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Ann Thorac Surg 1997;64:503-507
© 1997 The Society of Thoracic Surgeons


Original Articles: Cardiovascular

Arch Growth After Staged Repair of Interrupted Aortic Arch Using Carotid Artery Interposition

Mehdi Hakimi, MD, Sandra K. Clapp, MD, Henry L. Walters, III, MD, Juanita M. Lyons, MS, W. Robert Morrow, MD

Department of Cardiovascular Surgery and Section of Cardiology, Children's Hospital of Michigan and Wayne State University, Detroit, Michigan

Accepted for publication January 21, 1997.


    Abstract
 Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 
Background. Between 1980 and 1990, our practice was to perform carotid artery interposition as part of a staged repair of interrupted aortic arch with various associated cardiac defects.

Methods. This procedure was used in 16 patients with IAA type B. The median age at operation was 4.5 days and the median weight, 3.2 kg. Ten of the patients had an associated ventricular septal defect. Six more had complex anatomy. There were two deaths at carotid interposition, two interim deaths, and two deaths after intracardiac repair. Preoperative echocardiographic and angiographic studies were compared with postoperative studies in 11 survivors of arch repair to assess sequential growth of the interposed carotid artery. Measurements of the carotid artery were normalized to the descending aorta.

Results. Preoperatively, the left carotid artery had a median diameter of 3.7 mm and was 42.9% of the descending aortic diameter. Postoperative studies performed at a median age of 5.7 months disclosed that the interposed carotid artery had grown to a median diameter of 7.0 mm and was 69.6% of the diameter of the descending aorta (normal >= 81%). On follow-up at a median time of 4 years, 6 of 9 patients have no gradient by blood pressure measurements or echocardiographic Doppler studies, and 3 have modest gradients. No patient has required revision of the arch repair.

Conclusions. Survival is good after carotid artery interposition for interrupted aortic arch, and growth of the carotid artery approaches that of a normal arch. Carotid artery interposition is a viable alternative for repair of this lesion should primary definitive repair not be feasible.


    Introduction
 Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 
Interrupted aortic arch (IAA) is an uncommon form of congenital heart disease first described in the late 18th century by Steidele [1]. Although comprising only 1.4% of congenital cardiac anomalies, arch interruption with associated congenital heart disease is responsible for 4% of congenital heart disease–related deaths [2, 3]. The first successful arch reconstruction for IAA type B was performed by Merrill and associates [4] in 1957 using the proximal ductus arteriosus to complete the anastomosis in a 31/2-year-old boy. In 1959, Quie and colleagues [5] described repair of IAA type B using the left carotid artery in a 4-month-old infant. Since then, various two-stage procedures using endogenous vessels [6] or polytetrafluoroethylene grafts [1, 3] have been advocated for repair of IAA.

More recently, primary definitive repair of IAA with ventricular septal defect (VSD) has become the operation of choice, with outcomes similar to those for staged repairs [79]. At our institution, we have been performing primary complete repair since 1990 and consider this the treatment of choice for all types of IAA. Prior to 1990, we typically performed staged repair using carotid artery interposition for repair of type B IAA. In this report, we present our experience with staged repair using carotid artery interposition for the initial arch repair. This alternative operation is appropriate in the very rare circumstance when single-stage complete repair might not be possible.


    Material and Methods
 Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 
From January 1, 1980, through December 31, 1991, 16 patients had staged repair of type B IAA using carotid artery interposition at Children's Hospital of Michigan. All children were seen with congestive heart failure. There were 6 boys and 10 girls. The median age of the group at intervention was 4.5 days (range 1 to 50 days), and the median weight was 3.2 kg (range, 1.9 to 3.8 kg). The condition of 14 patients was stabilized with prostaglandin E1. Five of the 14 patients required additional inotropic support, and 3 of those 5 also were given sodium bicarbonate intravenously to correct metabolic acidosis in preparation for catheterization and operation. All 16 patients had IAA type B. Associated lesions are listed in Table 1Go.


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Table 1. . Associated Lesions and Initial Procedures
 
Surgical Technique
In each patient, the thoracic cavity was entered through a posterolateral thoracotomy in the fourth intercostal space. The thoracic aorta and the ductus arteriosus as well as the ascending aorta and its branches were completely dissected. The left carotid artery was mobilized extensively and ligated in the base of the neck. The carotid artery was then divided and its end beveled. This vessel was anastomosed to the proximal descending aorta in an end-to-end fashion in 13 patients (81%) (Fig 1Go) and in an end-to-side fashion in 3 patients (19%). In the end-to-end technique, the ductus arteriosus was ligated and divided, and all ductal tissue was removed from the descending aorta before the anastomosis was done. In the end-to-side technique, the anastomosis was made to the lateral aspect of the descending aorta opposite the ductus arteriosus.



