Ann Thorac Surg 1999;67:1386-1390
© 1999 The Society of Thoracic Surgeons
Original Articles
Operation for mid-arch coarctation
Takahiro Katsumata, MD, PhDa,
Stephen Westaby, FRCSa
a Department of Cardiac Surgery, Oxford Heart Centre, John Radcliffe Hospital, Oxford, England, UK
Accepted for publication December 7, 1998.
Address reprint requests to Dr Katsumata, Oxford Heart Centre, John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, England
e-mail: katsu{at}AHF.org.uk
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Abstract
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Background. Coarctation occurring within the aortic arch is rare and may present difficulties during surgical repair. We describe the operative technique and outcome in 6 patients with this unusual anomaly.
Methods. Five patients had antegrade perfusion with circulatory arrest. Three patients with presubclavian narrowing (one presenting with type B dissection) were operated through extended left thoracotomy. Two precarotid and paracarotid lesions were approached through a median sternotomy. All patients were perfused antegradely from the ascending aorta and operated with hypothermic circulatory arrest. One patient who had a complex presubclavian coarctation after two previous repairs received an ascending aorta to abdominal aorta bypass graft without cardiopulmonary bypass.
Results. All patients survived operation and are well at a mean follow-up of 3.3 years after the procedure. None had cerebral problems or spinal cord injury. Renal function was unchanged. The mean (± standard error of the mean) resting gradient across the coarctation decreased from 42 ± 4.0 mm Hg to 6 ± 1.2 mm Hg (p = 0.0004).
Conclusions. Hypothermic circulatory arrest using antegrade ascending aortic perfusion allows safe and effective repair of mid-arch coarctation. Complicated reoperations can be managed safely using ascending-to-abdominal aortic bypass.
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Introduction
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Coarctation of the aorta usually occurs distal to the left subclavian artery and is recognized and corrected in infancy. Coarctation occurring within the aortic arch is rare and presents difficulties in the conduct of surgical repair. This article describes the surgical treatment and outcome in 6 patients who underwent operation in a 10-year period.
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Patients and methods
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Patient demographics and differing modes of presentation are presented in Table 1. All patients were hypertensive with discernible differences in pulse pressure between right and left arm.
Patients 2 and 3 were asymptomatic but with hypertension difficult to control adequately with medical treatment. They were considered at risk for heart failure and acute type A dissection (Fig 1). Patient 3 had undergone left thoracotomy and attempted coarctation repair in childhood, then again early in adult life, using cross-clamping without perfusion. Both operations failed to correct the narrowing and left Dacron and calcified tissues at the surgical site.
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Fig 1. Aortogram of patient 2 showing parasubclavian lesion with poststenotic dilatation of the distal aorta.
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The four symptomatic patients had different problems. Patient 1 presented with repeated syncope during games at school. Pulse differences led to diagnosis by aortogram (Fig 2). Syncope was caused by the ostial stenosis of the left carotid artery. Patient 4 presented with shortness of breath because of chronic hypertension and left ventricular failure. Patient 5 had episodes of dizziness and a hoarse voice due to recurrent laryngeal nerve palsy. The postcoarctation arch was aneurysmal (Fig 3). Patient 6 presented with acute type B dissection and a progressively enlarging descending aortic aneurysm. Preoperative investigations, including an aortogram and nuclear magnetic resonance imaging, failed to demonstrate the coarctation, which was identified at operation. Curiously the dissection originated within the poststenotic dilatation distal to the coarctation.
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Fig 2. Preoperative aortogram of patient 1 demonstrates the presence of presubclavian coarctation and also narrowing at the base of the left common carotid artery.
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Fig 3. Aortogram of patient 5 showing excentric narrowing between the innominate and left common carotid arteries, and also an aneurysm of the distal aorta.
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Surgical techniques
The varied aortic pathology and evolving surgical experience led to different operative methods (Fig 4).
Five patients had antegrade perfusion with circulatory arrest. Patient 1 required replacement of the hypoplastic distal aortic arch beyond the enlarged innominate artery. This procedure was undertaken with a median sternotomy, using ascending aortic cannulation and hypothermic circulatory arrest at 20°C. The distal arch was resected and the enlarged roots of the subclavian and carotid arteries reimplanted directly into the Dacron graft. Patient 5 also required distal arch replacement to excise the coarctation and replace the aneurysmal segment. The operative procedure was similar in this patient, except the left common carotid and left subclavian arteries were reimplanted together on a "Carrel" patch of aorta. Patients 2 and 4 underwent arch repair through an extended left thoracotomy, whereas these patients would previously have been approached through a median sternotomy. The ascending aorta was cannulated for arterial return and a right angled venous cannula inserted through the main pulmonary artery into the right ventricle (Fig 5). The cannulas were conveniently situated anteriorly away from the main operative field, and access to the aortic arch and descending thoracic aorta is much better than with the sternotomy approach. In particular, the phrenic and recurrent laryngeal nerves were easily identified and preserved. Systemic temperature was then reduced to 22°C for a short period of total circulatory arrest. With a light pediatric titanium vascular clamp occluding the innominate and carotid arteries, the narrowed arch was opened longitudinally and reconstructed at the area of narrowing with an elliptical patch of aortic homograft and Dacron, respectively. After thorough deairing, perfusion and full rewarming were recommenced. A similar incision and perfusion technique were used in patient 6 for descending thoracic aortic replacement of a 15-cm chronic type B dissection. During the thoracotomy incision coarctation-collateral vessels were encountered and when the true lumen of the chronic type B dissection was opened, the mid-arch coarctation was recognized with the left subclavian artery originating from the poststenotic dilation. This part of the arch was resected and replaced with a Dacron graft. The subclavian artery was directly reimplanted and the Dacron graft clamped distal to the arch repair. Cerebral and coronary perfusion were then resumed with rewarming, whereas the entire descending thoracic aorta was replaced with preservation of the intercostal arteries between T7 and T12.
