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Jornal Vascular Brasileiro

Print version ISSN 1677-5449

J. vasc. bras. vol.8 no.4 Porto Alegre Dec. 2009 



Endovascular treatment of the middle aortic syndrome due to Takayasu’s arteritis: case report



Felipe NasserI; Joaquim Maurício da Motta-Leal-FilhoI; Francisco Cesar CarnevaleI; Caio Cesar Martins FocassioI; Dênis SzejnfeldI; Rodrigo OliveiraII; José Nery PraxedesII; Giovanni Guido CerriI

IServiço de Radiologia Intervencionista, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo, SP
IIServiço de Nefrologia, Hospital das Clínicas, FMUSP, São Paulo, SP





Middle aortic syndrome (MAS) is a clinical condition characterized by segmental or diffuse narrowing of the aorta, hypertension, and lower limb claudication. The main cause of MAS is Takayasu’s arteritis. Segmental aortic stenosis may be located at the suprarenal, renal or infrarenal aorta with high tendency to concomitant stenosis in both the renal and visceral arteries. Severity of hypertension is the primary indication for intervention. Endovascular therapy is a minimally invasive treatment for MAS and may provide good results. In the present report, we describe a successful endovascular treatment of stenosis of the thoracoabdominal aorta with recanalization of the superior mesenteric artery using angioplasty in a 34-year-old woman with Takaysu’s arteritis and severe hypertension.

Keywords: Takayasu’s arteritis, balloon angioplasty, stents; abdominal aorta, renal artery obstruction, celiac artery.




Takayasu's arteritis (TA) is a chronic inflammatory vascular disease of unknown etiology, characterized by lesions to major vessels, aortic branches, especially the supra-aortic trunks, and can even afflict the pulmonary artery. It is more common among young (15-25 years old) women (9:1 ratio) of Asian descent. Aortic and aortic branch lesions may be aneurysmal or stenotic-occlusive.1-3 Among parietal arteries, the most commonly afflicted vessel is the left subclavian artery; among visceral ones, the renal arteries, explaining the association between TA and renovascular hypertension. Treatment is based on:

1) controlling disease activity through use of immunosuppressants.

2) surgical correction for long stenotic-occlusive lesions or when several vsiceral branches become compromised;

3) or endovascular treatment by angioplasty and/or stent insertion.1,3-5

Endovascular treatment is effective for stenosis of the aorta and its parietal and visceral branches, especially for ostial lesions.3 It is widely known that disease progression causes high rates of early relapse, especially for renal arteries.1,3,6

Patients with TA may suffer from various clinical manifestations, depending on the location of the arterial damage. Lesions may be divided into four types: type I (Shimizu-Sano), involvement of the aortic arch and its branches; type II (Kimoto), involvement of the descending thoracic and abdominal aorta, without involvement of the aortic arch; type III, combination of arterial compromises types I and II; and type IV, arterial compromises types I, II or III associated with compromise of the pulmonary artery.7 When compromise of the thoracoabdominal and/or abdominal aorta leads to systemic hypertension associated with decreased femoral pulse and claudication of the lower limbs, the condition is known as middle aortic syndrome (MAS).4,5,8-12 Hypertension seems to be caused by mechanical obstruction of the aorta.

The objective of this article is to report a case of endovascular treatment for stenosis of the thoracoabdominal aorta and recanalization of the superior mesenteric artery by balloon angioplasty in a patient suffering from type III TA.1,7


Case report

The patient was female, 34, and asymptomatic. She had been diagnosed with TA, controlled by continuous use of immunosuppressants (prednisone 5 mg, 2/2 days and methotrexate 25 mg/week). She denied having symptoms compatible with claudication of the upper limbs, lower limbs, and gastrointestinal issues. She was undergoing treatment for renovascular hypertension using three medications (atenolol 100 mg/day, amlodipine 10 mg/day, and chlortalidone 25 mg/day). Physical examination: bruit in left carotid artery, both subclavian arteries, and left side of small of the back. Femoral pulses were decreased (+2/+4) and symmetric. Blood pressure (BP): 170 x 80 mmHg in upper right limb, 160 x 80 mmHg in upper left limb. Angiographic tomography from two years previous identified stenotic lesion to the left subclavian artery (LSA), brachiocephalic trunk (BCT), left common carotid artery (LCCA), thoracoabdominal artery (TAA), superior mesenteric artery (SMA), and renal arteries (RA). Current angiographic tomography showed lesions had worsened in SMA artery, left renal artery (LRA) and TAA (Figures 1 and 2). Inflammatory laboratory tests (C-reactive protein 1.64 mg/L (normal < 3.0); hemosedimentation speed = 14 mm/h (normal < 12 for women), without significant alterations, showed the disease was under control; and renal function was preserved (creatinine 0.6 mg/dL; creatinine clearance 102 mL/min). Considering the presence of major stenotic lesions and the patient's hypertension, it was decided she should undergo endovascular treatment.





