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Ann Thorac Surg 2004;78:1070-1072
© 2004 The Society of Thoracic Surgeons

Case report

Diagnostic criteria for penetrating atheromatous ulcer of the thoracic aorta

Shigeki Kimura, MDa, Makoto Noda, MDa,*, Michio Usui, MDa, Mitsuaki Isobe, MDb

a Department of Cardiology, Haibara General Hospital, Shizuoka, Japan
b Department of Cardiovascular Medicine, Tokyo Medical and Dental University, Tokyo, Japan

Accepted for publication June 25, 2003.

* Address reprint requests to Dr Noda, Department of Cardiology, Haibara General Hospital, 2887-1 Hosoe, Haibara, Shizuoka 421-0421, Japan
mnoda-circ{at}umin.ac.jp


   Abstract
Top
 Abstract
Introduction
 Comment
 References
 
An autopsy case of penetrating atheromatous ulcer of the ascending thoracic aorta is presented. The triplet imaging findings of mediastinal dilatation, interstitial pulmonary hemorrhage and pericardial effusion were diagnostic for penetrating atheromatous ulcer at the posterior wall of the ascending thoracic aorta. Immediate surgical treatment is indicated when these three findings emerge during examination.


   Introduction
Top
 Abstract
 Introduction
Comment
 References
 
Penetrating atheromatous ulcer (PAU) is a rare vascular condition that was designated by Stanson and asociates as a synonymous vascular disease of the dissecting aneurysm of the aorta [1], but the pathophysiology, diagnosis, and management of PAU are often difficult. We describe an autopsy case of PAU originated from the ascending thoracic aorta and propose new diagnostic criteria and therapeutic recommendation of PAU.

A 76-year-old man was admitted to our hospital because of sudden vertigo and chest dyspnea. He had been suffering from high blood pressure for more than 10 years. His symptoms disappeared upon his arrival at our hospital. Laboratory examination revealed slight anemia, and chest computed tomography (CT) revealed dilatation of the mediastinum and slight pericardial effusion (Fig 1A, B). The ascending thoracic aorta was dilated up to a maximum diameter of 48 mm, but repeated chest CT revealed no evidence of intimal flap, false lumen, mural thrombosis, or extravascular leakage of contrast medium throughout the aortic vasculature (Fig 1A). However, the space between the posterior wall of the ascending thoracic aorta and the anterior wall of the trachea exhibited heterogeneous low density (Fig 1A), and enlarged broncho-vascular markings were evident on the chest CT (Fig 1B). An echocardiogram revealed slight pericardial effusion without aortic insufficiency.



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  		Fig 1. (A) Chest computed tomography (CT) scan showing dilatation of the ascending thoracic aorta without extravascular leakage of contrast medium. Note the presence of an area of heterogeneous low density between the posterior wall of the ascending thoracic aorta and the anterior wall of the trachea (asterisk). (B) Chest CT scan showing enlarged broncho-vascular markings on the lung (see inset for a magnified view of the area enclosed by a dotted line) and the slight pericardial effusion.

 
We were unable to rule out an atypical form of dissecting aortic aneurysm, and therefore, hemodynamic monitoring was initiated immediately in the intensive care unit, even though the patient's vital signs were stable. His clinical course was uneventful until the second day of admission, when he started coughing and experienced progressive hemodynamic collapse with massive pericardial effusion. He died of cardiac tamponade and an autopsy was performed.

Autopsy findings included atherosclerotic plaques in the thoracic and abdominal aorta. A round atheromatous ulceration with a maximum diameter of 10 mm was found 5 cm distal to the aortic valve at the posterior wall of the aortic root just above the level of the upper pericardial reflection (Fig 2). The atheromatous ulceration had penetrated vigorously in a backward direction with a massive hematoma. A remarkably large clot and fluid of blood occupied the posterior mediastinal tissue and extended into visceral structures through the vasculature at hilus of both lungs. Bloody fluid had also entered the pericardiac space through the upper reflection of the lacerated pericardium. Histologic examination revealed abrupt and transmural disruption of the atheromatous aortic wall. Interstitial hemorrhage was demonstrated around the broncho-vascular structures in the lungs.



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  		Fig 2. Macroscopic view (left) of the right posterolateral side of the heart obtained from an independent autopsy, shown for reference, and a schematic representation (right) of the heart shown on the left. Steel rods were inserted through the left atrium, the right atrium, and the transverse pericardial sinus, respectively. The estimated site of rupture in our patient (asterisk) was located in the posterior wall of the aortic root, just above the level of the upper pericardial reflection (closed circles). Dotted circles represent the superior and inferior left pulmonary veins. (Arch = aortic arch; LA = left atrium; RA = right atrium; SVC = superior vena cava; IVC = inferior vena cava; PA = pulmonary artery; PV = pulmonary vein.)

