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Table of Contents
Year : 2019  |  Volume : 9  |  Issue : 4  |  Page : 237-239

Penetrating aortic root ulcer presenting as angina

1 Department of Cardiology, Fortis Hospital, Bengaluru, Karnataka, India
2 Department of Cardiothoracic and Vascular Surgery, Fortis Hospital, Bengaluru, Karnataka, India

Date of Web Publication11-Mar-2020

Correspondence Address:
Dr. Deepak Kadeli
Department of Cardiology, Fortis Hospital, Cunningham Road, Bengaluru - 560 052, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JICC.JICC_17_19

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We present a case of 58 year old female with acute aortic syndrome in a female presenting as angina. Acute aortic syndrome is a constellation of aortic pathologies which have been grouped together as they present in a similar manner. Penetrating aortic ulcer (PAU) and intramural hematoma (IMH) of aorta which were earlier considered to be part of the spectrum of aortic dissection are now considered to be acute aortic syndromes.

Keywords: Bentall procedure, intramural hematoma, penetrating aortic ulcer

How to cite this article:
Kadeli D, Keshava R, Reddy S, Gopi A. Penetrating aortic root ulcer presenting as angina. J Indian coll cardiol 2019;9:237-9

How to cite this URL:
Kadeli D, Keshava R, Reddy S, Gopi A. Penetrating aortic root ulcer presenting as angina. J Indian coll cardiol [serial online] 2019 [cited 2020 Apr 6];9:237-9. Available from: http://www.joicc.org/text.asp?2019/9/4/237/280342

  Introduction Top

Penetrating aortic ulcer (PAU) and intramural hematoma (IMH) of aorta are now distinct entities from aortic dissection and are considered under “acute aortic syndromes.”[1] Their earliest description has given by Krukenberg[2] who described IMH as dissection without flap as early as 1920. Rupture of an atheromatous plaque through the internal elastic lamina is thought to be the cause of penetrating ulcer.[3] Hemorrhage from vasa vasorum is believed to be the cause for IMH which is a localized collection of blood in the media of the aorta.

These entities do not have intimal flaps or false lumens, whereas penetrating ulcers may be visualized during conventional angiography. IMHs are not usually visualized with this study.

  Case Report Top

A 58-year-old female presented with left-sided chest pain. Electrocardiogram showed few ventricular premature beats. Troponin t was 51 pg/ml; two-dimensional echocardiography (echo) done showed moderate aortic regurgitation and normal left ventricular function with no regional wall abnormalities, but an indentation was noted in the root of aorta on transesophageal echo [Figure 1], and no flap was seen. Coronary angiogram done revealed normal coronaries. In view of the echo finding in the root of aorta, an aortogram was done which revealed an ulcerated plaque [Figure 2]. Computed tomography (CT) aortogram revealed ulceration of the aorta above the left coronary cusp with IMH [Figure 3]. Cardiothoracic team assessed the patient and decided for a composite graft replacement of aortic valve, aortic root, and ascending aorta with reimplantation of coronary arteries into the graft. She refused surgery and was discharged after explaining the risk. The patient had recurrence of symptoms after 2 weeks and was readmitted. Repeat CT aortogram done revealed no major extension of the plaque [Figure 4]. The family was explained about the nature of the disease and the urgent need for surgery. After consent, a successful Bentall procedure with 19-mm St. Judes mechanical valve conduit was done [Figure 5]. The decision to replace the aortic valve was done inspite of having only moderate aortic regurgitation, as the risk of a redo surgery in future, if aortic regurgitation worsens will be prohibitively high. She recovered over the course of hospital stay and was discharged on the 5th day, but was readmitted with sternotomy pain after a week. She was given analgesics and discharged the same day. The patient has been on regular follow-up and is symptom free.
Figure 1: Original transesophageal echo reveals translucent area in the aortic wall suggestive of intramural hematoma. No dissection flap seen

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Figure 2: A still from aortic angiogram showing the ulcer at the outer margin of the aortic root

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Figure 3: (a) Intramural hematoma resembling a false lumen seen in dissection (b) The penetrating ulcer protruding along the outer edge of the aortic root

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Figure 4: Serial computed tomography sagittal section showing the extent of the intramural hematoma from the aortic root up till the subclavian artery branch

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Figure 5: (a) Clot in the intraintimal layer of the penetrating ulcer at the aortic root (b) Graft in situ

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  Discussion Top

With advances in the field of imaging, more and more cases of PAU and IMH of the aorta are being recognized. They have characteristic pathophysiology distinct from typical dissections. Intimal flap which defines dissection is classically absent in these cases. PAU and IMH of the aorta appear to involve considerable risk of early rupture and concomitant late death.

