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Ann Thorac Surg 2005;79:1950-1956
© 2005 The Society of Thoracic Surgeons
Department of Cardiothoracic Surgery, Rush University Medical Center, Chicago, Illinois
Accepted for publication January 3, 2005.
* Address reprint requests to Dr Carr, Department of Cardiothoracic Surgery, E-500, University of Chicago Medical Center, 5841 South Maryland Ave, Chicago, IL60637 (E-mail: heartandbones{at}yahoo.com).
Presented at the Fifty-first Annual Meeting of the Southern Thoracic Surgical Association, Cancún, Mexico, Nov 2–4, 2004.
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Abstract |
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 Top Abstract IntroductionPatients and MethodsResultsCommentDiscussionReferences |
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METHODS: We retrospectively reviewed operations in 45 patients treated between 1978 and 2003.
RESULTS: From 1978 to 2001, there were 45 adolescents or adults between the ages of 11 and 53 years (mean 21, SD ± 10) who underwent surgical correction. The perioperative mortality rate was 0% and the morbidity rate was 18%. All patients had improved blood pressure before discharge after a mean of 7 days, which ranged from 160/90 mm Hg to 90/50 mm Hg (mean 128/73 mm Hg, SD ± 17/12 mm Hg). This was an average improvement of 35 mm Hg (SD ± 26) compared with the preoperative pressure (p < 0.0005). Long-term results (defined as 5 years or more) were documented for 30 (71%) with a mean follow-up of 18.2 years (range, 67 to 293 months; SD ± 70 months). At the time of last follow-up, the blood pressure was documented and averaged 122/73 mm Hg (SD ± 11/10 mm Hg), which was a decrease of 36 mm Hg (SD ± 29) compared with the preoperative pressure (p < 0.0005). Seventy-six percent of patients were on no medications for hypertension. None of the 30 patients available for long-term follow-up has required a second operation for recurrence.
CONCLUSIONS: Surgical repair of coarctation in the adolescent and adult is safe and durable, with a high success rate in curing patients of hypertension and making them medication-free for life. The recurrence rate is low, and most patients will not require any further intervention.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsCommentDiscussionReferences |
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The issue has been further confounded by an increasing number of articles proposing endovascular interventions as superior to a surgical approach [4–6]. However, most of these have only short-term to intermediate follow-up, and the long-term results of angioplasty and stenting for adults with coarctation are unknown [7–9].
We sought to determine the long-term outcome in this population after surgical intervention by reviewing the database at our institution. Furthermore, we wanted to define any potential limitations of the open surgical approach for which endovascular interventions may play a role in the future.
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Patients and Methods |
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We were able to document the blood pressure before and after surgery, as well as after a mean follow-up of 18 years. We also recorded changes in the number of antihypertensive medications required before and after surgery. Attention was also given to the preoperative and postoperative gradients across the coarctation site and its relevance to long-term outcome during follow-up.
Statistical analysis was performed by comparing individual variables before and after surgery using a two-tailed paired t test. In this way, each patient served as his or her own control. Data are presented as the mean ± standard deviation. Statistical significance was assumed for a p value of less than 0.05.
Presentation
From 1978 to 2001, there were 201 patients who underwent open surgical coarctectomy at our medical center for coarctation of the aorta. Although most of these were infants, 45 were adolescents or adults between the ages of 11 and 53 years (mean 21, SD ± 10), and they make up the population of this study.
Thirty-five patients (35 of 45, 78%) presented with hypertension and decreased femoral pulses, 3 (7%) with claudication, 2 (4%) with a heart murmur, 2 (4%) with chest pain, and 1 patient each with an abnormal chest radiograph, decreased femoral pulses alone, and right hemiparesis from subclavian steal. The presenting blood pressure in the right arm ranged from 250/110 mm Hg to 108/80 mm Hg (mean 155/84 mm Hg, SD ± 26/18 mm Hg) and tended to be higher than the left, which ranged from 220/90 mm Hg to 80/50 mm Hg (mean 148/86 mm Hg, SD ± 25/15 mm Hg). One patient was already taking three antihypertensive medications, 7 patients (16%) were on two medications, 12 (27%) were on one, and 25 (55%) were not yet taking any medications for blood pressure.
