Table of Contents  
Year : 2021  |  Volume : 8  |  Issue : 2  |  Page : 163-166

Beyond the arch: Ocular manifestation in middle aortic syndrome

Department of Cardiology, N.M. Wadia Institute of Cardiology, Pune, Maharashtra, India

Date of Submission08-May-2020
Date of Decision25-Jun-2020
Date of Acceptance14-Jul-2020
Date of Web Publication13-Apr-2021

Correspondence Address:
Ritu Bhatia
Department of Cardiology, N.M. Wadia Institute of Cardiology, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijves.ijves_54_20

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Middle aortic syndrome is a rare vascular disorder threatening life beyond the age of 40 years. We describe a rare case of atypical presentation of middle aortic syndrome in a 35-year-old female, diagnosed in the third decade of life. The patient was successfully treated with stent implantation; however, she developed vision disturbance in her right eye.

Keywords: Abdominal aorta, claudication, descending aorta, hypertension, percutaneous coronary intervention, stent, Takayasu arteritis

How to cite this article:
Bhatia R. Beyond the arch: Ocular manifestation in middle aortic syndrome. Indian J Vasc Endovasc Surg 2021;8:163-6

How to cite this URL:
Bhatia R. Beyond the arch: Ocular manifestation in middle aortic syndrome. Indian J Vasc Endovasc Surg [serial online] 2021 [cited 2023 Jan 29];8:163-6. Available from:

  Introduction Top

Middle aortic syndrome is a rare vascular disorder marked by segmental or diffuse narrowing of the distal thoracic descending aorta or abdominal aorta or both.[1] This disorder typically presents during childhood or adolescence and represents 0.5%–2.0% of all cases of stenosis of aorta. The spectrum of clinical presentation includes claudication of the lower limbs, absent femoral pulses, abdominal bruit, and disparate extremity pressure measurements. However, the most common clinical manifestation is uncontrolled hypertension. Uncontrolled hypertension can lead to cardiac failure due to cephalobrachial hypertension associated with morbidity of chronic kidney disease leading to mortality in this group of patients.[2]

  Case Report Top

A 35-year-old female presented with complaints of intermittent claudication, headache, and nausea. She had a history of accelerated hypertension and therefore adhered to a prescribed antihypertensive regime comprised of four antihypertensive drugs. She was also a tobacco user. Physical examination indicated pulse of 90 beats/min and blood pressure of 170/90 mmHg. Laboratory investigations revealed hemoglobin levels of 8.3 g/dL, estimated sedimentation rate of 20 mm/h, and high sensitivity C-reactive protein levels of 0.43 mg/dL. However, computed tomography (CT) angiography evidenced a short segment of significant irregular narrowing of the distal descending thoracic aorta above the level of the diaphragm [Figure 1] and [Figure 2]. The entire length of the descending thoracic aorta with thickening was approximately 7.5 cm in length, while the severely narrowed and stenosed segment measured approximately 10 mm in length. These findings of significant focal stenosis possibly attributable to coarctation of the descending thoracic aorta were consistent with middle aortic syndrome. All other surrounding vasculatures were normal including origin of the great vessels [Figure 3]. Multiple tortuous collaterals within the anterior chest and anterior abdomen wall were visible. Left ventricular hypertrophy was also confirmed. Magnetic resonance imaging of the brain and neck vessels displayed normal carotids and subclavian vessels. Renal arteries and mesenteric arteries were normal.
Figure 1: Computed tomography showing significant irregular narrowing of the distal descending thoracic aorta and mild dilatation of the of the ascending thoracic aorta

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Figure 2: Computed tomography angiography showing a short segment of significant irregular narrowing of the distal descending thoracic aorta above the level of the diaphragm

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Figure 3: Computed tomography showing sparing of visceral and renal vasculature

