days of age for atrioventricular-ventriculoarterial discordance, VSD, hypoplastic right ventricle, coarctation plus hypoplastic arch, and Ebstein's anomaly of the systemic atrioventricular valve. The operation consisted of a Senning repair plus arterial switch operation, arch reconstruction, and VSD closure. The hemodynamic result was satisfactory, but atrial arrhythmias necessitated medication after discharge. This patient died suddenly 12 months after the operation, presumably because of an arrhythmia. There was also one late death in group 3, related to recurrent arch obstruction and occurring 5 months after the operation. The actuarial survival curve for all 55 patients undergoing surgery is shown in Fig. 3. Including operative mortality, the actuarial survival probability was 75% (CL 65% to 83%) at 12 months, with no subsequent deaths over 73 months of follow-up. There have been 11 known cases of residual or recurrent arch obstruction in this series, necessitating either balloon angioplasty (4) or reoperation (4). Stenosis was found at the site of arch anastomosis in nine patients but in the hypoplastic ascending aorta (supravalvular) in two. Actuarial freedom from recurrent arch obstruction was 69% (CL 48% to 85%) at 46 months (Fig. 4). There have been two reoperations for subaortic stenosis. One patient had been free of subaortic stenosis at the time of the first operation. The other patient, who had combined subaortic and valvular aortic stenosis, was treated unsuccessfully with balloon catheter dilation. Problems with compression of the left bronchus by the aortic arch were encountered in three patients. The first, a baby with type B interrupted aortic arch plus truncus arteriosus, had compression of the left main bronchus and required interposition of an 8 mm polytetrafluoroethylene graft in the descending aorta 26 days after initial repair. Postoperative cardiac catheterization demonstrated a 60 mm Hg gradient across a short segment of hypoplastic ascending aorta, residual compression of the right pulmonary artery, and a 60 mm Hg gradient across the Hancock valved conduit. When the child was 14 months of age, the hypoplastic ascending aorta was replaced with a 16 mm pulmonary homograft segment, and a 15 mm pulmonary bifurcation homograft was inserted between the right ventricle and pulmonary arteries. A second patient with type B interrupted aortic arch plus truncus arteriosus also had compression of the left bronchus by the reconstructed aorta and underwent interposition of an 8 mm polytetrafluoroethylene graft in the upper descending aorta 14 days after initial repair, with a satisfactory out-
Volume 104 Number 3 September 1992
Neonatal aortic arch repair 693
PROBABILITY (70% Confidence Limits)
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..
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,
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Fig. 4. Actuarial freedom from recurrent arch obstruction (Kaplan-Meier) for survivors of arch repair. The last recurrence was detected at 46 months' follow-up.
come. The third patient requiring reoperation for relief of bronchial compression was a hospital mortality, as described earlier. Neurologic status, although difficult to assess completelyin the developing child, has been good in all but two survivors, both of whom have generalized developmental delay. One has cerebral atrophy and dilated ventricles. One nonsurvivor was known to have had a grade 3 intraventricular hemorrhage. Three patients have left vocal cord paralysis, which has been associated with chronic aspiration in one. It is possible that other neurologic events may have occurred in nonsurvivors.
Discussion Interrupted aortic arch and coarctation plus severe arch hypoplasia are uncommon congenital malformations that occur with associated intracardiac congenital anomalies of varying severity.v " A variety of surgical procedures have been used for treatment of these anomalies. Left thoracotomy for repair of the arch is complicated by poor exposure of the proximal arch and a marked difficulty in palliating more complicated lesions such as
TGA, truncus arteriosus, and aortic or subaortic stenosis.2, 3, 8 Furthermore, for patients with complex coarctation of the aorta plus hypoplastic transverse arch, both operative mortality and recoarctation rate are higher if the hypoplastic segment is disregarded, and the residual obstruction will compound left ventricular dysfunction. The hospital mortality rate in series in which this approach was used ranged from 41% to 50%, and longterm survival has been poor. This situation has stimulated interest in the one-stage approach now advocated for interrupted aortic arch in most centers. The best approach for coarctation plus hypoplastic arch, however, remains a controversy. The first successful one-stage correction of type A interrupted aortic arch was performed by Barratt-Boyes and associates? in 1970. Murphy and colleagues 10 reported correction of a type B interrupted aortic arch in 1973. Barratt-Boyes used a left posterolateral thoracotomy and midsternotomy for his repair, and Murphy extended his midsternotomy incision into the left third intercostal space for better exposure of the descending aorta. In both instances an interposition graft was used. In 1975, Tru-
