DOI: 10.1111/j.1540-8175.2010.01243.xC© 2010, Wiley Periodicals, Inc.

Ostial Left Main Stenosis Due to Takayasu Arteritis:Multimodality Imaging and Surgical Ostioplasty

Neeraj Bansal, M.D.,∗1 Nan Wang, M.D.,∗ Daniel Choo, M.D.,† and Ramesh C. Bansal, M.D.†∗Division of Cardiothoracic Surgery, and †Division of Cardiology, Loma Linda University School of Medicine,Loma Linda, CA

Takayasu’s arteritis is an inflammatory process, involving larger blood vessels—namely the aorta andits branches. The majority of these patients present with symptoms of vascular obstruction. We reporta case of a 38-year-old Chinese female who presented with one month history of angina pectoris asthe initial manifestation. Coronary angiography showed 99% ostial left main coronary stenosis. Thediagnosis was first suspected in the operating room by TEE and subsequently supported by laboratorystudies and aortic biopsy. The technique of myocardial revascularization was altered and she underwentpatch ostioplasty of the left main coronary artery and aortic valve repair to correct aortic regurgitation.(Echocardiography 2011;28:E5-E8)

Key words: Takayasu’s arteritis echocardiography, computed tomography, left main stenosis, aorticvalve repair, coronary ostioplasty

Case Report:A 38-year-old Chinese female presented withsymptoms of chest pain occurring for a month.She described substernal tightness during exer-tion and relief within 5–10 min of rest. She hadno major past medical history, previous surg-eries, or coronary risk factors. Physical examina-tion demonstrated a grade II/VI early diastolicdecrescendo murmur heard in the aortic area.Her blood pressure was normal and there wereno vascular bruits. Routine hematologic, bloodchemistries and lipid levels were normal. HerC-reactive protein (CRP) level was elevated to5.6 mg/dL (normal <0.3 mg/dL) and Wester-gren erythrocyte sedimentation rate (ESR) was100 mm/h (female normal < 20 mm/h). Thesestudies were done after surgery.

Stress echocardiography was markedly posi-tive for ischemia and showed marked hypokinesisof the anteroapical segments at a workload of 7.2

1Current affiliation: Department of Surgery, University ofSouthern California, Los Angeles, CA.

Funding: None.

Conflicts of Interest: All authors have no conflicts to disclose.All work in this manuscript is original. All authors had accessto the data and played a role in writing the manuscript, andeach accepts responsibility for the content.

Address for correspondence and reprint requests: Ramesh C.Bansal, M.D., F.A.C.C, Director, Adult Echocardiography Lab-oratory, Loma Linda University School of Medicine, 11234Anderson Street, Room 4404, Loma Linda, CA 92354. Fax:909.558.0903; E-mail: a.gianluca2@alice.it

METs. Selective coronary angiography showed99% ostial left main coronary artery stenosis andcollateralization from the right system (Figs. 1Aand B).

The patient was taken to operating roomfor surgical treatment. Intraoperative trans-esophageal echocardiography (TEE) showedthickening of the ascending aortic wall (3 mm)and mild dilation of the ascending aorta (Figs. 2Aand B and movie clip 1). The ostium of the leftmain was stenotic and pulsed Doppler interro-gation of left main showed turbulent and highvelocity diastolic flow (Fig. 3A, movie clip 2).There was mild thickening of all three cuspsof the aortic valve and dilation of aortoven-tricular junction. Color flow imaging showedmoderate aortic regurgitation (Fig. 4). The

Figure 1. Selective coronary angiogram shows 99% ostialstenosis of the left main coronary artery, A. Right coronaryangiogram, B. shows collateral filling of left system (arrow).

E5

Bansal, et al.

Figure 2. Transesophageal echocardio-graphic (TEE) short-axis view of the aor-tic root (AO). A. shows thickening of thewall (arrow). Left main ostium appearsnarrowed (thin arrow). B. Long-axis viewof the aorta by TEE, dilation (3.5 cm) and3 mm thickening (arrow) of ascendingaorta.

Figure 3. Transesophageal echocardio-graphic (TEE) short-axis view of the aor-tic root (AO) and left main (LM) coronaryartery with color flow imaging. A. On thepreoperative image, narrowing of LM os-tium (arrow) is noted. B. Repeat imageafter LM surgical ostioplasty shows wideopen ostium (arrow).