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Five months later in November 2019, the patient was readmitted with increased intensity of claudication pain. Check angiography revealed critical stenosis in the descending aorta at the level of the diaphragm. The option of surgical intervention versus endovascular treatment was discussed with the patient and her family. The chances of reocclusion and thrombosis were also discussed. The need of redo procedure in future was also explained. The patient chose endovascular treatment citing small children at home due to which she could not be away for a longer duration. In view of these discussions, we elected to treat the patient with endovascular treatment. A 6F radial sheath was inserted through the right radial artery. Pressure recorded was 210/102 mmHg. Subsequently, a 6F pigtail catheter was parked in the descending aorta, distal to the left subclavian artery with pressure injections taken in the anteroposterior view. At this position, the reference vessel diameter measured 11.94 mm proximal to the stenosis and 13.8 mm distal to the stenosis, with length of 32.6 mm. Next, the femoral artery was opacified with pressure injection. After opacification, the right femoral artery was punctured under fluoroscopic guidance taking the femur head as a reference. A 7F sheath was inserted through the right femoral artery. Pressure recorded in the right femoral artery was 86/75 mmHg. Pressure gradient of 124 mmHg (210–86 mmHg) was recorded.

We then began the stenting procedure. A 0.032” angled Terumo guidewire was crossed through the stenotic segment and then exchanged with a 0.35” ×260 mm Amplatz Ultra Stiff guidewire through cross exchange. A 45 mm CP covered stent (NuMed) was mounted on a 12 mm × 40 mm PTA Balloon Dilatation Catheter. After serial dilations with 9F, 10F, and 11F dilators, the sheath was exchanged for a 75 cm Cook Mullins sheath. The covered stent was mounted on the Atlas balloon and then advanced toward the thoracic aorta and the sheath was withdrawn below the stent. The position was confirmed with two shots, the first from the pigtail and the second from the Mullins sheath. The stent was deployed by dilation of the Atlas Balloon at 14 atm [Figure 4]. A second dilation was performed at 14 atm again. The procedure was uneventful. Postprocedure, pressure recorded in the right femoral artery was 187/100 mmHg and in the right radial artery was 196/100 mmHg. A pressure gradient of 9 mmHg (196–187 mmHg) was therefore recorded. The procedure was uneventful. The patient remained on a single antihypertensive agent, i.e., telmisartan after which she was introduced to steroids after discussing with an immunologist in periprocedural time though acute phase reactants were normal.
Figure 4: Aortography showing stent in situ

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Two months later in January 2020, the patient presented with complaints of blurred vision in the right eye, for duration of 15 days. Physical examination revealed pulse of 82 beats/min and blood pressure of 150/70 mmHg. Reduced (6/18) eyesight in her right eye led to a diagnosis of right eye branch vein occlusion with hypertension. The findings of ophthalmic examination were as follows: vision 6/36, slit-lamp examination normal, fundus showing branch retinal vein occlusion with neovascularization elsewhere, and relative afferent pupillary defect Grade 2. She was administered injection Avastin (bevacizumab). Her thrombophilia profile was unremarkable. Prothrombin time and international normalized ratio, protein S activity, protein C functional levels, and antithrombin activity were all within the normal range. The patient was prescribed steroids and azathioprine by her ophthalmologist and immunologist. Till date, there has been no progression of vision loss.

  Discussion Top

Middle aortic syndrome is a rare vascular disorder distinguished by diffuse or segmental narrowing of the thoracoabdominal aorta. Etiology may be acquired, caused by Takayasu's arteritis, temporal arteritis, neurofibromatosis, retroperitoneal fibrosis, William's syndrome, fibromuscular dysplasia, or congenital. In most cases, it involves both renal arteries (63%) and visceral arteries (30%).[3] Rarely, it may spare the visceral and renal vasculature (30%), develop collaterals (14.6%), consequence retinal abnormalities (10%), or occlude the distal thoracic aorta (3%).[2] Our case is rare in that our patient was revealed to have focal stenosis of the descending thoracic aorta with a vast collateral network, with all visceral and renal vasculature spared.