The method of diagnosis was by angiography in 37 patients (37 of 45, 82%), by echocardiography in 7 (17%), and by magnetic resonance imaging (MRI) in 1 patient. Overall, an echocardiograph was obtained in 67% of patients and angiography in 89%. Five patients were evaluated by echocardiography alone. The peak gradients across the coarctation as measured by echocardiography ranged from 21 to 92 mm Hg (mean 51 ± 20 mm Hg), while angiography-measured gradients ranged from 29 to 100 mm Hg (mean 46 ± 18 mm Hg). These tests determined that 56% of the patients had a bicuspid aortic valve.
Before presentation at our institution, 5 patients had undergone previous repairs of the coarctation earlier in life somewhere else, and 1 patient had two prior surgeries. Two patients had Dacron (C.R. Bard, Haverhill, PA) interposition grafts (10- and 18-mm grafts) used for the repair at 5 and 18 years old, respectively. Both presented with recurrences at 12 and 10 years later, respectively, with intimal hyperplasia and organized thrombus at the anastomotic sites. One patient each had subclavian flap repair, end-to-end anastomosis, and Dacron patch repair at the ages of 17 days, 15 days, and 6 years, and recurred after 11 years, 9 years, and 13 years, respectively. The subclavian flap stenosed along the suture line owing to significant intimal hyperplasia, as did the end-to-end anastomosis. The Dacron-patched area in the third patient was patent, but a tight narrowing was found just proximal to the patch. The final patient had undergone an end-to-end repair at 15 months of age and had a recurrence 2.5 years later. He then had a Dacron patch repair, which lasted for 17 years and then the area stenosed again just proximal to the patch at the origin of the left subclavian artery.
Operative Technique
In the present series, 18 patients (18 of 45, 40%) had either Dacron or Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) patch enlargements, 10 (22%) had resection with an end-to-end anastomosis, 9 (20%) had interposition Dacron grafts (from 14 to 22 mm), and 8 (18%) had subclavian flap repairs. Polypropylene suture (Prolene; Ethicon, Somerville, NJ) was used in 37 patients (37 of 45, 82%), expanded polytetrafluoroethylene (Gore-Tex) suture in 3, polyglyconate (Maxon; Davis & Geck, St. Louis, MO) suture in 1, and the type of suture was not mentioned in 4. Silk suture was not used. All of the collateral vessels around the coarctation were saved in 40 patients (89%).
Eleven patients (24%) had intraoperative pressure measurements made above and below the completed repair site using a needle transducer. At the end of the case, 4 patients had no gradient at all. The other 7 had gradients of 5, 8, 10, 10, 11, 13, and 20 mm Hg. The patient with a 20 mm Hg gradient had follow-up echocardiography 10 years later showing a widely patent repair site without any further increase in the gradient, and he remains asymptomatic.
The length of surgery ranged from 2.5 to 5.5 hours (mean 3.8 ± 0.7) and the cross-clamp time varied from 15 to 68 minutes (mean 31 ± 15). Five patients were repaired utilizing cardiopulmonary bypass, 3 femoral-femoral bypass, and 2 left atrial-femoral bypass. One patient very early in the series was repaired with the aid of a Gott shunt. The rest were repaired with a "clamp-and-sew" technique. Currently, we approach these patients with cardiopulmonary bypass on stand-by. If clamping of the aorta causes a drop in the femoral (distal) mean blood pressure to less than 50 mm Hg, then bypass is used. If the distal mean blood pressure remains above 50 mm Hg after clamping, then the patient can be safely repaired without bypass.
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Results |
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Hospital Course
In the earlier years, patients would be admitted 2 to 3 days before surgery to have angiography performed and the preoperative workup completed. This practice increased the overall length of hospitalization to a mean of 9 days (SD ± 3; range, 3 to 17). The time from operation to discharge varied from 3 to 12 days, with a mean of 7 days (SD ± 2).