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sler and Izukawa 11 reported correction of a type B interruption with a direct anastomosis of the descending aorta and closure of the YSD through the midline in a 13-dayold baby. By 1979, however, only four cases of successful neonatal repair of interrupted aortic arch had been reported.l? Recently, several groups have presented series of patients treated by total repair of the arch and intracardiac anomalies simultaneously through midline sternotomy.': 2, 4,13-16 Early mortality rates have varied from 8.3% to 30%, and all have recommended primary repair as the best approach for interrupted aortic arch with YSD. Our own 6% operative mortality rate for repair of interrupted aortic arch (8% for all types of arch reconstruction with biventricular repair) tends to support this philosophy. We believe that a large direct anastomosis is essential for effective arch reconstruction and that this is best achieved via a sternotomy for both interrupted and hypoplastic arch. The operative technique is now standardized and the results are reproducible for a variety of lesions. The problem of late recurrence of arch obstruction has been relatively infrequent and will probably be treatable with balloon dilation in the majority of cases in the future. Repair by sternotomy compares favorably in this respect with repair of isolated coarctation via left thoracotomy in newborn infants. The problem of severe left ventricular outflow tract obstruction, especially with interrupted aortic arch, should be addressed at the time of the initial operation. Direct muscle resection appears to provide effective relief, although YSD closure may itself tend to reduce the gradient. We expect to see further recurrence of subaortic stenosis as follow-up time increases. The presence of arch obstruction in patients with univentricular heart strongly suggests that subaortic stenosis may be present or developing. This tendency may be increased by pulmonary artery banding. The most difficult patients in this group are the neonates with duct-dependent circulation, arch obstruction, and established subaortic stenosis (usually in the setting of atrioventricular valve atresia or double-inlet left ventricle with TGA). Dissatisfied with results of Norwood type procedures in this group, we currently prefer arterial switch plus atrial septectomy concomitant to arch repair. The restrictive bulboventricular foramen then controls pulmonary blood flow, with a view to a subsequent Fontan operation. Our overall approach to subaortic stenosis in the univentricular heart has recently been reviewed. 17 A serious complication after direct anastomosis of the arch was left bronchial compression by the reconstructed aorta. In two of our patients with interrupted aortic arch and truncus arteriosus, left bronchial compression may have been related to construction of too proximal an aor-
tic anastomosis. We now believe that this complication may be avoided by making the anastomosis more distally in the ascending aorta. This may leave a short segment of hypoplastic ascending aorta (between the origin of the pulmonary arteries and the descending aortic anastomosis), which in one of our patients with truncus and interrupted arch necessitated subsequent revision. In such cases this segment should perhaps be enlarged with a pericardial or homograft patch at the time of initial repair. A more extensive review of our experience with truncus and interrupted arch appears in another publica-
tion." In conclusion, primary repair of interrupted aortic arch and coarctation plus hypoplastic arch compares favorably with staged repairs and can be performed in critically ill newborn infants with reasonable risk and good mediumterm outcome. Isolated myocardial perfusion during arch repair reduces the required cardiac ischemic time and may improve operative mortality. Results for arch repair plus palliation of univentricular heart and subaortic stenosis by arterial switch and septectomy are currently under evaluation, and improved results are expected in this group as well. One must remain aware of the potential for bronchial compression after arch repair, especially with truncus and interrupted aortic arch. Severity of associated intracardiac lesions should not be a contraindication to one-stage repair in the majority of newborn infants with severe arch obstruction. REFERENCES 1. NorwoodWI, Lang P, Castaneda AR, HougenTJ. Repar-
2. 3.
4. 5. 6.
ativeoperationsfor interrupted aortic arch with ventricular septaldefect.J THORAC CARDIOVASC SURG 1983;86:32-7. Scott WA, Rocchini AP, Bove EL, et al. Repair of interrupted aortic arch in infancy.J THORAC CARDIOVASC SURG 1988;96:564-8. Hammon JW, Merrill WH, Prager RL, et al. Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair. Ann Thorac Surg 1986;42:17-21. Schumacher G, Schreiber R, Meisner H, et al. Interrupted aortic arch: natural history and operative results. Pediatr Cardiol 1986;7:89-93. Sano S, Mee RBB. Isolated myocardial perfusion during arch repair. Ann Thorac Surg 1990;49:97-2. Van Praagh R, Bernhard WF, Rosenthal A, et al. Interrupted aortic arch: surgicaltreatment. Am J Cardiol 1971; 27:200-11.
7. Bharati S, Lev M. The surgical anatomy of the heart in tubular hypoplasia of the transverseaorta (preductalcoarctation). J THORAC CARDIOVASC SURG 1986;91:79-85. 8. Vouhe PR, Tringuet F, et al. Aortic coarctation with hypoplastic aortic arch: resultsof extendedend-to-end aor-
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9.
10.
II.
