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Year : 2021  |  Volume : 64  |  Issue : 1  |  Page : 152-154
Spontaneous aortic rupture: Report of two cases with review of literature

1 Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
2 Undergraduate Student, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
3 Forensic Medicine and Toxicology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Date of Submission14-Apr-2020
Date of Decision27-Apr-2020
Date of Acceptance08-Jun-2020
Date of Web Publication8-Jan-2021


Acute aortic syndromes comprise a heterogeneous group of life-threatening disorders, characterized by acute-onset chest or back pain with or without cardiovascular collapse. The major entities include acute aortic dissection, penetrating atherosclerotic ulcer, and intramural hematoma. Spontaneous rupture of the aorta is an additional, rare cause of this syndrome, which frequently leads to sudden cardiac death. This is a report of two cases of this uncommon entity with detailed pathological analysis.

Keywords: Acute aortic syndrome, aortic rupture, sudden cardiac death

How to cite this article:
Vaideeswar P, Kundu S, Singaravel S, Tyagi S. Spontaneous aortic rupture: Report of two cases with review of literature. Indian J Pathol Microbiol 2021;64:152-4

How to cite this URL:
Vaideeswar P, Kundu S, Singaravel S, Tyagi S. Spontaneous aortic rupture: Report of two cases with review of literature. Indian J Pathol Microbiol [serial online] 2021 [cited 2022 Jan 19];64:152-4. Available from: https://www.ijpmonline.org/text.asp?2021/64/1/152/306513

   Introduction Top

Acute aortic syndrome (AAS) was a term coined by Vilacosta et al.,[1] to encompass a heterogeneous group of life-threatening disorders that share a common clinical profile (acute-onset chest or back pain with or without cardiovascular collapse), mediated by alterations in the aortic wall. Acute aortic dissection (AAD), penetrating atherosclerotic ulcer (PAU), and intramural hematoma (IMH) are the major entities included under AAS, which have distinct demographic, pathological, and survival characteristics.[2] Spontaneous rupture of the thoracic aorta is an additional, but rare cause of AAS and sudden cardiac death.[3] Though it is said to occur in the absence of trauma, dissection, or aneurysm, the term “spontaneous aortic rupture” (SAR) is often used loosely. With this report of two cases of spontaneous rupture of the ascending aorta with detailed pathological analysis, we sought to clarify the clinicopathologic features of this uncommon entity.

   Case Reports Top

Case 1

A 70-year-old lady was brought in an unconscious state and expired within 30 min of admission. She had been a hypertensive on regular therapy (details of medications not known) and developed sudden onset retrosternal chest pain radiating to the left hand with shortness of breath and sweating, 10 min before becoming unconscious. A complete autopsy was performed. The pericardial cavity was distended with blood and blood clots (260 g). The heart was normal in size (270 g). Hemorrhage was present in the adventitia and periadventitial soft tissue of both great arteries. The aortic root and the ascending aorta were dilated; the valve cusps were normal. The circumferences measured at the cuspal attachment, sinutubular junction, and mid-portion of the ascending aorta were 3 cm, 3.2 cm, and 4.5 cm, respectively. There was a circumferential tear, seen in relation to an uncomplicated atherosclerotic plaque, present 4.2 cm above the sinutubular junction and 0.5 cm proximal to the origin of the right brachiocephalic artery [Figure 1]a. A section at this site revealed that the tear had involved both the intimal and the media (unlike the intimal tear alone in AAD) and the proximal and distal segments were held together by the congested appearing adventitia. A large hematoma lay between the media and adventitia on the anterior aspect and had ruptured into the pericardial cavity. A small localized dissection was seen as an adjunctive finding in the ascending aorta; no dissection was detected in the transverse and descending segments. The aortic histology showed only age-related aortopathy [Figure 2], characterized by focal intralamellar mucoid extracellular matrix accumulation; however, there was no fragmentation or loss of elastic fibers, loss of smooth muscle nuclei, or laminar medial collapse. The coronary arteries showed minimal atherosclerotic changes. The left ventricular myocardium did not show microscopic features of hypertrophy. Focal areas of scarring were noted in the kidneys.
Figure 1: (a) Case 1 – Opened out left ventricular (LV) outflow tract showing a circumferential transaction of the ascending aortic (AA) wall with periadventitial hemorrhage; (b) Case 2 – In this case, there was a rupture occupying nearly three-fourths of the circumference of the ascending aorta (AA), descending thoracic aorta (DTA), left atrium (LA), left coronary cusp (LCC), left common carotid artery (LCCA), left subclavian artery (LSA), mitral valve (MV), noncoronary cusp (NCC), right coronary cusp (RCC), right common carotid artery (RCCA), right subclavian artery (RSA)

