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Ann Thorac Surg 1997;63:975-980
© 1997 The Society of Thoracic Surgeons

Original Article: Cardiovascular

Management of Arch Hypoplasia After Successful Coarctation Repair

Maryann M. DeLeon, MD, Serafin Y. DeLeon, MD, Jose A. Quinones, MD, Patrick T. Roughneen, MD, Kathy E. Magliato, MD, Dolores A. Vitullo, MD, Frank Cetta, MD, Timothy J. Bell, MD, Elizabeth A. Fisher, MD

Departments of Thoracic-Cardiovascular Surgery and Pediatrics, Stritch School of Medicine, Maywood, Illinois

Accepted for publication October 18, 1996.


   Abstract
Top
 Footnotes
 Abstract
Introduction
 Material and Methods
 Results
 Comment
 Acknowledgments
 References
 
Background. Pronounced arch obstruction can be seen after a well-repaired coarctation, and this probably results from the failure of a somewhat hypoplastic arch to grow or from clamp injury at the time of the initial repair, or from both causes. Because of mediastinal adhesions and minimal collateral circulation, use of extraanatomic bypass grafts appears to be the preferred approach.

Methods. Six children or young adults presented with arch obstruction over a 3-year period. Their mean age was 13.5 ± 4 years, and the mean interval from the time of the initial repair was 10 ± 4 years. The mean age of the patients at the time of the initial repair was 3.2 ± 5 years. Symptoms included exertional headache and chest pain. The mean systolic gradients, as shown by echocardiography and cardiac catheterization, were 34 ± 7 mm Hg and 33 ± 6 mm Hg, respectively. Repair was accomplished through a midsternotomy using a polytetrafluoroethylene patch placed in the concavity of the arch, which extended from the ascending to the descending aorta. Dissection was kept close to the aorta and arch to minimize injury to the phrenic and recurrent laryngeal nerves. Cardiopulmonary bypass and moderate hypothermia (25° to 27°C bladder temperature) without circulatory arrest were used.

Results. All patients were discharged home 4 to 20 days postoperatively (mean, 7 ± 6 days). All patients were found to be normotensive at a mean follow-up of 1.3 ± 1 years. Postoperative echocardiograms, which were obtained in all patients, revealed no residual gradients. Exercise blood pressure was evaluated in 2 patients and found to be normal.

Conclusions. Transsternal arch enlargement using cardiopulmonary bypass and moderate hypothermia without circulatory arrest is an attractive and safe approach for the treatment of arch obstruction after coarctation repair. Unlike the use of extraanatomic bypass grafts, it allows complete relief of the obstruction, unhampered aortic growth, the minimal use of foreign material, and a repair that is protected deep within the mediastinal space.


   Introduction
Top
 Footnotes
 Abstract
 Introduction
Material and Methods
 Results
 Comment
 Acknowledgments
 References
 
See also page 980.

Pronounced obstruction resulting from arch hypoplasia developing several years after the successful repair of aortic coarctation is not a well-recognized problem, especially in the absence of associated intracardiac defects. Such obstruction occurs in a somewhat hypoplastic arch that has either failed to grow or was injured by the vascular clamps applied at the initial repair [1–3].

Because of the presence of mediastinal adhesions and the usual absence of significant collateral circulation, the considerations in the management of "isolated" arch obstruction are similar to those in the treatment of recurrent coarctation. Options for the management of recurrent coarctation, such as the use of an extraanatomic bypass graft (ascending to descending aorta) and direct repair with or without the aid of cardiopulmonary bypass, are also options for the treatment of arch obstruction [4–7].

Because of the inherent problems posed by extraanatomic bypass grafts in growing patients, however, we used transsternal arch enlargement procedures for the management of arch obstruction. This repair extends from the ascending to the descending aorta and involves the use of cardiopulmonary bypass and moderate hypothermia without circulatory arrest. To determine the usefulness of this approach, we reviewed our experience and now report our findings.