12.
tic arch anastomosis. J THORAC CARDIOVASC SURG 1988; 96:557-63. Barratt-Boyes BG, Nicholls TT, BrandtPWT, NeutzeJM. Aortic arch interruption associated with patent ductus arteriosus, ventricular septal defect, and total anomalous pulmonary venous connection. J THORAC CARDIOVASC SURG 1972;62:367-73. Murphy DA, Lemire GG, Tessler I, Dunn GL. Correction oftype B aortic arch interruption with ventricular and atrial septal defects in a three-day-old infant. J THORAC CARDIOVASC SURG 1973;65:882-6. Trusler GA, Izukawa T. Interrupted aortic arch and ventricular septal defect: direct repair through a median sternotomy incision in a 13-day-old infant. J THORAC CARDIOVASC SURG 1975;69:126-31. Van der Horst R, Hastreiter AR, Levitsky S, Fisher EA, DuBrow IW, Weinberg M. Interrupted aortic arch operation in the first week of life: hemodynamic and angiographic evaluation one year later. Ann Thorac Surg 1979;27:11220.
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13. Turley K, Yee ES, Ebert PA. The total repair of interrupted arch complex in infants: the anterior approach. Circulation I984;70(Pt 2):116-20. 14. Moulton AL, Bowman FO Jr. Primary definitive repair of type B interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus: early and late results. J THORAC CARDIOVASC SURG 1981;82:501-10. 15. Monro JL, Bunton RW, Sutherland GR, Keeton BR. Correction of interrupted aortic arch. J THORAC CARDIOVASC SURG 1989;98:421-7. 16. Sell JE, Jonas RA, Mayer JE, et al. The results of a surgical program for interrupted aortic arch. J THORAC CARDIOVASC SURG 1988;96:864-77. 17. Karl TR, Watterson KG, Sano S, Mee RBB. Surgery for subaortic stenosis in univentricular hearts. Ann Thorac Surg [In press]. 18. Sano S, Brawn WJ, Mee RBB. Repair of truncus arteriosus and interrupted aortic arch. J Cardiac Surg 1990;5: 15762.
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Repair of hypoplastic or interrupted aortic arch via sternotomy Herein we describe our experience with repair of interrupted aortic arch and coarctation plus hypoplastic aortic arch in 55 consecutive infants (1984 to 1990). Median age at operation was 6 days and median weight 3.1 kg. Associated severe intracardiac anomalies were the rule. All patients had significant congestive cardiac failure, and the majority required prostaglandin El resuscitation and inotropic support (with or without ventilation) before the operation. All operations were performed via sternotomy with core cooling and circulatory arrest. Isolated myocardial perfusion was used in 13 patients during arch repair. A complete intracardiac (biventricular) repair was performed except in patients expected to require a Fontan operation as definitive treatment. The operative mortality overall was 14.5 % (confidence limits 10% to 22%). For arch repair plus biventricular intracardiac repair, the operative mortality was 9% (confidence limits 5% to 15%), and for arch repair plus palliative intracardiac repair, 40 % (confidence limits 22 % to 60 %). The mortality in the isolated myocardial perfusion group was 0 % (confidence limits 0 % to 14 %), which may be related to reduced myocardial ischemic time (p < 0.05). Actuarial survival was 75% (confidence limits 65% to 83%) at 12 months, with no subsequent deaths over 1294 patient-months (mean 28 months) of follow-up. Actuarial freedom from recurrent arch obstruction was 69% (confidence limits 48% to 85%) at 46 months' follow-up. Primary repair of interrupted aortic arch and coarctation plus hypoplastic arch compares favorably with a staged approach and is recommended even when complex intracardiac anatomy is present. (J THORAe CARDIOVASC SURG 1992;104:688-95)
Tom R. Karl, MS, MD, Shunji Sano, MD, PhD,* William Brawn, FRCS,** and Roger B. B. Mee, FRACS, Melbourne. Australia
h e management of interrupted aortic arch and complex coarctation of the aorta with severe arch hypoplasia presents a medical and surgical challenge. Although the From Victorian Paediatriac Cardiac Surgical Unit, Royal Children's Hospital, Melbourne, Australia Received for publication April 6, 1990. Accepted for publication Aug. 27, 1991. Address for reprints: T. R. Karl, Royal Children's Hospital, Flemington Road, Parkville, Victoria, 3052, Australia. *Current address: University of Okayama Medical Center, Okayama, Japan. **Current address: Children's Hospital, Ladywood, Middleway Birmingham, B16AIT, England.
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use of prostaglandin E, resuscitation to maintain ductal patency and improve systemic circulation has dramatically improved the preoperative condition of these infants, significant operative mortality is still a problem." 2 Furthermore, the relative benefit of one- versus two-stage repair for some of these lesions remains controversial.v 4 The purpose of this paper is to review our experience with repair of interrupted aortic arch and coarctation with severe arch hypoplasia and concomitant repair of intracardiac lesions via midline sternotomy. Herein we present our surgical indications, techniques, and results for all patients treated since 1984 (the year in which we opted for one-stage repair of these lesions). We have considered interrupted and severely hypoplastic arch together, because of the similarities in physiologic characteristics
Volume 104 Number 3 September 1992
Neonatal aortic arch repair
689
Table I. Anatomic diagnosis in 55 infants with interrupted aortic arch or coarctation of the aorta with hypoplastic transverse arch Anatomic diagnosis (in addition to arch obstruction)
VSD Truncus arteriosus OILV + TGA + SAS VSD+SAS Tricuspid atresia + TGA + SAS APW Multiple VSDs VSD + anomalous RSCA VSD + anomalous RSCA + SAS VSD+ AS +SAS AVSD Taussig-Bing anomaly Taussig-Bing anomaly + SAS Taussig-Bing anomaly + SAS + AS TGA+ VSD TGA + VSD + SAS AS Mitral atresia + TGA + SAS AS +SAS PAPVD + ASD + VSD AV-VA discordance + VSD + Ebstein's anomaly VSD + RVOTO
No.