Takayasu arteritis is a common inflammatory manifestation of the aortic vasculature and an infamous underlying cause of middle aortic syndrome. Type III is distinguished by involvement of the thoracic descending aorta and the abdominal aorta with or without involvement of the renal arteries. It is the least reported type of Takayasu arteritis, documented in only 3.8% patients.[4] Involvement of thoracic descending aorta and the abdominal aorta observed in our patient lends credence to classification of type III Takayasu arteritis. Although type III encompasses cases with and without disease manifestation of the renal vasculature, as many as 80% cases report involvement of the renal vasculature.[5] Surprisingly, renal and visceral vasculature were completely spared in this case. Decreased vision is an ocular manifestation of Takayasu arteritis observed in 30% diagnosed patients.[6] However, it is almost unknown without involvement of the carotid arteries. Moreover, blood pressure of the patient was fairly controlled in that phase. Branch retinal vein occlusion has been reported as the first presentation of Takayasu arteritis by Conrath et al.[7] in 2004. This was the first case report of retinal vein occlusion revealing Takayasu arteritis. Though uncommon, small retinal vessel involvement can occur in Takayasu arteritis. Takayasu arteritis is a chronic inflammatory granulomatous vasculitis which affects large and medium arterial vessels. Sporadic cases of small vessel involvement have been documented in literature. Spared visceral and renal vasculature is rarely documented in inflammatory cases, thus the possibility of a congenital etiology was considered. Late presentation of the disease and decreased vision are again not fitting into congenital. Finally, temporal arteritis was not kept differential because of age and sparing of carotids and temporal vessels.

Treatment may be pharmacological, endovascular, or surgical. Pharmacotherapy may prove adequate for cases displacing mild to moderate stenosis, although more critical stenotic lesions accompanied by severe hypertension or kidney impairment warrant endovascular or surgical intervention.[2] Earlier experiences with endovascular treatment exposed inferiority to surgical procedure. Nevertheless, recent advances have paved the way to attain favorable short-term results with patency rates comparable to surgical intervention. Long-term results still remain controversial.[8] Percutaneous interventions present as a safe and minimally-invasive technique with covered stents as a better choice as compared to older generation of CP stents or jostents. Despite fear of restenosis and thrombosis, several cases of successful endovascular stenting of mid-aortic syndrome have been reported with increasing use of this treatment strategy.[1],[8],[9],[10]

  Conclusion Top

Middle aortic syndrome is a rare vascular disorder. Etiology is enigmatic although current literature in the form of case series and case reports evidence congenital, acquired, and inflammatory causes. Presentation may be atypical as illustrated in the present case. Nonetheless, selected cases may successfully be managed with stent implantation with favourable long-term outcomes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Lee ML, Chiu IS, Yang AD. Middle aortic syndrome treated by implantation of an advanta V12 large diameter stent. Arq Bras Cardiol 2016;106:73-5.  Back to cited text no. 1
Rumman RK, Nickel C, Matsuda-Abedini M, Lorenzo AJ, Langlois V, Radhakrishnan S, et al. Disease beyond the arch: A systematic review of middle aortic syndrome in childhood. Am J Hypertens 2015;28:833-46.  Back to cited text no. 2
Delis KT, Gloviczki P. Middle aortic syndrome: From presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther 2005;17:187-203.  Back to cited text no. 3
Jain S, Kumari S, Ganguly NK, Sharma BK. Current status of Takayasu arteritis in India. Int J Cardiol 1996;54 Suppl: S111-6.  Back to cited text no. 4
Zaki SA. Middle aortic syndrome caused by Takayasu arteritis. Heart Views 2012;13:116-7.  Back to cited text no. 5
[PUBMED]  [Full text]  
Peter J, David S, Danda D, Peter JV, Horo S, Joseph G. Ocular manifestations of Takayasu arteritis: A cross-sectional study. Retina 2011;31:1170-8.  Back to cited text no. 6
Conrath J, Hadjadj E, Serratrice J, Ridings B. Branch retinal vein occlusion reveals Takayasu's arteritis. J Fr Ophtalmol 2004;27:162-5.  Back to cited text no. 7
Dogan A, Sever K, Ozdemir E, Mansuroglu D, Kurtoglu N. Endovascular stenting of mid-aortic syndrome due to Takayasu arteritis. Acta Chir Belg 2018;118:264-8.  Back to cited text no. 8
Rabellino M, Garcia-Nielsen L, Gonzalez G, Baldi S, Zander T, Maynar M. Middle aortic syndrome percutaneous treatment with a balloon-expandable covered stent. J Am Coll Cardiol 2010;56:521.  Back to cited text no. 9
Glotzer OS, Bowser K, Harad FT, Weiss S. Endovascular management of middle aortic syndrome presenting with uncontrolled hypertension. Case Rep Vasc Med 2018;2018:9586025.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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