   Material and Methods
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
Results
 Comment
 Acknowledgments
 References
 
Over a 3-year period ending in August 1996, 6 children or young adults underwent arch enlargement procedures for the management of arch hypoplasia after a well-repaired coarctation of the aorta. The clinical data, surgical technique, and results were analyzed.

Clinical Data
The study included 5 male patients and 1 female patient. Most of the patient data are summarized in Table 1Go. The age of the patients at the time of the initial coarctation repair ranged from 15 days to 12 years (mean, 3.2 ± 5 years). In 3 patients the first repair for coarctation was done at an early age (15 days to 3 months). One patient underwent concomitant pulmonary artery banding for the treatment of a ventricular septal defect, which closed spontaneously. Debanding was performed at 1.5 years of age. All 3 patients had recurrent coarctation that was managed with repatching in 2 and balloon dilation in 1.


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  		Table 1. . Patient Data
 
In the other 3 patients, initial repair was done at an older age (3, 4, and 12 years). One patient, who had had a patch aortoplasty at 3 years of age, subsequently underwent left carotid–descending aorta bypass grafting at 12 years of age for the management of distal arch hypoplasia.

All patients were known to have no evidence of residual coarctation or an arch gradient until they presented to us with arch obstruction. The subsequent arch obstruction was probably due to failure of the arch to enlarge with the growth of the child. The ages of the patients at this time ranged from 9 to 18 years (mean, 13.5 ± 4 years), with a mean interval from the time of the first repair of 10 ± 4 years (range, 3 to 12 years). The patients who underwent repair of their coarctation at an early age presented with arch obstruction at 9, 10, and 12.5 years of age. All were asymptomatic. The entire arch was hypoplastic, but the more notable obstruction was located at the distal arch (Fig 1Go). The 3 patients who underwent their initial coarctation repair at an older age presented with arch obstruction at 15, 16, and 18 years of age. All of them were symptomatic, however, with elevated systolic blood pressures in the right arm ranging from 150 to 160 mm Hg. All complained of headache and chest pain with exertion. Two patients underwent exercise testing, which showed a severe elevation of their systemic systolic blood pressure. The entire arch was hypoplastic (Fig 2Go). One patient, who had undergone the initial procedure at 12 years of age, presented with arch obstruction after a growth spurt of 15 cm and a weight gain of 17 kg in 3 years.



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  		Fig 1. . Arch angiogram of a 10-year-old girl who had a subclavian artery flap repair at 15 days of age. The entire arch is hypoplastic, with the narrowest segment (three arrows) proximal to the vertebral artery (two arrows).

 


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  		Fig 2. . (A) Arch angiogram of an 18-year-old male patient who had a patch aortoplasty at 3 years of age, followed by a left carotid–descending aorta bypass graft (black arrows) at 12 years of age for the treatment of distal arch hypoplasia. The entire arch is hypoplastic (white arrows). (B) Arch angiogram of a 15-year-old male patient who had a patch aortoplasty at 12 years of age. The entire arch is hypoplastic, with the narrowest segment proximal to the left subclavian artery (arrows).

 
Operative Techniques
Partial cardiopulmonary bypass was established through a midsternotomy incision, with a single arterial cannula in the ascending aorta and a single venous cannula in the right atrium. Moderate hypothermia, ranging from a bladder temperature of 25° to 27°C, was achieved. The arch, arch vessels, and descending aorta were exposed during cooling (Fig 3Go), with the dissection kept close to the aorta to minimize injury to the phrenic and recurrent laryngeal nerves.



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  		Fig 3. . Application of vascular clamps. (A) The dotted line shows the location and extent of the incision. (B) The polytetrafluoroethylene patch extends from the ascending aorta to the descending aorta.