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Fig 1. . Carotid artery interposition repair of interrupted aortic arch. ( LCA = left carotid artery; LSCA = left subclavian artery; RCA = right carotid artery; RSCA = right subclavian artery.)

 
The suture technique was continuous in 7 patients (44%) and interrupted in 2 (13%). A combination of interrupted and continuous suture technique was used in 7 patients (44%). In 7 patients, 7-0 Prolene (Ethicon, Somerville, NJ) suture was used and in 9 patients (56%), 7-0 polydioxanone suture.

Aortic cross-clamp times ranged from 12 minutes to 38 minutes (median duration, 21.0 minutes). Intraoperative pressure measurements were performed after completion of the anastomosis in 9 patients (56%); gradients ranged from 0 mm Hg to 5 mm Hg. Pulmonary artery banding was performed simultaneously in 8 patients (50%).

Arch Growth Assessment
Arch measurements were performed by a single cardiologist (S.K.C.). All preoperative and postoperative catheterization studies were reviewed for optimal views of the ascending aorta, great vessels, arch, and descending aorta in the anteroposterior (AP) and lateral planes. All measurements in a given projection were taken from the same angiogram to minimize magnification error. For cineangiograms, the internal diameter of the catheter was measured and compared with its known size. The resultant ratio was applied to the vessel measurements in that plane for magnification correction. For digital subtraction angiograms, either the catheter diameter or a sphere of known size was used for calibration, permitting computerized direct measurement of vessel diameters.

The following vessel diameters were measured in the anteroposterior and lateral projections whenever possible: ascending aorta just above the sinuses of Valsalva, distal ascending aorta, proximal innominate artery, proximal left carotid artery, upper descending aorta below the insertion of the ductus arteriosus, and descending aorta at the diaphragm. Postoperatively, the diameter of the transverse arch (carotid interposition) was measured just distal to the innominate artery and again just proximal to the descending aorta. In the preoperative studies, the diameter of the left carotid artery was compared with that of the descending aorta at the diaphragm. In the postoperative studies, the proximal and distal transverse "carotid" arch diameters were compared with the diameter of the descending aorta at the diaphragm and with published normal diameters for age [10, 11]. Five patients underwent operation after diagnosis by echocardiography, and the echocardiographic measurements of the left carotid artery and descending aorta were used as the preoperative measurements.

To further assess the adequacy of the reconstructed arch, cardiology records were reviewed for arm versus leg blood pressure gradients and echocardiographic Doppler gradients at the last follow-up visit. Recent catheterization data were also included if available. All hospital records were reviewed for evidence of early or late neurologic sequelae that could be attributed to use of the carotid artery, including results of cranial computed tomography, magnetic resonance imaging, and neurologic follow-up.


    Results
 Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 
Operative Mortality
Ten of the 16 patients with IAA type B had uncomplicated anatomy with associated large malalignment VSDs. Five of these 10 underwent pulmonary artery banding. There was one death in this group. It occurred 8 hours after arch repair and was precipitated by an episode of profound bradycardia and hypotension. Postmortem examination did not disclose additional defects, problems with the arch reconstruction, or definitive cause of death. The remaining 9 patients underwent intracardiac repair at a median age of 10.7 months (range, 0.7 to 27.7 months), and all survived. In the 5 patients with pulmonary artery banding, VSD closure was performed at a median age of 11.0 months (range, 5.3 to 27.7 months). None of these 5 had development of subaortic stenosis before or after VSD closure. In 1 patient with a large malalignment VSD, subaortic stenosis developed after VSD closure performed when the patient was 8 months of age. This patient had not undergone pulmonary artery banding. The subaortic stenosis was resected without complications when the patient was 31 months old.

In the 6 patients with IAA type B with associated complex malformations, there was one operative death after carotid interposition, two interim deaths prior to the second-stage repair, and two operative deaths after the second-stage repair.

Arch Growth
Thirteen children had sequential data before and after arch reconstruction. In 1 patient, the quality of the postoperative angiograms prohibited accurate measurements, and in another, follow-up studies were performed at another institution, and the angiograms could not be obtained for review. The remaining 11 patients make up the population for sequential arch-growth studies.