Technique: Right femoral arterial access was obtained using the Seldinger technique. The anesthesia team administered mild sedation, and general anesthesia was not required. An aortogram using a pigtail catheter positioned on the distal thoracic aorta confirmed stenosis of the TAA, non-opacification of the SMA, moderate stenosis of renal arteries and moderate stenosis of the infrarenal abdominal aorta (IAA) (Figure 3). The aortic translesional systolic pressure gradient (proximal to diaphragm level and distal to level of bifurcation of iliac arteries) was 50 mmHg. Systemic heparinization at 100 UI/kg was followed by TAA angioplasty using a 10 x 40 mm balloon catheter (Ultrathin Diamond, Boston Scientific, Natick, EUA) (Figure 4). Balloon inflation lasted 3 minutes, at maximum pressure of 15 atm. Post-treatment angiography showed improvements to the aortic lesion and SMA opacification. The translesional pressure gradient dropped from 50 to 10 mmHg, showing the aortic lesion was effectively treated. Renal arteries were untreated. During immediate postoperative period, pressure dropped in the upper limbs (BP 130 x 80 mmHg) and use of antihypertensive medication decreased (atenolol 50 mg/day, amlodipine 5 mg/day). The patient remained asymptomatic; she was discharged on the third day. She was prescribed acetylsalicylic acid (ASA), 200 mg/day, and clopidogrel 75 mg/day, the latter for six months, as well as routine antihypertensive and immunosuppressant medications. She is currently in her tenth postoperative month, with pressure controlled solely by atenolol 25 mg/day; she is also using ASA 200 mg/day, prednisone 5 mg - 2/2 days and methotrexate 25 mg/week.






MAS, a limited or diffuse involvement of the thoracoabdominal aorta, is an unusual cause for systemic hypertension and claudication of the lower limbs.4,5,8,9 Its etiopathogeny is unknown and controversial. Some authors argue it is congenital, due to anomalous fusion of dorsal aortas in the fourth week of pregnancy.4,8-12 Others claim it is associated with aortitis. The syndrome is indeed associated with a few other diseases, such as Takayasu's arteritis, neurofibromatosis, retroperitoneal fibrosis, and mucopolysarccoidosis.4,8-12

In this case, the patient has had TA for 10 years (meeting for diagnostic criteria from the American College of Rheumatology and meeting Ishikawa's diagnostic criteria)2,13 and systemic hypertension for 3 years. She had no claudication of the lower limbs, but performed no regular physical activity and had decreased but symmetrical femoral pulses. The patient was receiving clinical treatment for TA. The treatment is well established in the literature, comprehending the use of immunosuppressants such as prednisone and/or methotrexate to decrease or eliminate inflammatory activity.1-3 Immunosuppressants should be administered continuously, even if markers for inflammatory activity become normal. Surgical and endovascular treatment should only be performed when there is no inflammatory activity, which can be achieved through continuous use of immunosuppressants. Otherwise, both treatments become risky and, if undertaken during a period of inflammatory activity, achieve poorer outcomes.3,14

The status of endovascular treatment is controversial in the literature. Several authors use angioplasty with stent insertion because they believe this leads to lower rates of complications (dissection and restenosis) and increase vessel permeability.1,3,5 Other authors use cutting balloons because they believe lesions are hardened and resistant.3 However, keep in mind that cutting balloons have maximum diameter of 8 mm, and not every patient has aortic lesions that allow their use. Balloon catheter angioplasty without stent insertion was chosen because the case involved an aorta 10 mm in diameter with stenotic lesions localized between the visceral branches of the TAA (celiac trunk, SMA and RA). Stent insertion in this region would create technical difficulties for further approaches to these visceral branches and could even occlude the branches. Notice that this is a progressive inflammatory disease which usually requires other endovascular procedures to maintain permeability for vessels receiving treatment.1,3,6 The post-angioplasty control arteriogram of the TAA revealed opacification of the SMA, leading to the conclusion that stenotic lesions were overlaid (severe stenosis in TAA, mild ostial stenosis in SMA) and that treating the TAA had enabled increased flow to the SMA, restoring normal arterial flow, making it less significant and precluding the need for angioplasty for the latter.