10 8 5
5 3
3 2 2
2 2 2 I
1 1 I I I I
1 I I
VSD, Ventricular septal defect; DILV, double-inlet left ventricle; TGA, transposition of the great arteries; SAS, subaortic stenosis; APW, aortopulmonary window; RSCA, right subclavian artery; AS, aortic stenosis; A VSD, atrioventricular septal defect; PAPVD, partial anomalous pulmonary venous drainage; AV- VA, atrioventricular-ventriculoarterial; RVOTO, right ventricular outflow tract obstruction.
(ductal dependency, high pulmonary blood flow), presentation (severe congestive cardiac failure, low cardiac output in the newborn period), and preferred surgical technique (arch and intracardiac repair via sternotomy).
Patients and methods Since July 1984,55 patients have undergone operations for repair of intracardiac defects complicating coarctation with severe arch hypoplasia or interrupted aortic arch. Excepting patients expected to require a Fontan operation as future correction, all patients had a definitive intracardiac repair. Age range was 2 days to 8 months (median 6 days), and weight range was 1.4 to 7.3 kg (median 3.1 kg). We considered the aortic arch to be hypoplastic if the transverse arch diameter was less than the patient's weight in kilograms plus one (i.e., 4 mm for a 3 kg baby). Patients with such anatomy were considered unsuitable candidates for coarctation repair alone as their initial procedure. In general, these patients were duct dependent and had significant congestive cardiac failure. Twenty-two patients had coarctation plus hypoplastic arch and 33 had complete interruption, either type A (n = 6) or type
Fig. 1. Cannulation technique for neonatal arch repair; Angled cannulas, size 10F, are inserted directly into the ductus and ascending aorta. The ductus is ligated proximal to the cannula after the start of CPB. LCA, Left carotid artery; LSCA, left subclavian artery; lA, innominate artery; AO, aorta; PA, pulmonary artery; LA, left atrium; RA, right atrium; RV, right ventricle; L V, left ventricle. B (n = 27). The presence of one or more associated cardiac anomalies was the rule, and specific lesions are summarized in Table I. In addition, seven patients, all with type B interruption of the aortic arch, had DiGeorge syndrome, diagnosed by preoperative T-Iymphocyte studies. For the purposes of this study, and for comparison with other reports, patients were considered in three groups (Table II). All patients had arch repair via sternotomy. Group I consisted of 30 patients with interrupted aortic arch having complete (biventricular) intracardiac repair. Group 2 consisted of 15 patients with complex coarctation of the aorta and hypoplastic arch having complete (biventricular) repair. Group 3 consisted of 10 patients with arch obstruction and complex univentricular heart having open palliation (without biventricular repair). Nine had double-inlet ventricle or atrioventricular valve atresia with transposition of the great arteries (TGA) and subaortic stenosis due to a restrictive bulboventricular foramen. The tenth had multiple ventricular septal defects (VSDs) and a hypoplastic left ventricle. Preoperatively, all the patients were in congestive heart failure and 10 were critically ill with hypotension, renal failure, and metabolic acidosis. Forty-one of the 55 patients received prostaglandin E l infusion preoperatively to improve perfusion of the distal aorta via the ductus arteriosus. Cardiac catheterization was performed in 12 patients, seven of whom had been referred from other units. In the remainder, the diagnosis was made
The Journal of Thoracic and Cardiovascular
6 9 0 Karl et al.
Surgery
Table II. Operations performed concomitant to
transsternal aortic arch repair in 55 patients with interrupted aortic arch or coarctation with hypoplastic arch
Fig. 2. Isolated myocardial perfusion technique for arch repair: The ascending aorta is clamped distal to the aortic cannula, and pump flow is reduced to 10% of full flow. The anastomosis is performed with the arch branches snared and the heart beating.