The median left carotid artery diameter preoperatively was 3.7 mm and was 42.9% (range, 30.6% to 100%) of descending aortic diameter at the diaphragm (Table 2Go). The postoperative studies were performed at a median age of 5.7 months (range, 16 days to 36.3 months). The proximal transverse arch was similar to the distal transverse arch in the AP plane (p = 0.813) and in the lateral plane (p = 0.23) (p values, Wilcoxon matched-pairs signed-rank test). Therefore, the proximal AP and lateral transverse arch measurements were used as representative. The median AP proximal transverse arch diameter was 5.9 mm, and the lateral proximal transverse arch diameter was 7.0 mm (p = 0.68; Wilcoxon matched-pairs signed-rank test). These were 77.8% (range, 46.4% to 100%) and 69.6% (range, 59.6% to 98.9%), respectively, of the diameter of the descending aorta at the diaphragm in the same plane (see Table 2Go). The 2 patients with the smallest interposed carotid arches were 16 and 18 days of age at postoperative study. The next two smallest arches were in the 2 patients who had a residual gradient by echocardiographic Doppler studies and blood pressure measurements at follow-up. The referenced normal ratio of isthmus to descending aorta of 0.81 or 81% is used for comparison [10].


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Table 2. . Arch Growth Data
 
Ten patients are long-term survivors with a completed intracardiac repair or palliation. One of the 10 has moved from the area, and no follow-up data are available. The remaining 9 patients have been followed for a median time of 4 years (range, 3 to 12 years). Six have no gradient by arm and leg blood pressure measurements or echocardiographic Doppler studies. Echocardiographic Doppler study in another patient suggested a transverse arch gradient of less than 20 mm Hg. Subsequent catheterization in that patient disclosed a 6 mm Hg gradient. One patient has a gradient of less than 20 mm Hg by blood pressure measurement and less than 10 mm Hg by Doppler studies. In 1 patient, arm blood pressure cannot be used to determine the arch gradient because both subclavian arteries arise distal to the arch reconstruction. This patient had a gradient lower than 30 mm Hg at the last catheterization in 1993 and had a 30 mm Hg gradient by the last echocardiogram in 1993.

Neurologic Status
Five patients have had neurologic evaluations at different times during the course of their operations and follow-up. In 2, seizures occurred transiently after arch reconstruction, and electroencephalograms in both initially showed epileptiform activity. One of the 2 had DiGeorge syndrome, and seizures were attributed to hypocalcemia. In both, evaluations including cranial ultrasound and computed tomographic scans were normal. Each is now neurologically normal and is not on a regimen of anticonvulsant medications. Another child had a neurologic evaluation for dysmorphic features and abnormal Moro's reflex postoperatively. Cranial computed tomographic scan was normal, and the child is neurologically normal at last follow-up.

One child had seizures, difficulty walking, and choreoathetoid movements 5 days after discharge from the second intracardiac operation at 31 months of age for repair of subaortic stenosis. She had previously been noted to be developmentally delayed with unusual facial features. Magnetic resonance imaging study was negative, and the electroencephalogram showed diffuse disorganized activity. Her neurologic evaluation and follow-up did not suggest any event related to the three operations as the cause of the neurologic findings, and the etiology remains unknown.

Another child, a 6-year-old girl, has migraine headaches. She has a strong family history of migraines in her mother and grandmother. She is also the patient with a 30 mm Hg gradient across the reconstructed arch and resultant elevated perfusion pressure to the single carotid artery. It is unclear whether the altered central nervous system blood flow created by the carotid interposition contributed to the early development of the migraine headaches. She has a normal cranial computed tomographic scan and a normal magnetic resonance imaging study.


    Comment
 Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 
With the improving results of surgical intervention in newborns [12, 13], our center, like others, has moved to primary definitive repair of IAA as the clear procedure of choice. This includes patients with major associated congenital heart defects such as tricuspid atresia with transposition of the great vessels in whom we now routinely perform a Norwood-Stansel type of procedure.

This review of our experience with staged repair demonstrates that carotid artery interposition for IAA with isolated VSD is associated with good survival (90%). However, the overall long-term survival in the patients with complex anatomy was low. This was, in part, due to timing of the second-stage repair, which was delayed to 6 months of age, as was our practice during the earlier part of the study.

Although staged repair is no longer the first choice for IAA, the surgical results reported here should make carotid artery interposition the primary alternative technique for any rare circumstance when total correction cannot be performed. The primary advantage of this alternative technique is that it involves the use of autogenous tissue with growth potential. The primary liability of this technique is the unknown long-term neurologic consequence of sacrifice of the left common carotid artery.