The risk of early rupture is higher than that for typical aortic dissection. There is a female predominance. Tittle et al. reported rupture in 38% of penetrating ulcers and 26% of IMH at initial presentation.[4]

Unlike with typical dissection, vascular complications are not known to occur with PAU and IMH. The high rates of rupture at presentation, the frequency of worsening on serial radiographic follow-up, and the continued incidence of death from rupture despite medical management attest to the virulence of these lesions.

Stanson et al. first described penetrating ulcers and intra-aortic hematomas as high risk for rupture, warranting surgical correction irrespective of symptoms.[5] Coady et al. supported the same view with data suggesting that PAU is an even more malignant course than classic dissection.[6]

Hussain et al.[7] advocated nonoperative management in many cases which had successful course. Tittle et al. recommend immediate operation for patients with realized or impending rupture on radiographic or clinical grounds and semi-elective repair for more patients with more stable lesions. These recommendations are intended for patients with acutely presenting penetrating ulcers and IMHs and not for patients in whom such lesions are found on incidental or routine imaging studies.

Baikoussis et al.[8] in their review on acute aortic syndromes had proposed an algorithm for the management of acute aortic syndromes, which suggests different therapeutic strategies used to treat IMH. Acute aortic syndrome is a dynamic process wherein events can occur very rapidly, so emphasis should be made on detailed diagnostic confirmation with subsequent treatment by either surgical repair or interventional stent–graft placement.[9],[10] Our case had persistent pain and hence emergency surgically correction was done.

With increasing experience, more and more cases are being treated with elective surgery. Intra-aortic stenting is being studied as a possible less invasive method to treat suitable lesions which are limited in longitudinal extent. As these ulcers are highly variable in their clinical courses and the natural history of disease is not well established, true benefit from stenting is difficult to analyze.

Biologic glues have evoked interest for the treatment of IMHs, but clinical experience is lacking. If it can be done with minimally invasive techniques, it can find a wider acceptance among surgeons.

  Conclusions Top

IMH and PAU are diverse aortic diseases with different epidemiology and pathophysiology but strictly associated with each other. Although IMH originates in an acute mode, PAU is a chronic disease that can develop rapidly, both with unpredictable natural courses.

The management of IMH is similar to that of classic aortic dissection, with open surgery for the treatment of ascending aortic involvement and thoracic endovascular aortic/aneurysm repair and/or medical therapy for those with only descending localization, based on a complication-specific approach.

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 Román JA. Acute aortic syndrome. Heart 2001;85:365-8.  Back to cited text no. 1
Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR. Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery. Tex Heart Inst J 2003;30:325-7.  Back to cited text no. 2
Lui RC, Menkis AH, McKenzie FN. Aortic dissection without intimal rupture: Diagnosis and management. Ann Thorac Surg 1992;53:886-8.  Back to cited text no. 3
Tittle SL, Lynch RJ, Cole PE, Singh HS, Rizzo JA, Kopf GS, et al. Midterm follow-up of penetrating ulcer and intramural hematoma of the aorta. J Thorac Cardiovasc Surg 2002;123:1051-9.  Back to cited text no. 4
Stanson AW, Kazmier FJ, Hollier LH, Edwards WD, Pairolero PC, Sheedy PF, et al. Penetrating atherosclerotic ulcers of the thoracic aorta: Natural history and clinicopathologic correlations. Ann Vasc Surg 1986;1:15-23.  Back to cited text no. 5
Coady MA, Rizzo JA, Hammond GL, Pierce JG, Kopf GS, Elefteriades JA. Penetrating ulcer of the thoracic aorta: What is it? How do we recognize it? How do we manage it? J Vasc Surg 1998;27:1006-15.  Back to cited text no. 6
Hussain S, Glover JL, Bree R, Bendick PJ. Penetrating atherosclerotic ulcers of the thoracic aorta. J Vasc Surg 1989;9:710-7.  Back to cited text no. 7
Baikoussis NG, Apostolakis EE, Siminelakis SN, Georgios SP, John G. Intramural hematoma of the thoracic aorta: who's to be alerted the cardiologist or the cardiac surgeon? J Cardiothorac Surg 2009;4:54.  Back to cited text no. 8
Kaji S, Akasaka T, Horibata Y, Nishigami K, Shono H, Katayama M, et al. Long-term prognosis of patients with type a aortic intramural hematoma. Circulation 2002;106:I248-52.  Back to cited text no. 9
Nishigami K, Tsuchiya T, Shono H, Horibata Y, Honda T. Disappearance of aortic intramural hematoma and its significance to the prognosis. Circulation 2000;102:III243-7.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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