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Fig 5. The central cannulation technique through an extended left thoracotomy that allows antegrade systemic perfusion.
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Patient 3 was severely hypertensive and anxious about the risk of paraplegia during a third direct approach. Therefore, we decided to perform an anterior extraanatomic bypass between the ascending thoracic aorta and the abdominal aorta. This operation was performed by a sternolaparotomy using side-biting clamps without cardiopulmonary bypass.
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Results
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There were no hospital deaths and no cerebral problems or spinal cord injury. Renal function did not change from the preoperative level.
At the time of discharge the mean (± standard error of the mean) rest gradient across the coarctation site (taking the higher value of the blood pressure measurements at the arms or Doppler-estimated corrected gradients), decreased significantly from 42 ± 4.0 mm Hg to 6 ± 1.2 mm Hg (p = 0.0004, Student’s paired t test). Differential (right upper body) hypertension resolved in all patients.
All 6 patients are alive and well up to 6 years after the procedure (average, 3.3 years). The mean (± standard error of the mean) rest gradient between both arms in all patients is 5.8 ± 1.1 mm Hg.
Two patients (5 and 6) with residual postoperative hypertension are receiving antihypertensive drugs. The remaining patients require no medication. Patient 3 has had a successful pregnancy.
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Comment
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Coarctation proximal to the left subclavian artery ("mid"-arch coarctation) is rare (about 1% of all coarctation cases) [1, 2]. Only infrequently is the coarctation detected before adult life when upper body hypertension is usually present. Although one of our patients (No. 6) had extensive collateral circulation in the chest wall, a well-developed collateral circulation is generally not present [2].
The mode of presentation is variable depending on the site of the narrowed aortic segment and involvement of the brachiocephalic vessels. All 6 patients were hypertensive, but only one had a cerebral event. Although the association of coarctation with acute type A dissection is well recognized [3], presentation with type B dissection is rare. In patient 6 the physical signs of coarctation were recognized before operation but attributed to the effects of type B dissection. Unusually, the dissection originated within the poststenotic dilatation in the distal aortic arch rather than in the proximal aorta with greater pulse pressure.
The surgical approach was of particular importance for a successful outcome in these patients. Attempts to correct presubclavian coarctation with direct aortic cross-clamping with or without left atriofemoral bypass are destined to fail through inadequate access to the proximal arch. Operations on the aortic arch (either through sternotomy or left thoracotomy) are usually performed with femoral arterial perfusion and hypothermic circulatory arrest. However, in the presence of a fixed obstruction proximal to the head vessels the risks of carotid malperfusion and global cerebral ischemia are significant. For this reason we elected to cannulate the ascending aorta in all patients for whom perfusion was used. We now preferentially use antegrade aortic perfusion through the ascending aorta and venous return from the pulmonary artery for all distal arch and descending aortic operations [4, 5]. This prevents retrograde embolism in patients with atheromatous aneurysms or malperfusion in chronic type B dissection. Reperfusion of the brain and coronary arteries can be restored early while the distal reconstruction is undertaken. The use of this technique prevented a cerebral disaster in patient 6 where mid-arch coarctation had not been diagnosed before repair of acute type B dissection.
The type of repair depends not only on the extension of the narrowed segment but also on the extent of associated pathologic change of the aortic tissue. Patients 1 and 5 presented with the fragile and dysplastic aortic tissue adjacent to the coarctation, needed graft interposition and reimplantation of aortic branches. In contrast, patients 2 and 4 were found with a well-preserved elastic aortic wall that allowed patch repair with satisfactory relief of the constricted side branches. Because the possibility of late aneurysm formation is a major concern after patch aortoplasty, these 2 patients have been followed up by periodic computed tomographic scans.
Although it is unusual to directly reimplant a carotid or subclavian artery end to side onto Dacron, we have carefully followed patient 1 with yearly nuclear magnetic resonance scans. Despite growth in his late teenage years there has been no narrowing at the origin of these vessels and flow through the arch is normal.
The difficulties and risks of a direct approach on recurrent coarctation are well recognized [6]. For this problem an extraanatomic bypass with a graft from the ascending to abdominal aorta proved simple (without perfusion) and effective [7–9]. Our patient is now without medication for hypertension and underwent a successful pregnancy without adverse events.
In conclusion, individually tailored surgical methods allow safe and effective repair of mid-arch coarctation. We consider antegrade aortic perfusion and hypothermic circulatory arrest to be more likely to succeed than unprotected cross-clamping between right and left carotid arteries.
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References
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Abstract
Introduction