All patients had reactive systemic hypertension immediately after repair, which was controlled with intravenous nitroprusside, nitroglycerin, or ß-blockers after surgery. However, all patients had dramatically improved blood pressure before discharge after a mean of 7 days, which ranged from 160/90 mm Hg to 90/50 mm Hg (mean 128/73 mm Hg, SD ± 17/12 mm Hg). This was an average improvement of 35 mm Hg (SD ± 26 mm Hg) compared with the preoperative pressure (p < 0.0005).
Long-Term Follow-Up
Three patients, 2 from Mexico and 1 from Nigeria, recovered and went back to their native countries and were not available for follow-up. Of the remaining 42 patients, long-term results (defined as 5 years or more) were documented for 30 (30 of 42, 71%) with a mean follow-up of 18.2 years (range, 67 to 293 months, SD ± 70). At the time of last follow-up, the most recent blood pressure was documented and averaged 122/73 mm Hg (SD ± 11/10 mm Hg), which was a decrease of 36 mm Hg (SD ± 29 mm Hg) compared with the preoperative pressure (p < 0.0005). The patient’s blood pressure at discharge was predictive of the long-term follow-up pressure in that there was not a statistically significant further decrease from the time of discharge until years later (p < 0.15). Of these patients, 76% (23 of 30) were on no medications for hypertension, and 20% (6 of 30) were on one medication. One patient was taking two medications for blood pressure. This was an overall decrease in the number of antihypertensives by an average of one medication per patient (SD ± 0.7).
Long-term follow-up imaging studies were obtained in 12 of the 30 patients (40%), and the type of study was echocardiography in 7 (7 of 12, 58%), a computed tomography scan in 3 (3 of 12, 25%), angiography in 3 (3 of 12, 25%), and an MRI in 1 patient. Two patients had echocardiography and angiography performed. These were all long-term studies done after a mean time of 18.3 years (range, 9.7 to 23 years, SD ± 55 months) after surgery. One patient had a slight recurrence (recurrence defined as gradient > 20 mm Hg) detected by echocardiography 18 years after surgery, but no intervention was performed as the patient has normal blood pressure and is asymptomatic. The remaining patients showed no evidence of stenosis and appeared to have a normal caliber to the aorta without recurrence and appropriate Doppler flow (Table 1).
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The long-term mortality is 6% (2 of 30). The oldest patient in the series (age 53 years) died suddenly while at work 6 years after her operation. The etiology was unknown, and no autopsy was performed. The second patient died 13 years after his operation at the age of 28 while going down a waterslide at an amusement park.
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Comment |
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TopAbstractIntroductionPatients and MethodsResultsCommentDiscussionReferences |
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Seventy-six percent of the patients became normotensive without medication shortly after surgery, and this benefit persisted throughout the duration of follow-up over 18 years. This finding is consistent with other reports documenting a durable cure rate (normal blood pressure without medication) of 66% to 69% over 5 to 14 years [10–12]. A statistically significant difference was seen when comparing preoperative blood pressure with the pressure at the time of discharge (mean drop of 35 mm Hg) and at the time of most recent follow-up (mean drop of 36 mm Hg). The level of improvement at the time of discharge (mean of 7 days) was predictive of their future blood pressure because, although there was a significant change immediately after surgery, there was not a significant further decrease from discharge to late follow-up (Fig 1). This result is consistent with the results of another large, recently published study [10].
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The postoperative 60-day mortality rate was zero and the morbidity rate was low. A common misconception concerning adult coarctation repair is that there is a high risk of paraplegia from cross-clamping the aorta. Despite the obvious presence of numerous collaterals supplying the lower body and spinal cord, as well as an often-noted lack of change in blood pressure during clamping, this myth seems to persist, even in the recent literature [14]. As noted in our results, none of the patients in this series, nor in any recent series, have suffered from postoperative paraparesis or paraplegia [10, 15], despite occasionally sacrificing some of the collateral vessels in close proximity to the repair site. The anticipated risk of paraplegia may emanate from an often-cited article by Brewer and colleagues [16] in 1972, despite their reporting a low incidence of paraplegia of 0.41% (66 cases from 12,532 operations). There is actually a higher risk of paraplegia in infants and younger children who have not yet formed mature collaterals [17]. Furthermore, endovascular interventions are not immune to this complication, and paraplegia has been reported after balloon angioplasty of a coarctation [18].