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Figure 2: (a) Scanned slide showing a tear * that has synchronously involved the intima I and the media M with localized dissection (H and E, A adventitia, PT pulmonary trunk) (b) The aorta at the rupture site *showed focal increases in the basophilic ground substance (arrow, H and E × 250)

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Case 2

A 68-year-old woman, a known case of hypertension with chronic kidney disease, had been admitted to a private healthcare facility for vomiting and diarrhea followed by oliguria. During the course of the treatment, she developed shortness of breath for which she was transferred to our tertiary healthcare center. Immediately after admission, she became acutely breathless and was then unconscious. At autopsy, the pericardial cavity was filled with nearly 300 cc of serosanguineous fluid and contained 400 g of blood clots. The adventitia of the ascending aorta and adjoining pulmonary trunk was hemorrhagic with small hematomas, particularly seen on the posterior aspects. These features were a consequence of a synchronous break of the intima and media of a dilated ascending aorta [Figure 1]b. It measured 5 cm in length and was present 5 cm above the aortic annulus. The circumferences at the annulus, sinutubular junction, and midascending aorta were 2.8 cm, 3.2 cm, and 4.2 cm, respectively. The aortic valve cusps were normal. The aorta showed moderate atherosclerosis, but the tear did not appear to be related to any plaque complication. There was also mild aortopathy. Other findings included moderate left ventricular hypertrophy (heart weight 390 g with increased sizes of the myocytes and accompanying nuclear hyperchromatism), critical stenosis of the left anterior descending artery, esophageal retention cysts, and benign nephrosclerosis.

   Discussion Top

Hemopericardium and resultant cardiac tamponade occurred in two elderly hypertensive women due to an almost identical pathology. There had been a tear, through and through, involving both the intima and media. We used the term SAR for this pathology. This characteristic pathological feature of a transmural laceration helps to differentiate SAR from the more common causes of AAS. AAD is caused by an intimal tear, which permits the entry of blood and splitting of the media to create a true and a false lumen for a variable length.[4] (MH (a controversial entity) represents localized aortic wall dissection without detectable intimal tear and absence of flow in the “false lumen” while PAU refers to a destructive extension of an ulcerated atheroma into the media to variable depths, culminating in a hematoma formation.[4] It is evident that all the three entities do not show synchronous disruption of the intima and media.

The term SAR is not clearly defined and hence in the several reviews in the past for such ruptures in different aortic segments, there have been a variable number of patients as well as etiologic factors, to the extent of even ruling out subclinical intimal or medial disease. We feel that SAR should be defined by the following criteria: 1. Nontraumatic disruption of the nonaneurysmal aorta when pathologies within AAS in the form of AAD, IMH, and PAU have been ruled out by appropriate imaging modalities and/or histopathological studies of the surgically excised segments or at autopsy and 2. Simultaneous rupture involving both intima and media. Though ruptures with normal aortic histomorphology truly exist, hypertension and atherosclerosis stand out as the underlying basis for most cases,[3],[5] and hence most of the patients are elderly. Both aging and hypertension can lead to aortopathy.[6],[7] Other risk factors include aortopathy and aortitis caused by different systemic or localized diseases.

