Table of Contents  
Year : 2022  |  Volume : 9  |  Issue : 4  |  Page : 331-334

A wolf in wolf's clothing? The complexities of decision involved with acute thoracic endovascular aortic intervention for penetrating aortic ulcer and intramural hematoma

1 Vascular Department, Royal North Shore Hospital, NSW, Australia; Senior Lecturer, Medicine, Sydney University, NSW, Australia
2 Vascular Department, Royal North Shore Hospital, NSW, Australia

Date of Submission31-Jan-2022
Date of Acceptance14-Mar-2022
Date of Web Publication8-Nov-2022

Correspondence Address:
Animesh Singla
Vascular Department, Royal North Shore Hospital, NSW, Australia; Senior Lecturer, Medicine, Sydney University, NSW
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijves.ijves_8_22

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This article provides a case and reviews the considerations involved in acute endovascular stent for penetrating aortic ulcers (PAU)/intramural hematoma (IMH). This case aims to highlight the decision-making and considerations in the acute endovascular management of a patient presenting with PAU and associated IMH. The literature was reviewed including Google Scholar, PubMed, and Embase. Acute aortic syndrome encompasses a spectrum of aortic disease ranging from aortic dissection to PAU and IMH. Whilst classical aortic dissection has been well studied, the PAU/IMH subset of this population is exceedingly rare. Only a handful of case reports have reported on technical considerations for repair. Despite limited natural history data, growing evidence suggests a more aggressive stance is warranted to prevent progression to rupture and late aortic-related mortality. This pathology poses unique challenges in anatomical and technical considerations of repair.

Keywords: Acute aortic syndrome, intramural hematoma, thoracic endovascular repair

How to cite this article:
Singla A, Cook V, Kotecha K, Mohabbat W. A wolf in wolf's clothing? The complexities of decision involved with acute thoracic endovascular aortic intervention for penetrating aortic ulcer and intramural hematoma. Indian J Vasc Endovasc Surg 2022;9:331-4

How to cite this URL:
Singla A, Cook V, Kotecha K, Mohabbat W. A wolf in wolf's clothing? The complexities of decision involved with acute thoracic endovascular aortic intervention for penetrating aortic ulcer and intramural hematoma. Indian J Vasc Endovasc Surg [serial online] 2022 [cited 2022 Nov 28];9:331-4. Available from:

  Introduction Top

Thoracic endovascular aortic intervention (TEVAR) has had an evolving role in the management of acute aortic syndrome.[1] Penetrating aortic ulcers (PAU) and intramural hematomas (IMH) represent a subset of this syndrome with limited data on natural history. There is some experience suggesting acute intervention for this population of patient is warranted due to a higher rate of progression.[2] However, there is controversy with respect to several aspects in acute endovascular management of these conditions.[3] The case highlights the complexity of planning and treatment with TEVAR of PAU and associated IMH including proximal sealing zone selection, appropriate choice, and sizing of a thoracic endograft.

  Case Report Top

A 74-year-old Korean male presented to a peripheral hospital with 2-day history of persistent mid-lower back pain radiating around to the abdomen. It was sudden onset whilst playing golf and was described as a tight, band-like pain. His past medical history included coronary artery bypass grafting (with left internal mammary to left anterior descending), hypertension, and hypercholesterolemia. He was a current smoker, with 60-pack-year history. He was discharged <24 h prior after a presumed diagnosis of renal colic due to the presence of microscopic hematuria on urine analysis.

On readmission, he underwent computed tomography scanning which identified a large PAU in the proximal descending thoracic aorta (Ishimaru zone 3). There were several fenestrations in the descending thoracic aorta and an IMH extending the entire length of the descending thoracic and proximally to Ishimaru zone 2 [Figure 1].[4] The patient was immediately commenced on anti-impulse therapy (systolic blood pressure <120 mmHg and heart rate <60 mmHg). Despite this, he had refractory back pain and progression of his IMH on follow-up imaging 48 h postadmission.
Figure 1: Preoperative view of extent of intramural hematoma involvement from Ishumuru zone 2 to celiac axis

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Decision was made to perform endovascular aneurysm repair using covered self-expanding expanded polytetrafluoroethylene thoracic stents (C-Tag, Gore). This was performed under general anesthetic with percutaneous access and percutaneous suture mediate closure (ProGlide device, Abbott). Two C-Tag conformable devices 28 mm × 150 mm and 31 mm × 200 mm grafts were placed extending from proximal celiac axis to the level distal to the left subclavian artery [Figure 2]. His symptoms resolved immediately postoperatively. His postoperative course involved medical management of subsequent worsening heart failure, idiopathic hepatitis suspected to be related to antibiotics, and in-patient co-rehab. He was cleared for discharge 3 weeks postadmission to home.
Figure 2: Multiplanar reformation and three-dimensional volumetric reconstructions of postthoracic endovascular aortic intervention Computed tomography angiogram indication good stent apposition