 
Once the desired core temperature was reached, clamps were applied to the ascending and descending aorta (Fig 3Go). The left subclavian artery or vertebral artery, if present, was temporarily occluded. The incision was initially made on the descending aorta and continued in a retrograde fashion to the arch. A polytetrafluoroethylene patch was fashioned and sutured in place with continuous polytetrafluoroethylene sutures. The polytetrafluoroethylene patch used is thin walled (0.64 mm) and quite pliable. It molds well to the aortic arch, and with the use of polytetrafluoroethylene suture, bleeding from the needle hole is negligible. The patch is cheaper and more readily available than a homograft. Suturing was begun in the distal aspect of the descending aorta, and as it approached the ascending aorta, the proximal clamp was adjusted so that the incision on the arch concavity could be extended. The aortic cannula was repositioned into the innominate artery in 3 patients and a tourniquet placed around it, thereby maintaining cerebral perfusion. The ascending aorta was clamped and blood cardioplegic solution administered in these 3 patients. This allowed liberal extension of the patch into the ascending aorta (Fig 4Go). The polytetrafluoroethylene patch in all patients extended from the ascending aorta to the arch to the descending aorta.



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  		Fig 4. . (A) Intraoperative photograph showing the accessibility and exposure of the ascending aorta (AAo), transverse arch (TA), and descending aorta (DA) through a median sternotomy. (B) Intraoperative photograph showing the polytetrafluoroethylene patch (PP) used for the arch enlargement.

 

   Results
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
Comment
 Acknowledgments
 References
 
All patients were discharged from the hospital 4 to 20 days postoperatively (mean, 7 ± 6 days). The patient who stayed in the hospital for 20 days did so because he required operative drainage of pericardial and pleural effusions. Because of the friability of the distal and proximal descending aorta at the time of repair, cardiac catheterization was performed in this patient before drainage of the effusions to rule out the possibility of a false aneurysm at the suture line. No aneurysm or arch gradient was found.

Postoperative echocardiograms were obtained in all patients, and no patient was found to have a residual gradient. Two patients underwent exercise testing, which showed a normal blood pressure response. All patients are doing well and are normotensive at 2 months to 3 years postoperatively (mean, 1.3 ± 1 years).


   Comment
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
Acknowledgments
 References
 
Arch hypoplasia has become an important and interesting issue from the standpoint of the management of aortic coarctation. Several authors have attempted to better define aortic arch hypoplasia anatomically. The portion of the aortic arch that extends from the innominate artery to the left subclavian artery is divided into proximal and distal segments by the left carotid artery. According to Moulaert and colleagues [8], a segment of the aortic arch is considered hypoplastic if its diameter is less than 50% of the diameter of the ascending aorta. Morrow and colleagues [9] have shown that, in cases of isolated coarctation in neonates, the arch–ascending aorta ratio is 0.54, as opposed to 0.76 in comparable normal neonates (p < 0.001). They also found that the ascending aorta is smaller in the setting of coarctation than it is in normal patients (6.6 mm versus 7.3 mm; p = <0.01), whereas the descending aorta is the same in both groups (5.9 mm), making the arch–descending aorta ratio more reliable in determining whether arch hypoplasia is present. The size of the ascending aorta can also be influenced by any associated lesions that may be present, such as bicuspid aortic valves and ventricular septal defects.

Although the incidence of pronounced arch hypoplasia is higher in patients with coarctation associated with intracardiac defects (complex coarctation), some degree of arch hypoplasia is also present in most patients with isolated coarctation [9, 10]. Currently it is not clear, which hypoplastic arch should be managed at the initial repair of a coarctation. However, this question appears to be more relevant to infants. In the absence of intracardiac shunts, repair of the coarctation alone allows for growth of the hypoplastic arch [10, 11]. However, when the arch is clearly hypoplastic, extended end-to-end repair has been successfully used to manage both the coarctation and the arch hypoplasia [12, 13].