Figure 4. TEE view of aortic valve. A.shows multiple jets (arrows) of aortic re-gurgitation (AR). B. Imaging after aor-tic valve repair shows trivial central AR(arrow).

Figure 5. Histology of aortic biopsy (Left panel: low powerand right panel: high power magnification) shows infiltrationof the media with lymphocytes (arrow).

Figure 6. A. Three-dimensional volume rendered CT Imageand B. multiplanar curved reformatted image after LM surgicalostioplasty show a wide open ostium of the LM (arrow).

E6

Ostial Left Main Stenosis Due to Takayasu Arteritis

diagnosis of Takayasu arteritis involving the leftmain coronary artery and aortic valve was made.The surgical management consisted of left maincoronary ostioplasty and aortic valvuloplasty.While on cardiopulmonary bypass, aorta wastransected 1 cm above the sinotubular junction.The aortic wall was noted to be 3-mm thick andhad an onion skin appearance. Additionally, it wasnoted that the aortic valve cusps had significantthickening and the left main had a very small pin-hole opening.

A longitudinal incision was made across theleft main until normal caliber was encountered,1–2 cm away from the aortic wall. Reconstruc-tion of the proximal left main was accomplishedby a generous triangular patch of autologouspericardium.1 Aortic valve competence was re-stored by performing a 240◦ posterior reductionannuloplasty. Interrupted U shaped stitches wereplaced between the right and left coronary os-tia along the posterior annulus below the aorticcusps. These sutures were tightened across a stripof bovine pericardium. Additionally, right- andleft-posterior commissuroplasty were performed.The aorta was closed with Prolene circumferen-tially. Intraoperative TEE confirmed the patencyof the left main coronary ostioplasty (Fig. 3B) andsatisfactory aortic valve repair (Fig. 4). The patientcame off the pump successfully.

The patient had an uneventful postoperativecourse. The histology of aortic specimen takenduring surgery showed vasovasoritis and lympho-cytic infiltration of the media (Fig. 5). A com-puted tomographic (CT) scan of the chest con-firmed the echocardiographic finding of diffusethickening of the wall of the ascending aorta andminimal involvement of arch and branch vessels(Fig. 6A). No stenosis at the origin of branch ves-sels was seen. Additionally, it showed a widelypatent left main ostioplasty site (Figs. 6A and B).Rheumatology was consulted and the patient wasstarted on a regimen of steroids and azathioprine.She felt symptomatically improved. Her ESR andCRP normalized within a month to 8 mm/h and<0.3 mg/dL, respectively.

Comment:Takayasu’s arteritis is a chronic inflammatory ar-terial disease of unknown cause that primarily in-volves aorta and its main branches. Takayasu’sarteritis typically affects young women and maycause stenosis, occlusion and aneurysm in variousarterial segments.2

Diagnosis is suspected using clinical criteria.In 1990, the American College of Rheumatologydeveloped a set of specific clinical criteria for diag-nosis of Takayasu’s arteritis.3 It is required that atleast three of six criteria are met to establish a clin-ical diagnosis of Takayasu’s arteritis. These criteria

include: (1) age of onset <40 years; (2) claudica-tion of extremities; (3) decreased brachial arterypulse; (4) blood pressure difference >10 mm Hg;(5) bruit over subclavian arteries or aorta; and(6) arteriogram abnormality.3 This patient clearlymet three of these six criteria as she presented atage 38 with angina (claudication of the heart) andhad an abnormal angiogram. Finally, serologicalfindings of elevated ESR and CRP with TEE find-ings of aortic thickening, ostial left main stenosisand aortic valve thickening in a young Asian fe-male are characteristic of Takayasu’s arteritis.

The 1994 International Takayasu Arteritis Con-ference used aortic angiography to determine thedistribution of involvement throughout the vas-culature and classified Takayasu’s arteritis on thebasis of these findings.4 Type-I involves branchvessels of the aortic arch. Type-IIa involves theascending aorta, aortic arch and its branches.Type-IIb involves the ascending aorta, aorticarch/branches and the descending thoracic aorta.Type-III involves the descending thoracic aorta,abdominal aorta and/or renal arteries. Type-IV in-volves the abdominal aorta and/or renal arteries.Type-V is the most severe with any combination oftype-IIb and IV. In addtion, involvement of coro-nary or pulmonary arteries would be designatedas C (+) or P (+). Our patient would be classifiedas type-IIa, C+.

