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IMAGES IN PATHOLOGY
Year : 2009  |  Volume : 55  |  Issue : 3  |  Page : 204-205

Missed aortic dissection - The Class 3 lesion


1 Departments of Pathology (Cardiovascular & Thoracic Division), Cardiovascular & Thoracic Surgery, Mumbai - 400012, India
2 Dr. PK Sen, Seth G. S. Medical College and K. E. M. Hospital, Mumbai - 400012, India

Date of Submission22-Aug-2008
Date of Decision04-Aug-2009
Date of Acceptance27-Aug-2009
Date of Web Publication2-Nov-2009

Correspondence Address:
P Vaideeswar
Departments of Pathology (Cardiovascular & Thoracic Division), Cardiovascular & Thoracic Surgery, Mumbai - 400012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0022-3859.57405

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How to cite this article:
Vaideeswar P, Patil L Y, Mishra P. Missed aortic dissection - The Class 3 lesion. J Postgrad Med 2009;55:204-5

How to cite this URL:
Vaideeswar P, Patil L Y, Mishra P. Missed aortic dissection - The Class 3 lesion. J Postgrad Med [serial online] 2009 [cited 2019 Dec 11];55:204-5. Available from: http://www.jpgmonline.com/text.asp?2009/55/3/204/57405


A 29-year-old male with Marfan syndrome was admitted for progressively increasing shortness of breath and palpitations for two months. On echocardiography there was severe aortic regurgitation and global hypokinesia (left ventricular ejection fraction of 15%). The dimensions at the aortic valvular regions were as follows: aortic annulus 32 mm, intersinus distance 53 mm, sinotubular junction 50 mm and ascending aorta 50 mm. The patient underwent Bentall procedure.

The aortic semilunar cusps, received separately, appeared translucent, stretched with rolled free margins. Three transversely-oriented intimal tears, measuring 2.2 x 0.3, 1.8 x 0.3 and 0.4 x 0.2 cm, respectively, were noted in the two resected flaps of the ascending aorta, associated with adventitial bulges [Figure 1]. There was no evidence of rupture. The intima was pale yellow and smooth. The tears revealed the walls to be composed of loose fibrocellular tissue and remnants of medial lamellar elastic units with a minor degree of undermining of the adjacent intima [Figure 2]. There was significant aortopathy in the form of elastic tissue loss and fragmentation, medionecrosis and increased collagenization [Figure 2]; only small foci of increased ground substance were noted.


 :: Discussion Top


The young man in the reported case had Marfan syndrome, a heritable autosomal-dominant disorder. It results from mutations in the genes coding for fibrillin-1, an integral component of elastin.[1] As a consequence, there is alteration in the quantity and quality of elastin, manifesting as a multisystemic disorder. Of clinical consequence is the involvement of the cardiovascular system, where abnormal aortic elasticity leads to aortic regurgitation and/or aneurysm.[2] Both features were present in the case presented. A more ominous complication is aortic dissection (AD), which has been traditionally classified on the basis of the extent of aortic involvement i.e. the Debakey and Stanford classifications.[3] The European Society of Cardiology classification has categorized AD into Classes 1 to 5, depending on the pathogenetic mechanisms.[4] Class 3 lesion has been described as "a stellate or linear intimal tear associated with exposure of underlying aortic media or adventitial layers" and importantly devoid of "progression and separation of the medial layers".[5] This description aptly fits with the gross morphology of the ascending aorta excised in this patient. These subtle forms of dissection are unusual and rare, discovered incidentally as they are not discernible on the current noninvasive imaging modalities such as transesophageal echocardiography, computed tomography or even cardiac magnetic resonance imaging; these Class 3 lesions are best picked up by aortography.[5] Alterations in the structure of the aortic media, as evident in our case, lead to dilatation of the aorta. Being less distensible, the intima tears-that leads to the more common Class 1 lesion or the classic AD and less often to Class 3 or limited AD. Though appearing innocuous, such intimal tears can sooner or later predispose to acute AD. In addition, due to the thinness of the wall, there can be seepage of fluid with pericardial effusion or rupture with hemopericardium. To prevent this dismal prognosis, it would be important to suspect this lesion in all patients with risk factors for aortic dissection, presenting with acute aortic syndromes, but without classic imaging characteristics.

 
 :: References Top

1.Isselbacher EM. Thoracic and abdominal aortic aneurysms. Circulation 2005;111:816-28.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Jeremy RW, Huang H, Hwa J, McCarron H, Hughes CF, Richards JG. Relation between age, arterial distensibility, and aortic dilatation in the Marfan syndrome. Am J Cardiol 1994;74:369-73.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Nienber CA, Eagle KA. Aortic dissection: New frontiers in diagnosis and management: Part I: From etiology to diagnostic strategies. Circulation 2003;108:628-35.   Back to cited text no. 3      
4.Erbel R, Alfonso F, Boileau C, Dirsch O, Eber B, Haverich A, et al. Diagnosis and management of aortic dissection. Recommendations of the Task Force on Aortic Dissection, European Society of Cardiology. Eur Heart J 2001;22:1642-81.  Back to cited text no. 4      
5.Svensson LG, Lahib SB, Eisenhauer AC, Butterly JR. Intimal tear with hematoma. An important variant of aortic dissection that can elude current imaging techniques. Circulation 1999;99:1331-6.  Back to cited text no. 5      


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  [Figure 1], [Figure 2]



 

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Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
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