If there are intracardiac shunts, both the coarctation and hypoplastic arch probably should be repaired to improve outcome. Extended end-to-end repair and pulmonary artery banding have been utilized successfully in several centers [12]. However, with the improvement in cardiopulmonary bypass techniques and in the accessibility of the entire aortic arch and descending aorta through a midsternotomy, the simultaneous repair of an aortic obstruction and intracardiac defects has become a quite feasible and viable option [14–16]. The coarctation and hypoplasia of the distal arch can be easily managed with an extended end-to-end repair performed through a midsternotomy. When there is hypoplasia of the whole arch, arch enlargement with a patch placed at the concavity of the arch that extends from the ascending aorta to the descending aorta together with the excision of ductal tissue is an attractive option [15].

Beyond infancy, the dilemma surrounding arch hypoplasia with coarctation appears to diminish in importance, especially in patients with an isolated coarctation. However, our experience indicates that this problem may not be so uncommon. Our patients initially did not have pronounced arch obstruction, but the arch did not grow proportionally with the child. Clamp injury may have also contributed to the problem. It is therefore important that patients who appear to have no residual aortic obstruction be followed up more closely during or after their growth spurt, as was done in our patients.

Management options for recurrent coarctation, such as the use of ascending–descending aorta bypass grafts, can also be used in the event of an isolated arch obstruction. For the management of recurrent coarctation these grafts have been implanted through a left or right thoracotomy, a midsternotomy, or a combination of a left thoracotomy and midsternotomy [5, 6, 17]. Other extraanatomic grafts, such as ascending–abdominal aorta and axillofemoral grafts, have also been used for this purpose [5, 6]. Bypass grafts have been favored to circumvent the problem with the formation of mediastinal adhesions and avoid the need for aortic cross-clamping to minimize the spinal cord ischemia resulting from minimal collateral circulation. Bypass grafts, however, are not suitable for young patients because of growth considerations. Additionally, the use of bypass grafts entails the introduction of a considerable amount of foreign material that may predispose to the development of infection and to disruption resulting from blunt trauma.

Direct patch enlargement is also an option for recurrent coarctation. Ralph-Edwards and colleagues [4] reported on 43 patients who underwent operation for recoarctation. They reported no case of spinal cord injury despite using no mechanical support for circulation during the repair of coarctation in 29 patients. They also reported on 11 patients with an associated arch obstruction who underwent patch enlargement through a median sternotomy with cardiopulmonary bypass and profound hypothermia and circulatory arrest. Two of the three deaths occurred in this group, and 2 patients from the cardiopulmonary bypass group suffered seizures and impaired consciousness. In our experience, circulatory arrest is only needed for enlargement of the whole arch in infants, because of the difficulty in applying partially occluding clamps on the ascending aorta [15]. The use of profound hypothermia and circulatory arrest should be minimized because of the high incidence of brain injury associated with its use [18].

In summary, direct arch enlargement in which a patch is placed in the concavity of the arch that extends from the ascending to the descending aorta using cardiopulmonary bypass and moderate hypothermia without circulatory arrest is an attractive and safe approach for patients with an isolated arch obstruction. It allows for complete relief of the obstruction and for unhampered growth of the arch. Additionally, unlike extraanatomic bypass grafts, it entails the minimal use of foreign material and accomplishes a repair that is well protected deep within the mediastinal space.


   Acknowledgments
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 Acknowledgments
References
 
This study is supported in part by the Alec W. Sakas Memorial Foundation.


   Footnotes
Top
 Footnotes
Abstract
 Introduction
 Material and Methods
 Results
 Comment
 Acknowledgments
 References
 
Address reprint requests to Dr DeLeon, Department of Thoracic and Cardiovascular Surgery, Loyola University Medical Center, 2160 S First Ave, Maywood, IL 60153.


