Thoracic Endovascular Stent Graft Repair of Middle Aortic Syndrome Joung Taek Kim, MD, Mina Lee, MD, Young Sam Kim, MD, Yong Han Yoon, MD, and Wan Ki Baek, MD Department of Thoracic and Cardiovascular Surgery, Inha University Hospital, Incheon, Korea
Middle aortic syndrome is a rare disease deﬁned as a segmental narrowing of the distal descending thoracic or abdominal aorta. A thoracoabdominal bypass or endovascular treatment is the choice of treatment. Endovascular therapy consists of a balloon dilatation and stent implantation. Recently, thoracic endovascular aortic repair has been widely used in a variety of aortic diseases. We report a case of middle aortic syndrome treated with a thoracic endovascular stent graft. (Ann Thorac Surg 2016;102:e233–5) Ó 2016 by The Society of Thoracic Surgeons iddle aortic syndrome (MAS) was ﬁrst described as a subisthmic coarctation by Dr Schlessinger in 1835 . It was described what we now call the middle aortic syndrome in 1963 by Sen and colleagues . He described a typical patient, a young woman of 25 years of age who had complained of symptoms of hypertensive disease or, less frequently, of lower limb claudication or abdominal angina . It manifests as the consequences of aortic or renal artery stenosis. The treatment was mainly surgical reconstruction of the aortic stenosis with bypass grafting . Endovascular treatment, primarily in the form of a balloon angioplasty, has been used with variable success [1, 4–7]. As endovascular stents have been widely used in coronary arteries, it was implanted in young adult patients with MAS. The stent contributed to more technical success and lower restenosis rate than balloon angioplasty alone, but an aneurysm around the Wall stent or intimal tissue prolapse on follow-up were discovered in a few cases . A stent graft, widely used in recent years for aortic disease can protect against these problems, even for full thickness tears.
A 48-year-old male patient was referred to our department for further evaluation of thoracic aorta calciﬁcations. He came to our hospital complaining of dizziness and chronic renal failure. His blood pressure was 160/100 mm Hg on his right arm, 135/100 mm Hg on his left arm, and his serum creatinine was 2.1 mg/dL. His past medical history included a cerebral hemorrhage 20 years previous to his visit. The patient complained of bilateral claudication and weakness of lower extremities. A soft systolic Accepted for publication Jan 28, 2016. Address correspondence to Dr Joung Taek Kim, Department of Thoracic and Cardiovascular Surgery, Inha University Hospital, 7-206, Sinheungdong 3-ga, Jung-gu, Incheon 400-711, Korea; email: [email protected]
Ó 2016 by The Society of Thoracic Surgeons Published by Elsevier
murmur was checked on his mid-back area. A 3-dimensional (3D) angio computed tomography (CT) revealed a diffuse and segmental narrowing of the descending thoracic aorta to the diaphragm level with severe calciﬁcation (Fig 1) and left subclavian artery obstruction. The minimum diameter of the stenosis was 4 mm and the length of the stenosis was 9 cm. The patient had developed extensive collateral vessels to the abdominal organs. We diagnosed him with middle thoracic syndrome, suprarenal aortic coarctation without renal involvement. The intervention was planned as a 2-stage procedure. First, we would implant a stent with only moderate dilatation of the stenosis segment, and then secondly, we would deﬁnitively dilate it after 3 months. First, a thoracic endovascular aortic repair (TEVAR) was performed under general endotracheal anesthesia. The stenosis was crossed from the right femoral approach using 18F sheath (St. Jude Medical, Plymouth, MN). The stent graft (18 mm 13 cm, S & G Biotech, SeoungNam City, Korea) was advanced to the stenosis lesion (Fig 2) and deployed. The stenosis lesion was dilated with a 10 mm Seal balloon catheter (S & G Biotech) after placement of the stent graft. The balloon was gradually inﬂated to its normal diameter up to a pressure of 10 to 20 atm. The systolic pressure gradient prior to the TEVAR was 44 mm Hg (right arm 150/90 mm Hg, right leg 106/74 mm Hg). The postTEVAR pressure gradient was 28 mm Hg (right arm 139/77mm Hg, right leg 111/77 mm Hg). A second dilation was done with a 14 mm Seal balloon catheter (S & G Biotech) after 3 months. The systolic pressure gradient was 18 mm Hg (right arm 122/73 mm Hg, right leg 104/59 mm Hg). One year follow-up 3D CT angiogram (Fig 3) revealed improved descending aorta stenosis and collateral vessels had decreased in abundance. His serum creatinine level had normalized. The ﬁnal systolic pressure gradient was remained less than 20 mm Hg at 20 months’ follow-up. The claudication symptom and systolic murmur had disappeared. The antihypertensive treatment was withdrawn.