Others [6, 14] have reported single cases of arch growth in patients in whom the carotid artery was interposed to repair IAA. In this study, all but the 2 patients who were still newborns at the time of follow-up catheterization have shown satisfactory arch growth. Even though there are measurement errors inherent to retrospective catheterization studies, we minimized these errors by looking at the ratios of the arch diameter to the diameter of the descending aorta. All patients who had no arch gradient at follow-up catheterization had an arch ratio of greater than 80% in either the AP or lateral view. The 2 older patients who had an arch diameter less than 75% of the descending aortic diameter had arch gradients at catheterization that were not clinically significant. Our data are consistent with the previous autopsy series of Van Meurs-Van Woeznik and associates [10] where a transverse arch to descending aorta ratio of greater than 0.81 was normal. Our findings clearly show that carotid arteries, when interposed to repair aortic arch interruption, have the potential for growth and can attain normal size.

Others [3, 15, 16] who have performed staged repairs have frequently reconstructed the arch with a polytetrafluoroethylene graft, with or without additional pulmonary artery banding. A review of reports from centers where staged repairs have been performed discloses an overall survival rate of 60% to 70% [3, 6, 1518]. Major disadvantages of staged repair using a polytetrafluoroethylene graft in the neonate have been that the graft has no potential for growth and is not amenable to balloon angioplasty. Therefore, all of these patients will eventually have to undergo a very difficult graft replacement procedure. On the other hand, the carotid artery interposition technique not only has growth potential but also is amenable to balloon angioplasty. There has been concern that the pulmonary artery banding sometimes used in staged repair could provoke or accelerate development of subaortic stenosis, particularly when the VSD is associated with a malaligned conal septum [2, 7, 18]. We did not see this problem in our limited number of patients who had pulmonary artery banding.

There is always concern that neurologic damage may occur when a carotid artery is sacrificed. Two recent reports [19, 20] summarizing many years of experience with extracorporeal membrane oxygenation in more than 7,000 babies have shown that there is no indication of damage to the right cerebral hemisphere resulting from the procedure when the right common carotid artery is ligated. Our experience in a limited number of patients also did not show detectable neurologic abnormalities directly attributable to the use of the left carotid artery for aortic arch repair. It is difficult to distinguish the morbidity attributable to carotid artery interposition from the neurologic effects of the underlying cardiac illness and the associated noncardiac syndromes.

In summary, staged repair of IAA using the left carotid artery can be performed successfully with reasonable outcome. The interposed carotid artery does grow and frequently attains normal arch size. Although primary single-stage repair is the procedure of choice, the two-stage repair reported here is applicable in the very rare circumstance when primary definitive repair cannot be performed.


    Footnotes
 Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 
Address reprint requests to Dr Walters, Department of Cardiovascular Surgery, Children's Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201.


    References
 Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 

  1. Singh MP, Bentall HH, Oakley CM. Successful total correction of congenital interruption of the aortic arch and ventricular septal defect. Thorax 1970;25:615–23.[Abstract/Free Full Text]
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  4. Merrill DL, Webster CA, Sampson PC. Congenital absence of the aortic isthmus. J Thorac Cardiovasc Surg 1957;33:311–6.
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  9. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:864–77.[Abstract]
  10. Van Meurs-Van Woeznik H, Klein HW, Krediet P. Normal internal calibres of ostia of great arteries and of aortic isthmus in infants and children. Br Heart J 1977;39:860–5.[Abstract/Free Full Text]
  11. Clarkston PM, Brandt PWT. Aortic diameters in infants and young children: normative angiographic data. Pediatr Cardiol 1985;6:3–6.[Medline]
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  13. Bailey LL, Jacobson JG, Doroshow RW, Merritt WH, Petry EL. Anatomic correction of interrupted aortic arch complex in neonates. Surgery 1981;89:554–7.
  14. Fishman NH, Bronstein MH, Berman W, et al. Surgical management of severe aortic coarctation and interrupted aortic arch in neonates. J Thorac Cardiovasc Surg 1976;71:35–48.[Abstract]
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  17. Qureshi SA, Maruszewski B, McKay R, Arnold R, West CA, Hamilton DI. Determinants of survival following repair of interrupted aortic arch in infancy. Int J Cardiol 1990;26:303–12.[Medline]
  18. Trusler GA, Freedom RM. Surgical approach to the management of interruption of the aorta. In: Godman MJ, Marquis RM, eds. Surgical approach to the management of interruption of the aorta; vol 2. Edinburgh: Churchill Livingstone, 1979:268–73.
  19. Kanto WP. A decade of experience with neonatal extracorporeal membrane oxygenation. J Pediatr 1994;124:335–47.[Medline]
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