(Circulation. 2000;102:3023.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
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 Crohns 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
2.5x0.6x0.5 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 Takayasus
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 Crohns 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 Crohns diseaseassociated 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 Lukes 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 Lukes 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 Crohns disease. Schweiz Med Wochenschr. 1997;127:935939.[Medline] [Order article via Infotrieve]
2. Burdick S, Tresch DD, Komokowski RA. Cardiac valvular dysfunction associated with Crohns disease in the absence of ankylosing spondylitis. Am Heart J. 1998;118:174176.
3. OMahone SA, Moss F, Jepson E. Aortic regurgitation in Crohns disease. Am Heart J. 1990;119:1444. Letter.[Medline] [Order article via Infotrieve]
4. Leung WH. Aortic regurgitation in Crohns disease. Am Heart J. 1990;119:1445. Letter.
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