clinically and confirmed with two-dimensional echocardiography. Operative technique. All patients were operated on using cardiopulmonary bypass (CPB), core cooling, profound hypothermia, and circulatory arrest. Median sternotomy was performed and the aortic arch and branches were extensively dissected. Arterial cannulas were inserted into the ascending aorta and the ductus arteriosus or main pulmonary artery and connected to the CPB circuit with a Y-connector (Fig. 1). In patients with interrupted aortic arch and truncus arteriosus or aortopulmonary window, a single arterial cannula was used. A single venous cannula was placed in the right atrial appendage (Fig. 1). CPB was instituted at 150 ml/kg per minute and the patients were core-cooled to a nasopharyngeal temperature of 18 0 C. Pulmonary blood flow on CPB was controlled either by ligating the ductus proximal to the arterial cannula or by snaring branch pulmonary arteries. During cooling the upper descending aorta was mobilized as far distally as possible, well beyond the ductus insertion. Aortic arch reconstruction was performed with profound hypothermia and total circulatory arrest in 42 of 55 patients. With clamps on the ascending and descending aorta and the head vessels snared, all ductal tissue was excised. The descending aorta was anastomosed to the underside of the aortic arch at the level of the innominate and left carotid arteries in an end-to-side fashion with 7-0 polypropylene continuous suture. In three of the patients with interrupted aortic arch and truncus arteriosus, the descending aorta was anastomosed more proximally on the ascending aorta. After completion of arch repair, the distal aortic clamp and head vessel snares were
Operation
No.
VSD closure Truncusrepair Switch + septectomy Switch + VSD closure VSD closure + resection SAS Repair APW Damus + RV-PA conduit Repair AVSD Switch + VSD + resection SAS + aortic valvotomy Aorticvalvotomy VSD + RVOTO resection Aorticvalvotomy + SAS resection PA band ASD closure Switch + Senning + VSD closure CreationAPW + septectomy + BTS CreationAPW + septectomy + PA band
14 8 7
5 5 3 2 2 I
VSD, Ventricular septal defect; APW, aortopulmonary window; SAS, subaortic stenosis; RV-PA, right ventricular-pulmonary arterial; AVSD, atrioventricular septal defect; RVOTO, right ventricular outflow tract obstruction; PA, pulmonary artery; ASD, atrial septal defect; BTS, Blalock-Taussig shunt.
removed and low-flow CPB was resumed. After deairing, flow was returned to normal and intracardiac repair followed. Mean CPB time was 93 minutes (37 to 240 minutes), mean cardiac ischemic time was 69 minutes (16 to 122 minutes), and mean circulatory arrest time was 53 minutes (17 to 105 minutes). The various operations performed concomitant to arch repair are listed in Table II. Recently, a new technique of isolated myocardial perfusion was used in 12 patients during arch reconstruction' After aortic mobilization and core cooling to 180 C, CPB flow was reduced to 10% of normal. A clamp was then placed on the ascending aorta just distal to the aortic cannula (Fig. 3). During arch reconstruction, the heart was allowed to beat slowly at low temperature, at a coronary perfusion pressure of 35 to 45 mm Hg. After completion of arch repair, the descending aortic clamp was removed and the ascending aortic clamp repositioned to allow cardioplegic arrest at full CPB flow (for intracardiac repair). By means of isolated myocardial perfusion, mean myocardial ischemic time was reduced from 69 to 55 minutes (p < 0.05). Left ventricular outflow tract obstruction was present in both group 1 (n = 8) and group 2 (n = 5) patients. The obstruction was usually at the subvalvular level and was treated by resection through the right ventricle (n = 1), through the pulmonary artery (n = I), or through the aorta (n = 5). Two patients had Damus aortopulmonary connections combined with insertion of a right ventricular-pulmonary arterial conduit. In group 3, nine patients had subaortic stenosis resulting from a restrictive bulboventricular foramen. The obstruction was treated by creation of an aortopulmonary window plus atrial
Volume 104 Number 3
Neonatal aorticarch repair 69 1
September 1992
PROBABILITY (70% confidence limits)
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Fig. 3. Actuarial survival (Kaplan-Meier) for 55 infants after arch repair, including operative mortality. The last death occurred at 12 months' follow-up. Two patients have been lost to follow-up.
septectomy plus Blalock-Taussig shunt or pulmonary artery band (n = 2) or by arterial switch plus atrial septectomy (n = 7).
Results Within group 1 there were two hospital deaths (7%, CL * 2% to 15%). The first occurred in a patient with type B interrupted aortic arch and VSD in whom support was terminated after postoperative diagnosis of a chromosomal defect incompatible with long-term survival. The second death occurred in a 2.1 kg premature baby, chronically ventilated, with hyaline membrane disease and Klippel-Feil syndrome. He died 24 hours postoperativelyof a sudden cardiac arrest. Autopsy revealed severe bronchopulmonary dysplasia. Ingroup 2 there were two hospital deaths (13%, CL 5% to 28%). The first occurred in a 2 kg baby with TGA, VSD, coarctation, and hypoplastic arch. He died of overwhelming sepsis 3 days after arch repair, arterial switch, and VSD closure. The second patient had coarctation, *CL
= 70% confidence limits.
hypoplastic arch, atrial septal defect, VSD, and partial anomalous pulmonary venous drainage. He died 3 months after arch repair because of severe tracheobronchial stenosis. Multiple attempts to relieve compression of the left bronchus by the aortic arch, including interposition graft placement in the upper descending aorta, were unsuccessful. The immediate cause of death was erosion of an intraluminal bronchial stent into the left pulmonary artery. Group 3 patients had more complex anatomic and physiologic problems. There were four hospital deaths (40%, CL 22% to 60%). Two occurred in patients having arch reconstruction, proximal aortopulmonary anastomoses, and regulation of pulmonary blood flow by either a modified Blalock-Taussig shunt (n = 1) or pulmonary artery band (n = 1). Both deaths were related to low cardiac output in the early postoperative period. A third patient, treated with arch reconstruction, arterial switch, and atrial septectomy, died of neoaortic valve incompetence. The fourth baby, who had a similar operation, died of hemorrhage and low cardiac output on the day of the operation.
