Innovations: Technology & Techniques in Cardiothoracic & Vascular Surgery:
Simultaneous Presentation of Coarctation of the Thoracic Aorta and Aneurysm of the Superior Mesenteric Artery
Karmy-Jones, Riyad MD; Bloch, Robert MD; Martin, M Alan MD
From the Heart and Vascular Institute, Southwest Washington Medical Center, Vancouver, WA, USA.
Accepted for publication February 23, 2009.
Address correspondence and reprint requests to Dr. Riyad Karmy-Jones, Southwest Washington Medical Center, Suite 300, Physician’s Pavilion, 400 NE Mother Joseph Place, Vancouver, WA 98664 USA. E-mail: firstname.lastname@example.org.
A 34-year-old woman presented with both coarctation of the thoracic aorta and aneurysm of the superior mesenteric artery. The former was managed by open surgical repair, the latter by stent-graft. This case illustrates the need for facility with both percutaneous and open approaches to diseases of the aorta and its branches.
Coarctation of the thoracic aorta can present in adult life, and untreated is associated with a reduction in life expectancy primarily due to complications of unremitting hypertension.1 Superior mesenteric aneurysm is rare, but following the principles laid out by other visceral aneurysms, symptomatic or large (>2 cm) lesions should be treated electively because of the risk of rupture.2 There have been proponents of both endovascular and open repair, for both these lesions, but ultimately anatomic considerations and risks of proposed procedure can be used to determine the optimal approach in individual cases.
A 34-year-old woman presented with persistent midepigastric pain following oopherectomy for a benign adnexial mass. A computed tomographic angiogram (CTA) was performed that revealed an aneurysm of the superior mesenteric artery (Fig. 1). She was referred to the thoracic/vascular service for evaluation. The history and physical identified a number of abnormalities. As a child, she recorded having had a “murmur” that disappeared at the age of 3 years. Subsequently, she recalled always having easy fatigue in her legs, and developing early onset hypertension, for which she was currently taking hydrochlorothiazide and lisinopril. She had had two normal pregnancies before her oopherectomy. Physical findings included very weak femoral pulses and a bruit in the upper left chest in the region of the subclavian artery. Right brachial systolic pressure was 170 mm Hg, left 150 mm Hg and both posterior tibial pressures 100 mm Hg. This, in conjunction with the fact that the abdominal aorta seemed extremely small, prompted a CTA of the chest which confirmed the diagnosis of coarctation of the thoracic aorta (Fig. 2). The CTA noted a bovine arch, marked intercostal collaterals without rib notching and dilation of both internal mammary arteries. The lesion was estimated to be 2 to 3 mm in diameter, just distal to the origin of the left subclavian artery. The diameter of the aorta proximal to the coarctation was 22 mm but distally rapidly decreased to 14 mm.
Because of the rapid change in diameter, we did not feel that a stent would be suitable as it would either be too large for the distal portion or too small for the proximal. In addition, a stent would “cage” the origin of the left subclavian artery. Because of the patient’s age, we felt a definitive repair would be optimal. A preoperative echocardiogram noted a bicuspid aortic valve without stenosis and mild left ventricular hypertrophy. The patient underwent correction with placement of a 14-mm interposition graft, beveled at the both ends to allow growth as well as too take into account the size discrepancy, via a postero-lateral thoracotomy. Left heart bypass was used.
The superior mesenteric artery aneurysm was focal, starting just distal the origin and ending distal to the first branch. On the fourth postoperative day the aneurysm was managed by placement of 2 8 mm × 2.5 cm Viabahn (W.L. Gore, Flagstaff, AZ) stents. The stents were placed under local anesthesia using a femoral approach. The patient was discharged on 8 days after the original procedure and at 1 year follow-up continued to do well with successful exclusion of the visceral aneurysm (Fig. 3). She remains normotensive and not requiring medication.