   References
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 Acknowledgments
 References
 

  1. Fishman NH, Bronstein MH, Berman W, et al. Surgical management of severe aortic coarctation in interrupted aortic arch in neonates. J Thorac Cardiovasc Surg 1976;71:35–47.[Abstract]
  2. Zannini L, Gargiulo G, Albanese SB, et al. Aortic coarctation with hypoplastic arch in neonates: a spectrum of anatomic lesions requiring different surgical options. Ann Thorac Surg 1993;56:288–94.[Abstract]
  3. Foster ED. Reoperation for aortic coarctation [collective review]. Ann Thorac Surg 1984;38:81–9.[Abstract]
  4. Ralph-Edwards AC, Williams WG, Coles JC, Rebeyka IM, Trusler GA, Freedom RM. Reoperation for recurrent aortic coarctation. Ann Thorac Surg 1995;60:1303–7.[Abstract/Free Full Text]
  5. Grinda JM, Mace L, Dervanian P, Folliguet TA, Neveux JY. Bypass graft for complex forms of ischemic aortic coarctation in adults. Ann Thorac Surg 1995;60:1299–302.[Abstract/Free Full Text]
  6. Jacob T, Cobanoglu A, Starr A. Late results of ascending aorta–descending aorta bypass grafts for recurrent coarctation of aorta. J Thorac Cardiovasc Surg 1988;95:782–7.[Abstract]
  7. Beekman RH, Rocchini AP, Behrendt DM, Rosenthal A. Reoperation for coarctation of the aorta. Am J Cardiol 1981;48:1108–14.[Medline]
  8. Moulaert AJ, Bruins CC, Oppenheimer-Dekker A. Anomalies of the aortic arch and ventricular septal defects. Circulation 1976;53:104–5.
  9. Morrow WR, Huhta JC, Murphy DJ, McNamara DG. Quantitative morphology of the aortic arch in neonatal coarctation. J Am Coll Cardiol 1986;8:616–20.[Abstract]
  10. Siewers RD, Ettedgui J, Pahl E, Tallman T, del Nido PJ. Coarctation and hypoplasia of the aortic arch: will the arch grow? Ann Thorac Surg 1991;52:608–13.[Abstract]
  11. Sade RM, Crawford FA, Hohn AR, Riopel DA, Taylor AB. Growth of the aorta after prosthetic patch aortoplasty for coarctation in infants. Ann Thorac Surg 1984;38:21–5.
  12. Vouhé PR, Trinquet F, Lecompte Y, et al. Aortic coarctation with hypoplastic arch. Results of extended end-to-end aortic arch anastomosis. J Thorac Cardiovasc Surg 1988;96:557–63.[Abstract]
  13. Elliott MJ. Coarctation of the aorta with arch hypoplasia: improvements on a new technique. Ann Thorac Surg 1987;44:321–3.[Abstract]
  14. Heineman M, Ziemer G, Luhmer J, Haverich A, Kallfelz HC, Borst HG. Coarctation of the aorta in complex congenital heart disease: simultaneous repair via sternotomy. Eur J Cardiothorac Surg 1990;4:482–6.[Abstract]
  15. DeLeon SY, Downey FX, Baumgartner NE, et al. Transsternal repair of coarctation and associated cardiac defects. Ann Thorac Surg 1994;58:179–84.[Abstract]
  16. Lacour-Gayet F, Bruniaux J, Serraf A, et al. Hypoplastic transverse arch and coarctation in neonates. Surgical reconstruction of the aortic arch: a study of sixty-six patients. J Thorac Cardiovasc Surg 1990;100:808–16.[Abstract]
  17. Barron DJ, Lamb RK, Ogilvie BC, Monro JL. Technique for extraanatomic bypass in complex aortic coarctation. Ann Thorac Surg 1996;61:241–4.[Abstract/Free Full Text]
  18. DeLeon S, Ilbawi M, Arcilla R, et al. Choreoathetosis after deep hypothermia without circulatory arrest. Ann Thorac Surg 1990;50:714–9.[Abstract]

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