Comment MAS is produced by congenital factors or by a number of acquired etiologies [1–3]. Congenitally, it is a subtype of aortic coarctation, which locates just distal to the ductus arteriosum or ligamentum arteriosum mostly, and accounts for 0.5% to 2% of all coarctations . Morphologically, coarctation presents as a luminal narrowing of the aorta several centimeters in length or as a sharp, web-like constriction, whereas MAS looks like a diffuse segmental narrowing of descending aorta. Dr. Sen, who described MAS ﬁrst in 1963, reported that the most common lesion in MAS was nonspeciﬁc allergic panarteritis of a segmental nature involving the middle part of aorta. More often it is supradiaphragmatic but not infrequently infradiaphragmatic, and occasionally it is both. Involvement of the renal arteries is an additional factor responsible for hypertension in some cases 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2016.01.099
CASE REPORT KIM ET AL MIDDLE AORTIC SYNDROME
Ann Thorac Surg 2016;102:e233–5
Fig 1. Three-dimensional angio computed tomography showed diffuse and segmental narrowing (arrows) of descending thoracic aorta to diaphragm level with calciﬁcation.
Fig 3. Three-dimensional angio computed tomography at 1-year follow-up shows widened stenosis.
. The external diameter of the aorta was not reduced but there was signiﬁcant thickening of the adventitia as well as the intima coats resulting in much narrowing of the lumen in necropsies . A histologic examination showed an allergic panarteritis similar to that described in Takayasu’s syndrome [2, 3]. Our patient seems to have had Takayasu’s arteritis because clinical or radiological test results satisfy all of the diagnostic criteria of Takayasus’s arteritis for the American College of
Rheumatologists [2, 3]. We think that the patient had suffered from Takayasu’s arteritis in his second decade of life considering the left subclavian artery obstruction. Treatment of the MAS has been reported in a variety of ways according to the anatomical location of the affected aortic segment. Recently, intravascular stents have been used as an alternative to surgery for treatment of MAS. An aortic stent was used to treat restenosis after coarctation surgery, originally . A covered stent had yielded a good indication of when such a coarctation and aneurysm coexist , but bear stents have also been commonly used in MAS . There was a report of aneurysm formations after stent implantation in coarctation of the aorta and MAS [4, 5]. Intimal hyperplasia may develop, and it will impose potential risks especially in patients with active arteritis. Extensive data about bear stents exist in patients treated for isthmic coarctation [4–6], yet, limited data are available on the efﬁcacy and durability of stent grafting for MAS . There were 2 tight stenoses of 4 mm in diameter and a 9 cm length of calciﬁcation in this case. Rupture after balloon dilatation of these extreme stenotic segments would impose possible hazardous complications. Dilatation of the balloon in the covered stent and a 2-stage procedure gave us a sense of safety. Although there was still an 18 mm Hg pressure gradient due to recoil of aortic wall calciﬁcation, technical success has been achieved because technical success is deﬁned as residual gradient of 20 mm Hg or less . The radial force of usual thoracic stent grafts are less than 3 Newton . They are produced using with 0.30 to 0.35 inches wire usually. We recommend a stent graft using with stronger wire to get more radial force and to prevent recoil in these case.
Fig 2. The stent graft (arrow) is advancing to the stenosis lesion.
Ann Thorac Surg 2016;102:e233–5
In summary, the midterm results of the implantation of the covered stent in MAS are encouraging. These may give another treatment option to treat middle aortic syndrome. This report was supported by an Inha University Research Grant.
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CASE REPORT KIM ET AL MIDDLE AORTIC SYNDROME
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