Both of our patients were in their sixth decades of life with long-standing hypertension; diffuse atherosclerosis was seen at autopsy in one of them. The ascending aorta was the site of rupture, and this can be explained by the elevated hemodynamic stress generated by hypertension. Since the first reported case on SAR by Castleman et al. in 1970,[8] there has been a dearth in the number of reported cases as well as an available source of information on this topic and hence the exact incidence of this condition is not known. In our review of literature for the past 10 years, we could find only 21 cases (including two of our cases) have been reported;[9],[10],[11],[12] only one of the seven cases reported by Bin Mahmood et al.[12] satisfied the criteria for SAR. Age and sex were not available in three of the reported cases.[9],[12] Among the remaining 18 patients, there were 11 males and 7 females with an age range of 47–76 years and a mean age of 62 years. Five each of these patients did not have any apparent risk factors[9],[10],[11] or revealed aortopathy,[9] respectively. The rest of the patients had hypertension and/or atherosclerosis,[9],[12] as seen in two of our index cases. The presentation in all was like AAS. Almost half of these patients had a sudden death. Due to the nonspecific clinical presentation and overlap with other entities, clinical suspicion is crucial for the diagnosis of these cases for which computed tomography is the recommended modality. Furthermore, an endovascular approach is more suitable than open-heart surgery. Hence, it would be important to keep SAR in the differential diagnoses in patients presenting with chest pain and cardiovascular collapse so that timely and emergency intervention can be deployed to ensure better chances of survival. But, it must be borne in mind that rapid onset, misdiagnosis, and unpredictability could be the basic contributing features for the poor outcome of the disease. Since the diagnosis may elude even the most experienced and knowledgeable clinicians, missing the diagnosis does not necessarily represent negligent medical care. At the same time, the clinical findings, differential diagnosis, investigations, treatment plan, and follow-up instructions should be documented scrupulously.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Vilacosta I, San Roman JA. Acute aortic syndrome. Heart 2001;85:365-88.  Back to cited text no. 1
Morris JH, Mix D, Cameron SJ. Acute aortic syndromes: Update in current medical management. Curr Treat Options Cardiovasc Med 2017;19:29.  Back to cited text no. 2
Yokoyama H, Ohmi M, Sadahiro M, Shoji Y, Tabayashi K, Moizumi Y. Spontaneous rupture of the thoracic aorta. Ann Thorac Surg 2000;70:683-89.  Back to cited text no. 3
Corvera JS. Acute aortic syndrome. Ann Cardiothorac Surg 2016;5:188-93.  Back to cited text no. 4
Kasahara H, Hachiya T, Mori A. Emergency endografting for spontaneous thoracic aortic rupture. Ann Thorac Cardiovasc Surg 2018. doi: 10.5761/atcs.cr. 18-00020.  Back to cited text no. 5
Fritze O, Romero B, Schleicher M, Jacob MP, Oh DY, Starcher B, et al. Age-related changes in the elastic tissue of the human aorta. J Vasc Res 2012;49:77-86.  Back to cited text no. 6
Homme JL, Aubry MC, Edwards WD, Bagniewski SM, Shane PV, Kral CA, et al. Surgical pathology of the ascending aorta: A clinicopathologic study of 513 cases. Am J Surg Pathol 2006;30:1159-68.  Back to cited text no. 7
Castlemann B, McNeely BU. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 43-1970. N Engl J Med 1970;283:862-70.  Back to cited text no. 8
Hirai S, Hamanaka Y, Mitsui N, Morifuji K, Uegami S. Spontaneous rupture of the ascending thoracic aorta resulting in a mimicking pseudoaneurysm. Ann Thorac Cardiovasc Surg 2006;12:223-7.  Back to cited text no. 9
Brizzio ME, Zapolanski A, Kesselbrenner M. Spontaneous rupture of a nonaneurismatic ascending thoracic aorta. J Card Surg 2009;24:548-50.  Back to cited text no. 10
Highbloom RY, Schwartz YY, Rosenbloom M, Bowen FW, Datwani? N. Nontraumatic ascending aortic disruption. Ann Thorac Surg 2013;96:1480-1.  Back to cited text no. 11
Bin Mahmood SU, Ulrich A, Safdar B, Geirsson R, Mangi AA. Spontaneous rupture of the ascending aorta. J Card Surg 2018;33:107-14.  Back to cited text no. 12

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular & Thoracic Division), Seth GS Medical College, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_382_20

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