We compared the morbidity, mortality, restenosis, and reintervention rates of the current series and another recent surgical series with five recent reports of endovascular interventions in adults with coarctation (Table 2) [19–23]. The morbidity rates tended to be higher in the angioplasty patients, ranging from 9% to 24%. The more common complications included femoral and iliac dissection, stent migration, traumatic aneurysm formation, and aortic dissection [19–23]. While there were no deaths among the surgical patients, 3 patients died after angioplasty. There are several reports in the literature of angioplasty and stenting of native and recurrent coarctation leading to aortic rupture, dissection, and death [24–26]. Since balloon angioplasty relieves coarctation by tearing the aortic intima and media, it is not surprising that the rate of traumatic aneurysm formation at the site of dilation ranges from 0% to 40% [20, 27–29].
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The ultimate success of any intervention for coarctation will have to address its effects in relieving hypertension for the duration of the patient’s life. Our series found that 76% of patients had normal blood pressure and were taking no medication during follow-up. This finding is similar to results from another large surgical series [10]. The results from endovascular interventions are diverse, with the occurrence of normotension without any medications occurring in 18% to 74%; however, this is often not reported [19–23]. One study that did have long-term follow-up on 28 patients who underwent angioplasty reported normal blood pressure without any medications in only 18%, with a restenosis rate of 40% over 6.6 years [22]. The eventual decision as to whether endovascular interventions are equivalent to surgery will have to address this underreported outcome measure.
After surgical intervention, historic guidelines would suggest that the patients should be followed up for aneurysmal development around the repair site. No aneurysm eventually developed in any of the patients in this series, regardless of the technique of repair. Previous reports had documented an incidence of later aneurysm formation from 5% to 46% occurring 6 to 18 years after repair [30, 31]. The cited risk factors for this complication include use of the patch graft technique and advanced age at the time of repair [32]. Neither of these risk factors appeared to predispose to aneurysm formation in this study. It is now well accepted that the previous use of silk suture for aortic anastomoses is a major risk factor for the formation of false aneurysms, occurring 2 to 27 years after the initial operation [33–35]. Most of the literature on false aneurysm development after coarctation repair is quite old, when silk suture was used more commonly. All of the suture material used in the current review was monofilament, and polypropylene was used in 82%. We believe that this greatly contributed to the lack of false aneurysms in our series. Polypropylene has been shown to be quite safe [36]. It is our suture of choice for aortic reconstruction.
In summary, surgical repair of coarctation in the adolescent and young adult is safe and durable, with a high success rate in curing patients of hypertension and making them medication-free for life. The recurrence rate is very low, and most patients will not require any further intervention. Surgical repair remains the "gold standard" by which other interventions will need to document such a high degree of success before supplanting surgery as the therapy of choice.
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Discussion |
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TopAbstractIntroductionPatients and MethodsResultsCommentDiscussionReferences |
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DR CARR: Thank you for the question, Dr Mavroudis. Most of the patients who had the clamp-and-sew technique were earlier in the experience, in the early and mid 1980s. We currently repair those older patients using cardiopulmonary bypass, usually left heart partial bypass or fem-fem bypass, and we also shoot for a distal perfusion pressure around 50 to 60 mm Hg, and we monitor that through a femoral arterial line.
DR MAVROUDIS: So you do all of them with femoral bypass, you say?
DR CARR: Yes, we do in the older patients.
DR MAVROUDIS: So in every patient over 18 years of age you will perform the operation under bypass or partial bypass. Is that accurate?
DR CARR: Well, that is usually our intention, yes.
DR MAVROUDIS: That is not the general course of therapy in most centers. Perfusion techniques for coarctation are generally used selectively. What made you apply this therapy to every patient? Why are you heading in that direction?
