Granulomatous Aortitis Presenting as an Acute Myocardial Infarction in Crohn’s Disease
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 1⇓). 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 ≈4 cm above the valve, with no evidence of aortic insufficiency (Figure 2A⇓).
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 2B⇓). MRI (Figure 2C⇓) and CT scans (Figure 2D⇓) 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 2.5×0.6×0.5 cm (Figure 3A⇓). 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 3B⇓). These findings were consistent with the granulomatous aortitis seen in giant cell arteritis and Takayasu’s disease.
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.
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.
- Copyright © 2000 by American Heart Association