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Ann Thorac Surg 1999;67:528-531
© 1999 The Society of Thoracic Surgeons

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Original Articles

Extended end-to-end repair and enlargement of the entire arch in complex coarctation¹

^a Departments of Pediatrics and Thoracic-Cardiovascular Surgery, Loyola University Medical Center, Stritch School of Medicine, Maywood, Illinois, USA

Accepted for publication July 21, 1998.

Address reprint requests to Dr Vitullo, Division of Pediatric Cardiology, Loyola University Medical Center, 2160 South First Ave, Maywood, IL 60153

	Abstract

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
Background. Treatment of hypoplasia of the entire arch in coarctation is a surgical challenge. The current approaches have technical difficulties, high recurrence rates, and increased morbidity and mortality.

Methods. Over a 14-month period, a combined extended end-to-end repair with patch enlargement of the concavity of the entire arch was performed in 6 neonates and 1 infant. Through a midsternotomy and using cardiopulmonary bypass and hypothermia, extended end-to-end repair was performed initially leaving the proximal anastomosis open. The enlarging polytetrafluoroethylene patch was then sutured starting at the incised descending aorta distal to the extended end-to-end repair and continued retrogradely through the transverse arch to the ascending aorta proximal to the aortic cannulation site. One neonate had a patent ductus arteriosus and another had ventricular septal defect closure. One neonate had arterial switch and 3 had Norwood-type procedures performed with the enlarging patch extended to the pulmonary artery anastomosis. The remaining infant had arch enlargement performed after an arterial switch procedure and extended end-to-end repair.

Results. All patients did well and showed no residual gradient up to 1 year follow-up. Two patients successfully had bidirectional Glenn shunt at 9 months of age, and one had closure of residual arterial septal defect at 8 months of age.

Conclusion. The combined extended end-to-end repair and arch enlargement procedure should minimize recurrence rates because of a tension-free enlargement of the entire aortic arch and elimination of the coarctation ridge and ductile tissues. Combined with the arterial switch and Norwood-type procedures, the approach results in a large neoaorta.

	Introduction

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
Treatment of coarctation of the aorta in the presence of significant hypoplasia of the entire transverse arch is difficult [1–3]. Repair of the coarctation and enlargement of the entire arch can be accomplished successfully by excision of the ductal tissues and patching the concavity of the aortic arch extending from the ascending to descending aorta under cardiopulmonary bypass and hypothermia with or without circulatory arrest [4]. Some patients, however, will have residual or recurrent narrowing at the area of the coarctation probably because of the presence of the coarctation ridge or remnant of ductal tissues.

Extended end-to-end repair effectively eliminates the coarctation ridge and most of the ductal tissues [1–3, 5, 6] and is probably the best approach in infants with coarctation with or without hypoplasia of the distal arch. However, in the presence of hypoplasia of the entire arch, it is difficult to perform (radically extended end-to-end repair) and carries a significant recoarctation rate [1, 3].

To eliminate the coarctation ridge and most ductal tissues and concurrently enlarge the entire aortic arch, we combined extended end-to-end repair and patching of the concavity of the entire arch using cardiopulmonary bypass and hypothermia with or without circulatory arrest.

	Material and methods

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
From April 1996 to June 1997, we performed enlargement of the entire arch in combination with extended end-to-end repair in 7 patients. The clinical profile, surgical techniques, and results were retrospectively analyzed.

Clinical profile
There were 6 neonates who were 6 to 17 days old (mean, 10 days) and 1 infant who was 5 months old at the time of surgical repair.

Associated lesions included patent ductus arteriosus in 1 patient, ventricular septal defect in 1, transposition of the great arteries in 2, and severe hypoplasia of the left ventricle in 3. One of the patients with transposition of the great arteries had a Taussig-Bing anomaly as well as tracheoesophageal fistula and imperforate anus.

Surgical procedures and techniques
All operations were performed through a midsternotomy using cardiopulmonary bypass and moderate or deep hypothermia with or without circulatory arrest. The distal arch, left subclavian artery, and descending aorta were temporarily occluded, and the extended end-to-end repair was first done in all patients by leaving the most proximal anastomosis open using an absorbable monofilament suture in a continuous interlocking fashion. The medical aspect of the descending aorta was incised distal to the end-to-end anastomosis (Fig 1 A and B). A thin-walled (0.4 mm) polytetrafluoroethylene (PTFE) patch was sutured starting at the descending aorta and carried retrogradely through the transverse arch to the ascending aorta using PTFE suture (Fig 1 C and D).

View larger version (24K): [in this window] [in a new window]   Fig 1. (A and B) Extended end-to-end repair and enlargement of the entire aortic arch. The isthmus has been transected; the concavity of the arch has been incised extending proximally to the ascending aorta. The dotted line indicates incision on the anterior part of the descending aorta. (C and D) Completed repair with the polytetrafluoroethylene patch in place. (E) Extension of the polytetrafluoroethylene patch to the proximal ascending aorta in the arterial switch procedure.

In 4 other neonates, one with Taussig-Bing transposition of the great arteries and 3 others with severe hypoplasia of the left ventricle, the PTFE patch was extended proximally to be part of the arterial switch in 1 and Norwood anastomosis in the other 3 (Fig 1E). Two of the 3 patients with hypoplasia of the left ventricle had patent but severely stenotic aortic valves. All 4 patients had deep hypothermia (18° to 20°C) and circulatory arrest ranging from 25 to 50 minutes.