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Karl et al.
Table III. Operative (hospital) mortality for 55 infants after repair of interrupted aortic arch or coarctation with hypoplastic arch plus various intracardiac lesions Patient category IAA, biventricular repair IAA, all patients IAA + truncus arteriosus CoA/hypoplastic arch, biventricular repair Arch repair + palliation Isolated myocardial perfusion Overall operative mortality
Mortality
CL
No.
(%)
(%)
30 33 8 15
7 6 0 13
2-15 2-14 0-21 5-28
10 13
40 0
22-60 0-13
55
14.5
10-22
fAA, Interrupted aortic arch; CoA, coarctation of the aorta.
The overall operative mortality rate was 14.5% (CL 10% to 22%) (Table III). For patients undergoing arch reconstruction plus definitive (biventricular) repair, the operative mortality rate was9% (CL 5% to 15%). The operative mortality rate for all patients having repair of interrupted aortic arch, irrespective of intracardiac defect, was 6% (CL 2% to 14%). Operative mortality was not significantly different in groups 1 and 2 (p < 0.41). The subset having arch reconstruction concomitant to open palliation had the highest mortality (40%, CL 22% to 60%, p < 0.03). For concomitant repair of truncus arteriosus and interrupted aortic arch, the operative mortality rate was 0% (CL 0% to 21%). Likewise, there were no operative deaths in the group having repair with isolated myocardial perfusion (0%, CL 0% to 13%, p < 0.09). Of the 47 early survivors, 45 have been followed up clinically for a total of 1294 patient-months (mean 28 months) after the operation. Two patients have been lost to follow-up. There have been three late deaths in group 1. The first occurred in a 1.4 kg premature baby 7 months after repair oftype B interrupted aortic arch plus VSD. The cause was chronic respiratory insufficiency resulting from tracheobronchial malacia. The second occurred 2 months after the operation in a term baby, also with type B interrupted aortic arch plus VSD, in whom left ventricular outflow tract obstruction developed after the initial operation. She died after attempted balloon catheter dilation of the aorta and subaortic stenosis in another hospital. The third patient died 12 months postoperatively of cardiac failure, cause unknown, in another hospital. No postmortem examination was performed. There was one late death in group 2, occurring 12
months postoperatively. This patient had been operated on at 5 days of age for atrioventricular-ventriculoarterial discordance, VSD, hypoplastic right ventricle, coarctation plus hypoplastic arch, and Ebstein's anomaly of the systemic atrioventricular valve. The operation consisted of a Senning repair plus arterial switch operation, arch reconstruction, and VSD closure. The hemodynamic result was satisfactory, but atrial arrhythmias necessitated medication after discharge. This patient died suddenly 12 months after the operation, presumably because of an arrhythmia. There was also one late death in group 3, related to recurrent arch obstruction and occurring 5 months after the operation. The actuarial survival curve for all 55 patients undergoing surgery is shown in Fig. 3. Including operative mortality, the actuarial survival probability was 75% (CL 65% to 83%) at 12 months, with no subsequent deaths over 73 months of follow-up. There have been 11 known cases of residual or recurrent arch obstruction in this series, necessitating either balloon angioplasty (4) or reoperation (4). Stenosis was found at the site of arch anastomosis in nine patients but in the hypoplastic ascending aorta (supravalvular) in two. Actuarial freedom from recurrent arch obstruction was 69% (CL 48% to 85%) at 46 months (Fig. 4). There have been two reoperations for subaortic stenosis. One patient had been free of subaortic stenosis at the time of the first operation. The other patient, who had combined subaortic and valvular aortic stenosis, was treated unsuccessfully with balloon catheter dilation. Problems with compression of the left bronchus by the aortic arch were encountered in three patients. The first, a baby with type B interrupted aortic arch plus truncus arteriosus, had compression of the left main bronchus and required interposition of an 8 mm polytetrafluoroethylene graft in the descending aorta 26 days after initial repair. Postoperative cardiac catheterization demonstrated a 60 mm Hg gradient across a short segment of hypoplastic ascending aorta, residual compression of the right pulmonary artery, and a 60 mm Hg gradient across the Hancock valved conduit. When the child was 14 months of age, the hypoplastic ascending aorta was replaced with a 16 mm pulmonary homograft segment, and a 15 mm pulmonary bifurcation homograft was inserted between the right ventricle and pulmonary arteries. A second patient with type B interrupted aortic arch plus truncus arteriosus also had compression of the left bronchus by the reconstructed aorta and underwent interposition of an 8 mm polytetrafluoroethylene graft in the upper descending aorta 14 days after initial repair, with a satisfactory out-
Volume 104 Number 3 September 1992
Neonatal aortic arch repair 693
PROBABILITY (70% Confidence Limits)
. '-
..