DR CARR: Well, there is such a high fear factor for patients having paraplegia. Now, obviously when we clamp the aorta, oftentimes there is not a dramatic change in blood pressure, and we can monitor the femoral arterial pressure and see when we put the clamp on if there is a dramatic change. If there is, we would want to proceed to using some type of bypass. But we go into the operating room with that in mind. I don’t think we go into the operating room thinking we are just going to clamp and sew.
DR MAVROUDIS: So you are selective then?
DR CARR: Yes, we are selective.
DR MAVROUDIS: So what the audience would get from your presentation is that you are selective in which ones you will use partial cardiopulmonary bypass?
DR CARR: That is correct. I am sorry for the confusion.
DR PIOTR BEDNARSKI (Nuremberg, Germany): Very nice presentation. I have two questions regarding this patient group. Did I understand correctly that about 40% of all patients operated on for coarctation had Dacron patch plasty and about half of all patients had late follow-up with CT scan? If so, how many patients with Dacron patch plasty were actually followed-up? I ask you this question because in the early ’90s I took part in a follow-up at the Hannover Medical School of 140 adolescent and adult survivors at 1.5 to 17.2, mean 9.1 years postoperatively after repair of coarctation. In 27 patients (35.1%) of the patch plasty group significant dilatation at the operative site was observed and reoperation for aneurysm formation was required in 15 patients (Aebert H, Laas J, Bednarski P et al. High incidence of aneurysm formation following patch plasty repair of coarctation. Eur J Cardiothorac Surg 1993;7:200–4). There are also similar quotas in the literature. Did you observe this problem among your patients? Could you comment please on this issue.
DR CARR: I think that commonly there has been a trend in the literature toward thinking that using a Dacron patch angioplasty leads to late aneurysm formation. Some of this is confounded by the fact of whether or not the intimal ridge or the intimal shelf has been excised at the time the patch was placed. Some people feel that this leads to weakening of the wall of the aorta where that intimal ridge had been excised, and that is why it is always the area opposite the patch that forms the aneurysm. So if that segment has not been excised, people think that Dacron patch angioplasty does not lead to aneurysm formation, and we found that to be true in our series. Thank you for that question.
DR ERLE H. AUSTIN (Louisville, KY): You mentioned that 2 patients died suddenly. The first one didn’t have an autopsy and the second one died suddenly during a water slide. Did that second patient have an autopsy? Can you be sure that those patients had not developed aneurysms at the coarctation repair site before their demise?
DR CARR: The patient who died while she was at work did not have an autopsy, so we really don’t know whether or not there was an aneurysm in that patient or what her status was. The patient who died traumatically, no autopsy was performed on him either. The family did not want an autopsy performed because he had been through so much. So we honestly don’t know the status there either.
DR AMATO: As the attending surgeon on a majority of the cases, I rise to assist Dr Carr on the question of having no aneurysms in our series of cases. There are, I believe, two reasons that aneurysms did not occur in our series. The first is that we do not carve out or in any way remove the posterior shelf. We believe that at the aneurysm at the area of coarctation does not occur within the patch, but rather in the posterior wall exactly where that posterior shelf has been removed. Secondly, we have gone away from prior use of the rigid grafts and presently use a Gore-Tex patch. We found no aneurysms in our series at all.
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References |
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This article has been cited by other articles:
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  S. Tabbutt, S. C. Nicolson, T. E. Dominguez, W. Wells, C. L. Backer, J. S. Tweddell, P. Bokesch, and M. Schreiner Perioperative course in 118 infants and children undergoing coarctation repair via a thoracotomy: A prospective, multicenter experience. J. Thorac. Cardiovasc. Surg., November 1, 2008; 136(5): 1229 - 1236. [Abstract] [Full Text] [PDF]
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 J. A. Carr The Results of Catheter-Based Therapy Compared With Surgical Repair of Adult Aortic Coarctation J. Am. Coll. Cardiol., March 21, 2006; 47(6): 1101 - 1107. [Abstract] [Full Text] [PDF]
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