Though the patient had stenotic lesions in her renal arteries, especially the left RA, only the aorta was treated because hypertension was caused by stenosis of the TAA (MAS), a finding confirmed by the decreased pressure gradient immediately after percutaneous transluminal angioplasty (from 50 to 10 mmHg), decreased systemic blood pressure, and the subsequent diminished need for antihypertensive medications.

Conventional surgical treatment is viable, and recommended especially when aortic lesions are extensive, but has higher rates of morbidity and mortality.1,3,8,9

Post-operative follow-up of these patients requires routine examinations, blood pressure monitoring, inflammatory laboratory tests, and imaging of the aorta and aortic branches to diagnoses treatable stenotic lesions, since even when the disease is under clinical control, arterial lesions may progress and require further interventions.1,3,5,6,14

MAS caused by Takayasu's arteritis is an extremely serious, progressive disease. Endovascular treatment is recommended, since it has the advantages of being a minimally invasive technique, enabling further reinterventions and having good immediate outcomes, as well as allowing patients to return to their routines more quickly. Due to its progressive nature, patients who suffer from it need to control the disease through the use of anti-inflammatory and/or immunosuppressant medication, periodical angiographic tomography monitoring, and new procedures whenever necessary.



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2. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33:1129-34.         [ Links ]

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4. Perrone-Filardi P, Costanzo P, Cesarano P, et al. Long abdominal aortic stenosis: a rare presentation of Takayasu arteritis treated with percutaneous stent implantation. J Thorac Cardiovasc Surg. 2007;133:1647-8.         [ Links ]

5. Bialkowski J, Szkutnik M, Bermúdez-Cañete R, Kusa J, Regiec S, Mullins CE. Middle aortic syndrome caused by Takayasu’s disease and treated by stent implantation: a report of medium-term follow-up. Rev Esp Cardiol. 2002;55:682-5.         [ Links ]

6. Giordano JM, Hoffman GS. Takayasu’s disease: nonspecific aortoarteritis. In: Rutherford RB, editor. Vascular surgery. 5ª ed. Philadelphia: WB Saunders; 2000. p. 364-73.         [ Links ]

7. Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE. Takayasu’s arteritis. Clinical study of 107 cases. Am Heart J. 1977;93:94-103.         [ Links ]

8. Adovasio R, Canci U. Middle aortic syndrome: an atypical case - a case report. Angiology. 2000;51:525-8.         [ Links ]

9. Seki A, Ogawa H, Fujiu K, Kawagoe Y, Kasanuki H. Middle aortic syndrome diagnosed at 54 years of age - a case report. Angiology. 2002;53:605-8.         [ Links ]

10. Sen PK, Kinare SG, Engineer SD, Parulkar GB. The middle aortic syndrome. Br Heart J. 1963;25:610-8.         [ Links ]

11. Daimon S, Kitamura K. Coarctation of the abdominal aorta. Jpn Heart J. 1964;5:562-73.         [ Links ]

12. Lande A. Takayasu’s arteritis and congenital coarctation of the descending thoracic and abdominal aorta: a critical review. AJR Am J Roentgenol. 1976;127:227-33.         [ Links ]

13. Ishikawa K. Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu’s arteriopathy. J Am Coll Cardiol. 1988;12:964-72.         [ Links ]

14. Park MC, Lee SW, Park YB, Lee SK, Choi D, Shim WH. Post-interventional immunosuppressive treatment and vascular restenosis in Takayasu’s arteritis. Rheumatology (Oxford). 2006;45:600-5.         [ Links ]

Joaquim Maurício da Motta Leal Filho
Rua Diogo Jacome, 1030/121, Vila Nova Conceição
CEP 04512-001 - São Paulo, SP - Brazil
Tel.: +55 (11) 5052.9028, +55 (11) 9974.7794

Manuscript received Jul 13 2008, accepted for publication Sep 25 2009.



No conflicts of interest declared concerning the publication of this article.

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