.'_.......• ..... . ,
,
-w
-.
0.8
0.6
0.4
0.2
OL----------------------------------' 20 40 60 80 o
POSTOPERATIVE MONTH
Fig. 4. Actuarial freedom from recurrent arch obstruction (Kaplan-Meier) for survivors of arch repair. The last recurrence was detected at 46 months' follow-up.
come. The third patient requiring reoperation for relief of bronchial compression was a hospital mortality, as described earlier. Neurologic status, although difficult to assess completelyin the developing child, has been good in all but two survivors, both of whom have generalized developmental delay. One has cerebral atrophy and dilated ventricles. One nonsurvivor was known to have had a grade 3 intraventricular hemorrhage. Three patients have left vocal cord paralysis, which has been associated with chronic aspiration in one. It is possible that other neurologic events may have occurred in nonsurvivors.
Discussion Interrupted aortic arch and coarctation plus severe arch hypoplasia are uncommon congenital malformations that occur with associated intracardiac congenital anomalies of varying severity.v " A variety of surgical procedures have been used for treatment of these anomalies. Left thoracotomy for repair of the arch is complicated by poor exposure of the proximal arch and a marked difficulty in palliating more complicated lesions such as
TGA, truncus arteriosus, and aortic or subaortic stenosis.2, 3, 8 Furthermore, for patients with complex coarctation of the aorta plus hypoplastic transverse arch, both operative mortality and recoarctation rate are higher if the hypoplastic segment is disregarded, and the residual obstruction will compound left ventricular dysfunction. The hospital mortality rate in series in which this approach was used ranged from 41% to 50%, and longterm survival has been poor. This situation has stimulated interest in the one-stage approach now advocated for interrupted aortic arch in most centers. The best approach for coarctation plus hypoplastic arch, however, remains a controversy. The first successful one-stage correction of type A interrupted aortic arch was performed by Barratt-Boyes and associates? in 1970. Murphy and colleagues 10 reported correction of a type B interrupted aortic arch in 1973. Barratt-Boyes used a left posterolateral thoracotomy and midsternotomy for his repair, and Murphy extended his midsternotomy incision into the left third intercostal space for better exposure of the descending aorta. In both instances an interposition graft was used. In 1975, Tru-
694
The Jourrialof Thoracic and Cardiovascular Surgery
Karl et al.
sler and Izukawa 11 reported correction of a type B interruption with a direct anastomosis of the descending aorta and closure of the YSD through the midline in a 13-dayold baby. By 1979, however, only four cases of successful neonatal repair of interrupted aortic arch had been reported.l? Recently, several groups have presented series of patients treated by total repair of the arch and intracardiac anomalies simultaneously through midline sternotomy.': 2, 4,13-16 Early mortality rates have varied from 8.3% to 30%, and all have recommended primary repair as the best approach for interrupted aortic arch with YSD. Our own 6% operative mortality rate for repair of interrupted aortic arch (8% for all types of arch reconstruction with biventricular repair) tends to support this philosophy. We believe that a large direct anastomosis is essential for effective arch reconstruction and that this is best achieved via a sternotomy for both interrupted and hypoplastic arch. The operative technique is now standardized and the results are reproducible for a variety of lesions. The problem of late recurrence of arch obstruction has been relatively infrequent and will probably be treatable with balloon dilation in the majority of cases in the future. Repair by sternotomy compares favorably in this respect with repair of isolated coarctation via left thoracotomy in newborn infants. The problem of severe left ventricular outflow tract obstruction, especially with interrupted aortic arch, should be addressed at the time of the initial operation. Direct muscle resection appears to provide effective relief, although YSD closure may itself tend to reduce the gradient. We expect to see further recurrence of subaortic stenosis as follow-up time increases. The presence of arch obstruction in patients with univentricular heart strongly suggests that subaortic stenosis may be present or developing. This tendency may be increased by pulmonary artery banding. The most difficult patients in this group are the neonates with duct-dependent circulation, arch obstruction, and established subaortic stenosis (usually in the setting of atrioventricular valve atresia or double-inlet left ventricle with TGA). Dissatisfied with results of Norwood type procedures in this group, we currently prefer arterial switch plus atrial septectomy concomitant to arch repair. The restrictive bulboventricular foramen then controls pulmonary blood flow, with a view to a subsequent Fontan operation. Our overall approach to subaortic stenosis in the univentricular heart has recently been reviewed. 17 A serious complication after direct anastomosis of the arch was left bronchial compression by the reconstructed aorta. In two of our patients with interrupted aortic arch and truncus arteriosus, left bronchial compression may have been related to construction of too proximal an aor-