 

   Comment
Top
 Abstract
 Introduction
 Comment
References
 
It is important to obtain both a rapid and a correct diagnosis of an acute dissecting or rupturing aneurysm of the thoracic aorta during the initial examinations. Stanson and associates recently proposed a synonymous vascular disease of the thoracic aorta that they designated "penetrating atheromatous ulcer" [1]. According to their designation, PAU is characterized by ulceration of an atheromatous plaque that disrupts the internal elastic lamina of the aorta [1]. The prognosis of PAU is extremely poor when transmural rupture and subsequent hemorrhage develop progressively towards the mediastinum, the pericardium, or both [1, 2]. The correct initial diagnosis and immediate appropriate management of PAU are essential. It is often very difficult for attending physicians to make a correct diagnosis of PAU because the pathophysiology and the procedure for the diagnosis of PAU have not been fully established, and the number of case reports in the literature is small [3, 4].

Cooke and associates suggested that PAU originates from the descending thoracic aorta [2], but there are few case reports of PAU in the ascending thoracic aorta [5]. It is often difficult to estimate the precise location and the size of PAU in the ascending thoracic aorta, in particular when the lesion is less than 10 mm in diameter and when CT, aortography, or both fail adequately to reveal the extravascular leakage of contrast medium. It is also difficult to evaluate the appropriate initial management of PAU because opinions differ as to whether patients with PAU should be treated medically or surgically. Some authors insist that surgery is essential for symptomatic patients with PAU [1, 2]. Others insist that conservative treatment should be recommended for patients with slowly progressive PAU [6]. Unfortunately, there are no accepted guidelines for the management of patients with PAU and, in particular, for the decision to perform surgery.

Interstitial pulmonary hemorrhage (IPH) is an extremely rare condition that can be produced by mediastinal hemorrhage from the rupture of the ascending or descending thoracic aortas [7]. Mediastinal hemorrhage can dissect the broncho-vascular sheaths, producing enlarged broncho-vascular markings on chest CT [7]. We suspected that the heterogeneous dilatation of the posterior mediastinum and IPH in our patient were the result of rupture at the thoracic aorta. However, it was quite difficult to specify the point of aortic rupture because repeated examination of chest CT failed to demonstrate the leakage of contrast medium. Moreover, the presence of IPH itself does not necessarily indicate the specific point at which aortic rupture has occurred. Nevertheless, at autopsy, our case demonstrated clearly the three-dimensional consequences of the rupture of PAU in the ascending aorta. Therefore, it appears that the possibility of rupture of PAU in the ascending thoracic aorta is very high when mediastinal dilatation, IPH, and pericardial effusion are obtained as triplet imaging findings, even when the site of the rupture is not adequately defined and the patient's hemodynamic condition is stable. If this hypothesis is correct, the triplet imaging findings themselves might be a guide that suggests the importance of surgery for the initial management of some patients with PAU. Moreover, the use of reconstructed CT images or magnetic resonance images or both might further clarify the three-dimensional structure of PAU as a diagnostic procedure.


   References
Top
 Abstract
 Introduction
 Comment
 References
 

  1. Stanson AW, Kazmier FJ, Hollier LH, et al. Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinicopathologic correlations. Ann Vasc Surg. 1986;1:15–23[Medline]
  2. Cooke JP, Kazmier FJ, Orszulak TA. The penetrating aortic ulcer: pathologic manifestations, diagnosis, and management. Mayo Clin Proc. 1988;63:715–718
  3. Ganaha F, Miller DC, Sugimoto K, et al. Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. Circulation. 2002;106:342–348[Abstract/Free Full Text]
  4. Tittle SL, Lynch RJ, Cole PE, et al. Midterm follow-up of penetrating ulcer and intramural hematoma of the aorta. J Thorac Cardiovasc Surg. 2002;123:1051–1059[Abstract/Free Full Text]
  5. Yano K, Makino N, Hirayama H, et al. Penetrating atherosclerotic ulcer at the proximal aorta complicated with cardiac tamponade and aortic valve regurgitation. Jpn Circ J. 1999;63:228–230[Medline]
  6. Harris JA, Bis KG, Glover JL, Bendick PJ, Shetty A, Brown W. Penetrating atherosclerotic ulcers of the aorta. J Vasc Surg. 1994;19:90–99[Medline]
  7. Panicek DM, Ewing DK, Markarian B. Interstitial pulmonary hemorrhage from mediastinal hematoma secondary to aortic rupture. Radiology. 1987;162:165–166[Abstract/Free Full Text]



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