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  Discussion Top

PAU and IMH represent entities within the spectrum of acute aortic syndrome. The natural history of these has been poorly defined and limited to case reports, series, and single-institution reviews.[5] PAU is thought to arise from ulcerated atherosclerotic lesions which penetrate the internal elastic lamina into the media, whilst IMH refers to bleeding within the medial layer.[6],[7] There is growing international literature that IMH with PAU, which were once thought benign, have higher rate of progression to complication than classical Type B aortic dissection.[8],[9]

Whilst the mainstay of therapy remains anti-impulse therapy to control the forceful ejection forces and limited propagation of disease, endovascular therapy has been playing an increasing role. However, the presence of PAU, particularly in the presence of IMH, raises several dilemmas in planning TEVAR management.

Size of endograft

Normally, for thoracic endografting, sizing recommendations vary from nominal 1:1 sizing in cases of traumatic injury, to up to 15% oversizing for sufficient seal to avoid endoleak in aneurysmal disease.[10] There is no agreed guidance for the appropriate measurement of luminal diameter for sizing a thoracic endograft in the setting of IMH.[11] Whilst normal atherosclerotic plaque/thrombus may narrow the true lumen of the vessel, the presence of IMH may act in a different manner. As IMH represents an acute process, some or complete resolution of IMH diameter may occur. As a result, it may be considered reasonable to include the IMH component within the luminal diameter sizing. Conversely, this can lead to oversizing, and risk of extension of the IMH proximally or distally.[11],[12] There is some data on averaging the luminal diameter by including 50% of IMH thickness in the luminal measurement. The aim of our patient was to achieve adequate coverage of PAU to seal the suspected leak site causing the IMH. Thus, we included the majority of the IMH diameter in our measurements to reduce the risk of subsequent migration of the thoracic stent, acknowledging a small but balanced risk that any oversizing may have propagated a retrograde dissection.

Proximal seal zone

Classically, international standards (Society for Vascular Surgery guidelines, ESVES guidelines) recommend landing within 2 cm of healthy aorta proximally for all endovascular treatment of thoracic aortic pathologies.[1],[13],[14] This often will involve landing in zone 2 or more proximally within the aortic arch. This often necessitates either extra-anatomic debranching of supra-aortic vessels or fenestrated/branched technology. Our patient had a PAU within zone 3 with IMH extending into zone 2 of the aortic arch. It was a type 3 aortic arch with significant angulation and a bovine configuration. Aortic wall involved in the IMH has been previously shown to be friable and unhealthy, with higher risk of complications such as Type A retrograde dissection.[15] As such, it has been advocated to perform seals more proximally and debranch the aorta appropriately.[16] Kuo et al. analyzed this question of sealing within IMH in a series of 71 patients treated with TEVAR. They found that the majority of their patients (68%) were sealed in the aortic segment affected by IMH, and that nearly 90% of cases would require some form of debranching, with nearly half requiring total aortic arch debranching through a sternotomy.[15] In our patient, this would have necessitated a carotid-subclavian bypass and landing in proximal portion of zone 2, just distal to bovine origin of left common carotid. Given our patient's comorbidities and primary aim of sealing entry PAU which was in the descending thoracic aorta, it was felt a proximal seal just distal to the left subclavian achieved an acceptable compromise. As this patient had a dominant bypass graft through the left internal mammary artery (LIMA), the hemodynamic changes related to the coronary bypass during subclavian artery clamping could risk cardiac arrhythmia/or destabilize a poorly reserved myocardium. Despite the documented concern regarding retrograde dissection by landing TEVAR stent within IMH, with incidence of 1%–5%, overall aortic remodeling appears to be similar to case in which more proximal seal is achieved.[17] Perhaps, of more importance is the choice of stent used and avoiding adjunctive procedures such as coda ballooning to avoid any undue pressure on already friable aortic tissue.

Another aspect of proximal seal is the degree of arch angulation, determined in part of aortic arch configuration, and exact nature of thoracic aortic pathology. Our patient had a type 3 aortic arch suggesting, a likely severe angle, combined with the presence of bovine configuration leant the patient's anatomy to a long zone 2 sealing site. However, as this would have required coverage of LIMA reliant coronary graft, without revascularization, coverage of the left subclavian origin would not be possible. As we had decided not to perform revascularization, the question arose whether to land proximal or distal to the angulation of the aortic arch. The main disadvantage of inadequate landing proximal to the aortic angulation is risk of bird-beaking and subsequent graft infolding with inadequate inferior aortic arch coverage.[18] However, the use of the conformable C-Tag (Gore) was used to offset this intraoperatively. This was achieved by the use of active control to allow the stent graft to concertina to take the natural aortic angle, combined with the placement of stiff wire against outer curvature of aortic arch. This may represent a unique anatomy for which the C-Tag (Gore) conformable device is ideally suited over other stainless steel stent grafts.