tic anastomosis. We now believe that this complication may be avoided by making the anastomosis more distally in the ascending aorta. This may leave a short segment of hypoplastic ascending aorta (between the origin of the pulmonary arteries and the descending aortic anastomosis), which in one of our patients with truncus and interrupted arch necessitated subsequent revision. In such cases this segment should perhaps be enlarged with a pericardial or homograft patch at the time of initial repair. A more extensive review of our experience with truncus and interrupted arch appears in another publica-
tion." In conclusion, primary repair of interrupted aortic arch and coarctation plus hypoplastic arch compares favorably with staged repairs and can be performed in critically ill newborn infants with reasonable risk and good mediumterm outcome. Isolated myocardial perfusion during arch repair reduces the required cardiac ischemic time and may improve operative mortality. Results for arch repair plus palliation of univentricular heart and subaortic stenosis by arterial switch and septectomy are currently under evaluation, and improved results are expected in this group as well. One must remain aware of the potential for bronchial compression after arch repair, especially with truncus and interrupted aortic arch. Severity of associated intracardiac lesions should not be a contraindication to one-stage repair in the majority of newborn infants with severe arch obstruction. REFERENCES 1. NorwoodWI, Lang P, Castaneda AR, HougenTJ. Repar-
2. 3.
4. 5. 6.
ativeoperationsfor interrupted aortic arch with ventricular septaldefect.J THORAC CARDIOVASC SURG 1983;86:32-7. Scott WA, Rocchini AP, Bove EL, et al. Repair of interrupted aortic arch in infancy.J THORAC CARDIOVASC SURG 1988;96:564-8. Hammon JW, Merrill WH, Prager RL, et al. Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair. Ann Thorac Surg 1986;42:17-21. Schumacher G, Schreiber R, Meisner H, et al. Interrupted aortic arch: natural history and operative results. Pediatr Cardiol 1986;7:89-93. Sano S, Mee RBB. Isolated myocardial perfusion during arch repair. Ann Thorac Surg 1990;49:97-2. Van Praagh R, Bernhard WF, Rosenthal A, et al. Interrupted aortic arch: surgicaltreatment. Am J Cardiol 1971; 27:200-11.
7. Bharati S, Lev M. The surgical anatomy of the heart in tubular hypoplasia of the transverseaorta (preductalcoarctation). J THORAC CARDIOVASC SURG 1986;91:79-85. 8. Vouhe PR, Tringuet F, et al. Aortic coarctation with hypoplastic aortic arch: resultsof extendedend-to-end aor-
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9.
10.
II.
12.
tic arch anastomosis. J THORAC CARDIOVASC SURG 1988; 96:557-63. Barratt-Boyes BG, Nicholls TT, BrandtPWT, NeutzeJM. Aortic arch interruption associated with patent ductus arteriosus, ventricular septal defect, and total anomalous pulmonary venous connection. J THORAC CARDIOVASC SURG 1972;62:367-73. Murphy DA, Lemire GG, Tessler I, Dunn GL. Correction oftype B aortic arch interruption with ventricular and atrial septal defects in a three-day-old infant. J THORAC CARDIOVASC SURG 1973;65:882-6. Trusler GA, Izukawa T. Interrupted aortic arch and ventricular septal defect: direct repair through a median sternotomy incision in a 13-day-old infant. J THORAC CARDIOVASC SURG 1975;69:126-31. Van der Horst R, Hastreiter AR, Levitsky S, Fisher EA, DuBrow IW, Weinberg M. Interrupted aortic arch operation in the first week of life: hemodynamic and angiographic evaluation one year later. Ann Thorac Surg 1979;27:11220.
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13. Turley K, Yee ES, Ebert PA. The total repair of interrupted arch complex in infants: the anterior approach. Circulation I984;70(Pt 2):116-20. 14. Moulton AL, Bowman FO Jr. Primary definitive repair of type B interrupted aortic arch, ventricular septal defect, and patent ductus arteriosus: early and late results. J THORAC CARDIOVASC SURG 1981;82:501-10. 15. Monro JL, Bunton RW, Sutherland GR, Keeton BR. Correction of interrupted aortic arch. J THORAC CARDIOVASC SURG 1989;98:421-7. 16. Sell JE, Jonas RA, Mayer JE, et al. The results of a surgical program for interrupted aortic arch. J THORAC CARDIOVASC SURG 1988;96:864-77. 17. Karl TR, Watterson KG, Sano S, Mee RBB. Surgery for subaortic stenosis in univentricular hearts. Ann Thorac Surg [In press]. 18. Sano S, Brawn WJ, Mee RBB. Repair of truncus arteriosus and interrupted aortic arch. J Cardiac Surg 1990;5: 15762.
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