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Circulation. 2000;102:3023-3024

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(Circulation. 2000;102:3023.)
© 2000 American Heart Association, Inc.


Images in Cardiovascular Medicine

Granulomatous Aortitis Presenting as an Acute Myocardial Infarction in Crohn’s Disease

Michael H. Goldman, MD; Bechara Akl, MD; Shayryar Mafi, MD; Lucia Pastore, MD

From Fairfax Medical Center, Fairfax, Va.

Correspondence to Michael H. Goldman, MD, 1635 N George Mason Dr, Suite 150, Arlington, VA 22205. E-mail cardiovascularcare{at}erols.com

A 33-year-old man was admitted emergently with evidence of an acute inferior apical myocardial infarction. He had no previous cardiac history but did have a 20-year history of symptomatic Crohn’s disease. His subsequent coronary angiography revealed a long filling defect in the apical portion of the left anterior descending coronary artery, consistent with intraluminal thrombus (Figure 1Down). Left ventriculography demonstrated inferior apical akinesia. Visualization of the ascending aorta during ventriculography unexpectedly revealed a filling defect above the aortic valve. Aortography was performed, and it confirmed the presence of an apparently pedunculated mass {approx}4 cm above the valve, with no evidence of aortic insufficiency (Figure 2ADown).



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Figure 1. Coronary angiography in right anterior oblique projection reveals a filling defect in apical portion of left anterior descending coronary artery, consistent with a coronary embolus.



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Figure 2. A, Aortography in left anterior oblique projection reveals a filling defect (arrow) in aorta above aortic valve. B, Transesophageal echocardiography reveals a mobile mass (arrow) attached to anterior surface of aorta. C, MRI reveals small linear defect (arrow). D, CT scan reveals faint linear defect (arrow).

A transesophageal echocardiogram demonstrated a mobile, pedunculated mass attached to the ascending aorta, which was suggestive of an intimal flap and/or possible thrombus. No clear dissection was demonstrated (Figure 2BUp). MRI (Figure 2CUp) and CT scans (Figure 2DUp) demonstrated a "linear defect" consistent with a limited dissection.

At surgical exploration, the outer surface of the aorta appeared normal, without evidence of hematoma, dissection, or enlargement. The aorta was transected. Its intimal surface was smooth, with occasional scattered fibrofatty plaques. Adherent to the aorta was a pedunculated and friable sterile fibrin thrombus measuring 2.5x0.6x0.5 cm (Figure 3ADown). A short segment interposition graft was placed. Microscopic examination of the aorta revealed circumferential granulomatous aortitis with multinucleated giant cells, multifocal necrosis of the media, and perivascular chronic inflammation of the adventitia (Figure 3BDown). These findings were consistent with the granulomatous aortitis seen in giant cell arteritis and Takayasu’s disease.



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Figure 3. A, Resected portion of aorta with an adherent, pedunculated, sterile fibrin thrombus. B, Giant cell granulomas of inner media aorta with focal necrosis (hematoxylin and eosin; magnification, 40x).

The patient was subsequently continued on prednisone and methotrexate, as well as aspirin and Plavix (clopidogrel). During his hospitalization, blood cultures were negative. A full rheumatological evaluation (including HLA-B27 typing) was normal. There was no hematological evidence of hypercoagulability. One year later, he remains asymptomatic on prednisone and methotrexate.

Abnormalities of the ascending aorta in Crohn’s disease are almost always associated with ankylosing spondylitis and often a positive HLA-B27 phenotype.1 There have been 5 reports of aortic insufficiency in the absence of ankylosing spondylitis and HLA positivity.2 3 4 There have been no previous reports of Crohn’s disease–associated aortitis without aortic dilatation or valvular insufficiency.

Footnotes

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

References

1. Wackerlan A, Zund G, Maggiorini M, et al. Aortic valve insufficiency in Crohn’s disease. Schweiz Med Wochenschr. 1997;127:935–939.[Medline] [Order article via Infotrieve]

2. Burdick S, Tresch DD, Komokowski RA. Cardiac valvular dysfunction associated with Crohn’s disease in the absence of ankylosing spondylitis. Am Heart J. 1998;118:174–176.

3. O’Mahone SA, Moss F, Jepson E. Aortic regurgitation in Crohn’s disease. Am Heart J. 1990;119:1444. Letter.[Medline] [Order article via Infotrieve]

4. Leung WH. Aortic regurgitation in Crohn’s disease. Am Heart J. 1990;119:1445. Letter.




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