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Cardiovascular Pathology
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Case 4 -
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Necrotizing Ortitis, Consistent with Takayasu Disease, with Involvement of Coronary Ostium and Ostial Thrombosis (Sudden Death)
Allen P. Burke
CVPath Institute
Gaithersburg, MD
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Clinical History
A 33-year-old Asian woman complained of dizziness for several months. She did not seek medical care,
and had no prior medical history other than for obstetric care, which was uncomplicated. There was no
family history of cardiovascular disease or sudden death. She died suddenly and unexpectedly after a
witnessed collapse.
A forensic autopsy was performed.
Case 4 - Slide 1
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Gross findings
The aorta demonstrated mild aneurysmal dilatation (Figure 1) . Upon opening the aorta, there were
patches of intimal irregularities and wrinkling suggestive of tree-barking (Figure 2). The heart weighed
310 grams. The aortic root was 3.5 cm in diameter and there was mild thickening of the valve leaflets at
the commissures (Figure 3). There was minimal narrowing of the arch vessels, but significant narrowing
of the visceral aortic branches at the ostia (Figure 4). Histologic sections of the aorta demonstrate
patchy destruction of the media with inflammation (Figure 5). The inflammatory infiltrate was composed
of chronic inflammation and macrophage giant cells (Figure 6). The heart was unremarkable, except for
coronary ostial extension of the inflammatory process, which resulted in mural thrombus in the right
ostium (Figure 7). The thrombus was organizing with surface erosion (Figure 8). The media of the right
coronary demonstrated elastic disruption with chronic inflammation (Figure 9).
Diagnosis
Necrotizing Ortitis, Consistent with Takayasu Disease, with Involvement of Coronary Ostium and Ostial Thrombosis (Sudden Death)
Discussion
Aortitis can be classified as infectious or non-infectious. Infectious aortitis includes syphilitic
aortitis, which is rare in developed countries, [1] and bacterial aortitis, which may be a complication
of endocarditis or sepsis and often results in so-called "mycotic" aneurysms. Non-infectious aortitis
may be isolated or associated with systemic collagen-vascular disease. [2] The most common histologic
form is characterized by patchy necrosis of the media, with loss of elastin and eventual replacement by
smooth muscle cells and extracellular matrix. The inflammation that accompanies the necrosis is
typically chronic, with macrophage giant cells at the interface between the necrotic zone and the normal
media. Infrequently, there may be a prominent neutrophilic infiltrate. There is invariably irregular
intimal fibrosis, which results in the gross appearance of "tree-barking", and adventitial scarring. The
inflammation and fibrosis of the adventitia is often accompanied by inflammation and occlusion of the
aortic vasa vasorum, or endarteritis obliterans. [2]
Necrotizing aortitis may be clinically isolated, or associated with a variety of autoimmune syndromes.
[3] The most frequent of these is Takayasu disease, followed by giant cell arteritis and rheumatoid
arthritis. There are distinct clinical differences between Takayasu aortitis and giant cell aortitis.
Takayasu disease occurs in young patients, typically women, in whom the aortitis is extensive, extending
into arch vessels and other aortic branches resulting in symptoms of vascular occlusion. This case
clinically fits Takayasu syndrome because of smaller vessel involvement, including the coronary and
mesenteric ostia, although absence of arch involvement is unusual. Giant cell aortitis is almost
exclusively seen in the elderly, and typically causes aortic root dilatation and aortic valve
incompetence. It is unclear if there are histologic differences between Takayasu and giant cell
aortitis; the degree of adventitial and initmal scarring tends to be less in necrotizing aortitis of the
elderly, and those not associated with Takayasu syndrome. A variant of non-infectious aortitis has been
described, in which there is no necrosis and minimal scarring, and diffuse medial chronic inflammation.
[4] This form of aortitis is associated with giant cell arteritis, is seen only in the elderly, and has a
propensity for medial dissection.
In this case, the immediate cause of death was coronary ostial thrombosis. Coronary ostial
involvement is uncommon in Takayasu aortitis, and is a rare cause of non-atherosclerotic coronary artery
disease. Sudden death due to coronary ostial occlusion has been reported rarely, especially with
coronary thrombosis
[5,
6,
7].
References
- Rojo-Leyva F, Ratliff NB, Cosgrove DM, 3rd, Hoffman GS. Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases. Arthritis Rheum 2000;43:901-7.
- Tavora F, Burke A. Review of isolated ascending aortitis: differential diagnosis, including syphilitic, Takayasu's and giant cell aortitis. Pathology 2006;38:302-8.
- Miller DV, Isotalo PA, Weyand CM, Edwards WD, Aubry MC, Tazelaar HD. Surgical Pathology of Noninfectious Ascending Aortitis: A Study of 45 Cases With Emphasis on an Isolated Variant. Am J Surg Pathol 2006;30:1150-1158.
- Burke A, Tavora F, Narula N, Virmani R. Aortitis and ascending aortic aneurysm: description of 52 cases and proposal of a histological classification Hum Pathol 2008;In press.
- Hjortshoj SP, Busk M, Gregersen M. [Takayasu's arteritis. An unusual cause of AMI and sudden death in a young man]. Ugeskr Laeger 2002;164:3366-7.
- Meyer W, Schneider J. [Sudden heart death in Takayasu aortitis with severe stenosis of the left coronary ostium]. Vasa 1985;14:180-3.
- Seguchi M, Hino Y, Aiba S, et al. Ostial stenosis of the left coronary artery as a sole clinical manifestation of Takayasu's arteritis: a possible cause of unexpected sudden death. Heart Vessels 1990;5:188-91.
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