Coarctation of the thoracic aorta has been noted in 1 in 3000 to 4000 autopsies. One classification described pre, para, and postductal forms, the latter two traditionally being more commonly diagnosed in older children and adults. The postductal form, as was seen in this case, has a lower incidence of intracardiac defects, but can still be associated with aneurysm formation, dissection, coronary hyperplasia, and chronic effects of hypertension including ventricular hypertrophy. Typically, with increasing age, marked collaterals form, that can lead to rib notching and may be associated with chest wall bruits. Because of the associated complications and reduced life expectancy in patients with untreated coarctation, intervention is recommended.1,3
Surgical repair, whether by resection, patch, extra-anatomic bypass, or interposition graft, has been associated with excellent results, particularly in infants, although in adults the profuse collaterals can complicate the approach.1,4–6 Prosthetic patch or interposition graft may have slightly better long-term outcomes in the adult patient.7 Compared with angioplasty, there seems to be a lower incidence of postprocedure aneurysm formation with direct operative repair.4,5
The use if angioplasty and/or stents in the setting of recurrent coarctation after surgery has generally been accepted.8 Despite the results of operative management, there has been increasing emphasis in using catheter-based techniques in managing adult native coarctation with the hopes that equivelant results can be achieved at lower morbidity.9 Fawzy et al10 described their experience with angioplasty of native coarctation in 58 patients (24 ± 9 years of age) with a median follow-up of 13.4 years. Aneurysm developed in 7%, restenosis in 8%, and ½ patients had normalized blood pressure without the requirement for medical therapy. Because of the concern regarding restenosis, creating a dissection, development of aneurysm and possibly not being as effective in reducing hypertension, angioplasty has been supplanted by the use of stent grafts.8,11–16 It has been argued that covered stent grafts are particularly advantageous in allowing a more aggressive dilation with reduced complications and improved intermediate and long-term outcomes. Tanous and associates reported on 21 adult patients (14 with native coarctation) who underwent management with covered stents. On patient developed focal aortic rupture that was immediately corrected with a second stent. At 1 year there was a significant reduction in hypertension, utilization of antihypertensive medication, and no evidence of stent recoil.17 Fink-Josephi et al18 described the management of 12 adult patients with a thoracic endograft. The mean diameter at the coarctation site was 3.4 mm, but all procedures were successful, with no transfusions required, an average hospital stay of just over 2 days and at mean follow-up of 52 months no complications.
Managing coarctation by interventional techniques clearly offers an attractive alternative to open repair. There is the potential for much reduced length of stay, need for transfusion, and reduction in morbidity. Nevertheless, in healthy patients, surgical repair has the advantage of direct and durable amelioration of hypertension with long-term data that supports this approach. In addition, in this case, a stent would have had to be laid across the origin of the left subclavian artery, leading to the risk of emboli formation. In addition, because of the size discrepancy between the proximal and distal landing zones, two stents of different sizes would have been required, which raised concern regarding compression and endoleak. Thus, an operative approach was chosen.
Although coarctation in adults does have an association with intracranial aneurysms, there is no known predilection for visceral aneurysms.3 Visceral aneurysms, of which those involving the splenic artery are most common, have varying etiology, including medial degeneration, infection, peri-vascular inflammatory processes, and possibly atherosclerosis. With increasing use of CT an increasing number of asymptomatic aneurysms are detected.19,20 In general, visceral aneurysms have been described as having a risk of rupture of up to 40%, depending on location and suspected etiology, the risk increasing if ≥2 cm in diameter. Thus, consideration should be given for elective repair/resection of any symptomatic (or possibly symptomatic) aneurysm, those in higher risk areas (hepatic and splenic) and/or those ≥2 cm in diameter.2,21
Superior mesenteric artery aneurysm is quite rare. In four series totaling 144 visceral aneurysms, the superior mesenteric artery was involved in 11 (7.6%).19,20,22,23 The risk of rupture is not defined, but in this particular case the patient had abdominal pain in the epigastrium as well as a relatively large aneurysm.
As with coarctation, both open surgical as well as catheter-based techniques have been advocated.2,19,20 Saccular aneurysms may be embolized (while maintaining vessel patency) but the preferred approach, if anatomically suitable, is stent-grafting.22,23 This reduces the risk of failure to thrombose the aneurysm as well as complications of distal embolization. Sachdev et al24 compared the outcomes between surgical and endovascular management in 59 patients who between them had 61 visceral aneurysms. Of the 35 who underwent endovascular repair, the primary success rate was 89% and the secondary success (after repeat embolization or stenting) was 100%. The endovascular approach was associated with a markedly shorter length of stay. An alternative approach to persistent superior mesenteric aneurysm filling after stent-grafting can be image-directed injection of thrombin or other glue-like substances.25 In this case, the patient had excellent anatomy for treating the aneurysm with a stent-graft, with clear landing zones both proximally and distally.
Thus, this patient presented with an unusual pair of lesions, either of which could have been managed by open repair or endovascular stent grafts. The choice of which to perform for each lesion was based on anatomic considerations and an assessment of the patient’s risk factors. As endovascular techniques continue to evolve careful consideration to these factors remain critical. The optimal team should have facility with both open and interventional techniques so that all possibilities are considered.
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This enlightening case report contains valuable information for physicians involved in both the medical and surgical treatment of patients with diverse manifestations of cardiovascular disease. The particular combination of cardiovascular pathology in this article has not been previously described, and thus, this report can help guide the future treatment of other patients which may develop similar manifestations. Of significant importance also is the current perspective provided by the authors concerning the appropriate management of adult coarctation. There are several published case reports that describe an interventional strategy for adult coarctation; however, for the patient described, an open repair seemed to be the best option particularly because of the aneurysm of the superior mesenteric artery. The creative treatment approach developed by the authors for this patient could only have been successfully achieved in a collaborative environment using a combination of open surgical and endovascular skills. Other physicians should take note and continue to develop their own endovascular multispecialty teams to best care for patients with complex cardiovascular diseases.
Coarctation; Visceral; Aneurysm; Endovascular; Angioplasty
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