Incidence of coronary involvement inTakayasu’s arteritis has been reported to be10.5% on autopsy and up to 38% on angio-graphic studies.5 Internal mammary and otherarterial grafts should be avoided in TA, particu-larly during the active stage.1 Surgical ostioplastyusing a patch of autologous pericardium, bovinepericardium, or saphenous vein has been usedas an alternative approach.1 The inflammationcan extend to aortic annulus and leaflets andcause AR. The aortic valve was repaired usingpartial pericardial patch annuloplasty and right-posterior commissuroplasty. Intraoperative TEEand postoperative CT scan showed excellentearly results. She was treated with recommendedmedical therapy6 for disease activity.

Angiography and computed tomographyhave been utilized for evaluation of Takayasu’sarteritis.2–4 Circumferential thickening of the aor-tic wall was noted in 71% of the aortic segmentsstudied by TEE in 14 patients in chronic stage ofTakayasu’s arteritis.7 These findings on TEE andcomputed tomography have occasionally beenconfused with intramural hematoma.8,9 Delayedhyperenhancement MRI may detect disease ac-tivity in the arterial wall.10 Clinicians should havea high index of suspicion for arteritis in youngpatients with systemic complaints and elevatedESR and CRP. The findings of imaging stud-ies should be interpreted in context of clinical

E7

Bansal, et al.

presentation. TEE is an extremely useful tool thatcan evaluate aortic wall thickening, aortic regur-gitation and the left main ostium. Furthermoreostioplasty rather than traditional bypass surgerymay be a more optimal revascularization strategyin these patients. Young patients with ostial leftmain stenosis should be considered for TEE to ruleout aortitis.

References1. Dhareshwar J, Zehr KJ, Schaff HV: Surgical ostio-

plasty for isolated left main stenosis. Ann Thorac Surg2007;83:1562–1563.

2. Johnston SL, Lock RJ, Gompels MM: Takayasu arteritis: Areview. J Clin Pathol 2002;55:481–486.

3. Arend WP, Michel BA, Bloch DA, et al: The American Col-lege of Rheumatology 1990 criteria for the classificationof Takayasu arteritis. Arthritis Rheum 1990;33:1129–1134.

4. Moriwaki R, Noda M, Yajima M, et al: Clinical manifesta-tions of Takayasu arteritis in India and Japan–new classifi-cation of angiographic findings. Angiology 1997;48:369–379.

5. Endo M, Tomizawa Y, Nishida H, et al: Angiographicfindings and surgical treatments of coronary artery in-volvement in Takayasu arteritis. Thorac Cardiovasc Surg2003;125:570–577.

6. Koening CL, Langford CA: Takayasu’s Arteritis. Curr TreatOptions Cardiovasc Med 2008;10:164–172.

7. Lira-Filho BE, Campos O, Compos O, et al: Thoracicaorta evaluation in patients with Takayasu’s arteritis bytransesophageal echocardiography. J Am Soc Echocardiogr2006;19:829–834.

8. Theodore S, Vaidyanathan K, Jagannath BR, et al:Takayasu’s arteritis mimicking acute aortic dissection. AnnThorac Surg 2007;83:1876–1878.

9. Halapas A, Kalogeropoulos AP, Georgiopoulou VV,et al: Takayasu’s arteritis mimicking aortic iintramuralhematoma in a female patient with chest pain. HellenicJ Cardiol 2008;49:280–283.

10. Desai MY, Stone JH, Foo TK, et al: Delayed contrast-enhanced MRI of the aortic wall in Takayasu’s arteritis:Initial experience. Am J Roentgenol 2005;184:1427–1431.

Supporting InformationAdditional Supporting Information may be foundin the online version of this article:

Movie clip 1. Transesophageal echocardio-graphic (TEE) long-axis view of the aorta showsdilation (3.5 cm) and thickening, typical of aorti-tis.

Movie clip 2. Transesophageal echocardio-graphic (TEE) short-axis view of the aortic root(AO) and left main (LM) coronary artery withcolor flow imaging. On this preoperative clip nar-rowing of the LM ostium is noted.

Please note: Wiley-Blackwell are not responsi-ble for the content or functionality of any support-ing materials supplied by the authors. Any queries(other than missing material) should be directedto the corresponding author for the article.

E8