Choice of stent graft

There has been some conflicting data in the literature regarding the use of bare versus covered stents in the proximal seal zone. One meta-analysis did not find any relationship between risk of retrograde Type A dissection and use of bare metal versus covered proximal stents.[13] However, a more recent review of literature actually found a ×2 increased relative risk of extension with the use of bare metal stent in the proximal stent graft configuration. Due to concern of landing in potentially friable, and damaged aortic tissue, we avoided the use of any bare metal stent grafts. Theoretically, the dynamic movement of the cardiac cycle may interact negatively with the rigid stent design, predisposing to retrograde dissection. This risk may increase with more proximal extension of sealing zones beyond zone 2 of the aortic arch.[12]

  Conclusion Top

This case report highlights the complexity of decision-making for acute endovascular management of PAU for the thoracic aorta. This involves consideration of hemodynamic forces, aortic arch anatomy, and measured consideration in deciding the degree of surgical “invasiveness.” As this technology and our understanding of the pathology continues to evolve, we must continuously challenge the decision-making dictum to determine the optimal outcome for these patients.

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

Riambau V, Böckler D, Brunkwall J, Cao P, Chiesa R, Coppi G, et al. Editor's choice – Management of descending thoracic aorta diseases: Clinical practice guidelines of the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg 2017;53:4-52.  Back to cited text no. 1
Eggebrecht H, Plicht B, Kahlert P, Erbel R. Intramural hematoma and penetrating ulcers: Indications to endovascular treatment. Eur J Vasc Endovasc Surg 2009;38:659-65.  Back to cited text no. 2
Eagle KA, Bossone E. Intramural hematoma: When does a sheep become a wolf? J Am Coll Cardiol 2017;69:40-2.  Back to cited text no. 3
Fillinger MF, Greenberg RK, McKinsey JF, Chaikof EL. Reporting standards for thoracic endovascular aortic repair (TEVAR). J Vasc Surg 2010;52:1022-33.e15.  Back to cited text no. 4
Evangelista A, Czerny M, Nienaber C, Schepens M, Rousseau H, Cao P, et al. Interdisciplinary expert consensus on management of type B intramural haematoma and penetrating aortic ulcer. Eur J Cardiothorac Surg 2015;47:209-17.  Back to cited text no. 5
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. 6
Erbel R. Diseases of the thoracic aorta. Heart 2001;86:227-34.  Back to cited text no. 7
Vilacosta I, Aragoncillo P, Cañadas V, San Román JA, Ferreirós J, Rodríguez E. Acute aortic syndrome: A new look at an old conundrum. Postgrad Med J 2010;86:52-61.  Back to cited text no. 8
Evangelista A, Mukherjee D, Mehta RH, O'Gara PT, Fattori R, Cooper JV, et al. Acute intramural hematoma of the aorta: A mystery in evolution. Circulation 2005;111:1063-70.  Back to cited text no. 9
Lee WA, Matsumura JS, Mitchell RS, Farber MA, Greenberg RK, Azizzadeh A, et al. Endovascular repair of traumatic thoracic aortic injury: Clinical practice guidelines of the Society for Vascular Surgery. J Vasc Surg 2011;53:187-92.  Back to cited text no. 10
Chen Y, Zhang S, Liu L, Lu Q, Zhang T, Jing Z. Retrograde type A aortic dissection after thoracic endovascular aortic repair: A systematic review and meta-analysis. J Am Heart Assoc 2017;6:e004649.  Back to cited text no. 11
Canaud L, Ozdemir BA, Patterson BO, Holt PJ, Loftus IM, Thompson MM. Retrograde aortic dissection after thoracic endovascular aortic repair. Ann Surg 2014;260:389-95.  Back to cited text no. 12
Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr., et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation 2010;121:e266-369.  Back to cited text no. 13
Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J 2014;35:2873-926.  Back to cited text no. 14
Kuo EC, Veranyan N, Johnson CE, Weaver FA, Ham SW, Rowe VL, et al. Impact of proximal seal zone length and intramural hematoma on clinical outcomes and aortic remodeling after thoracic endovascular aortic repair for aortic dissections. J Vasc Surg 2019;69:987-95.  Back to cited text no. 15
Patel HJ, Sood V, Williams DM, Dasika NL, Diener AC, Deeb GM. Late outcomes with repair of penetrating thoracic aortic ulcers: The merits of an endovascular approach. Ann Thorac Surg 2012;94:516-22.  Back to cited text no. 16
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  [Figure 1], [Figure 2]


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