The remaining patient, a 5-month-old infant, had transposition of the great arteries with intact ventricular septum and had undergone arterial switch procedure at 4 days of age. Although coarctation of the aorta was suspected preoperatively, closure of the patent ductus arteriosus at initial operation showed increased umbilical artery pressure from 50 to 70 mm Hg. One week postoperatively, significant coarctation gradient developed and the patient had extended-to-end repair through a left thoracotomy. Postoperatively, persistent gradient was present. At 5 months of age, enlargement of the entire arch was accomplished through a midsternotomy using cardiopulmonary bypass and moderate hypothermia (25°C) without circulatory arrest. The PTFE patch extended from the ascending aorta to the descending aorta distal to the end-to-end anastomosis. A pulmonary branch stenosis was also repaired.

	Results

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
All patients recovered and have been followed up for at least 9 months. None had significant postoperative gradient clinically and echocardiographically (Fig 2). Three patients had postoperative angiograms that showed widely patent repair (Fig 3).

View larger version (52K): [in this window] [in a new window]   Fig 2. (A) Echocardiogram of neonate with patent ductus arteriosus coarctation and hypoplasia of the entire arch. (B) Postoperative echocardiogram showing the enlarged aortic arch.

View larger version (144K): [in this window] [in a new window]   Fig 3. Angiogram of a patient who had the arterial switch procedure, coarctation repair, and enlargement of the ascending aorta and entire arch.

	Comment

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
The incidence of arch hypoplasia is high in both simple and complex coarctation [1, 3, 7]. Morrow and colleagues [7] have shown that some hypoplasia of the transverse arch is present in all infants with isolated coarctation. According to Moulaert and colleagues [8], significant hypoplasia is present if the diameter of the proximal arch (segment between the innominate and left carotid arteries) and distal arch (segment between the left carotid and subclavian arteries) is less than 60% and 50%, respectively, of the diameter of the ascending aorta.

To improve outcome, significant arch hypoplasia should be repaired at the time of coarctation repair [3]. The mortality rate is higher when there is residual arch obstruction especially in the presence of increased pulmonary blood flow or pulmonary artery banding [2, 3, 9].

In simple coarctation with significant hypoplasia of the distal transverse arch, extended end-to-end repair has been used successfully in most cases [1–3, 5]. In the presence of less significant hypoplasia of the aortic arch, repair of coarctation alone has been accomplished successfully with reasonable expectation of growth of the aortic arch [10, 11]. Pathologically, the aortic arch that has less growth potential has decreased amounts of elastin lamella and alpha-actin–positive cells [12]. These findings are more common in older infants. Although the implication of these findings is not clear in the treatment of hypoplastic arches, it might explain the appearance of significant arch obstruction in older children and young adults after successful coarctation repair. The arch obstruction usually appears after a growth spurt [13].

Although hypoplasia of the entire aortic arch can occur in simple coarctation, it is more commonly associated with complex coarctation [1–3]. The radically extended end-to-end repair with the anastomosis extending proximal to the left carotid artery has been applied in patients with hypoplasia of the entire arch [2, 3, 6]. The procedure is usually done through a left thoracotomy. It entails extensive dissection and mobilization of the ascending aorta, entire arch and its branches, and descending aorta. Although the procedure can be performed in most patients, there could be difficulties in applying the proximal aortic clamp that occludes the left carotid and subclavian arteries without causing bradycardia and hypotension [1, 5, 6]. There is also a significant recurrence rate probably because of tension in the anastomosis and limited extension of the repair proximal to the left carotid artery and to the ascending aorta [1, 3]. The procedure is particularly difficult to perform, and the recurrence rate is extremely high in the presence of complex aortic arches, such as BOVINE innominate trunk and extremely long distal arches [1].

Extended anastomosis of the descending aorta to the undersurface of the aortic arch has also been used in patients with hypoplasia of the proximal or entire arch [14]. The technique of mobilization and application of the aortic cross clamp is similar to the radically extended end-to-end repair. The size of anastomosis, however, is smaller because the descending aorta is simply anastomosed end to side of the aortic arch. The distal arch and isthmus are not used. The problem associated with this approach is similar to that of the radically extended end-to-end repair. There is also an associated neurologic complication [2, 14] probably from occlusion of the left carotid artery during the repair, resulting in brain ischemia in patients with inadequate collateral flow through the circle of Willis.

Our approach of combining extended end-to-end repair of the coarctation with patch enlargement of the concavity of the entire aortic arch is beneficial in hypoplasia of the entire aortic arch and complex aortic arches because they are usually associated with intracardiac defects that can be repaired simultaneously. The coarctation ridge and ductal tissues are completely excised and the arch enlargement repair is tension free. Combined with the arterial switch and Norwood procedures, this novel approach to coarctation repair also allows creation of a large neoaorta anastomosis to the proximal main pulmonary artery.

Simultaneous repair of coarctation and associated cardiac defects is being done increasingly. Accessibility of the entire aortic arch and proximal descending aorta through a midsternotomy and better understanding of the use of cardiopulmonary bypass and hypothermia have made one-stage repair feasible and safe [1, 4, 15, 16]. Enlargement of the entire arch with repair of the coarctation and other associated defects will minimize residual aortic obstruction and the need for reoperations.

In summary, the combination of extended end-to-end repair of coarctation and patching of the concavity of the aortic arch allows complete excision of the coarctation ridge and ductal tissues and excellent enlargement of the entire arch. Simultaneous repair of the associated cardiac defects will minimize reoperations. Because the arch-enlarging patch extends proximal to the aortic cannulation site, aortic constriction from the purse string suture in some patients with a small ascending aorta is minimized. In combination with the arterial switch and Norwood procedures, this approach results in a large neoaorta.

	Footnotes

Top Footnotes Abstract Introduction Material and methods Results Comment References

 
¹ This article has been selected for the open discussion forum on the STS Website: http://www.sts.org/section/atsdiscussion/

	References

Top Footnotes Abstract Introduction Material and methods Results Comment References

 

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