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Heart, Lung and Circulation (2014) 23, e139–e141 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2013.12.011
Type A Aortic Dissection Secondary to Ruptured Penetrating Ascending Aortic Ulcer in an Immunosuppressed Patient Vinesh Appadurai, MBBSa,b*, Ahmad Al-Hindawi, MBBSc, Paul Leschke, MBBSc, Kim Greaves, MD, FACC, FRCPa,b a
Department of Cardiology, Nambour General Hospital, Hospital Road, Nambour, Queensland 4560 School of Medicine, The University of Queensland, St Lucia, Queensland 4072 Department of Radiology, Nambour General Hospital, Hospital Road, Nambour, Queensland 4560
b c
Received 26 October 2013; received in revised form 15 December 2013; accepted 29 December 2013; online published-ahead-of-print 22 January 2014
Keywords
Cardiac Imaging Penetrating Aortic Ulcer Cardiac tamponade Aortic Dissection Vascular Intervention
A 79 year-old female presented after a syncopal episode on a background of 12 hours of lethargy, dry cough and severe left shoulder pain. A liver transplant was performed 20 years ago for primary biliary cirrhosis with a daily immunosuppressive regimen of Prednisolone, Cyclosporine and Azathioprine. Further history included hypertension and osteoarthritis. On physical examination the patient was hypotensive and required high flow oxygen to maintain oxygen saturations above 95%. General clinical examination was unremarkable except for a delayed response to verbal commands. Laboratory investigations revealed an elevated white cell count of 16.5109/L and reduced glomerular filtration rate of 57 ml/min/1.73m2, otherwise all other values were within normal limits. Contrast enhanced computed tomography (CT) of the chest and abdomen revealed three penetrating aortic ulcers of varying size along the ascending and descending aorta (Figs. 1–3) complicated by type A dissection and haemopericardium along the central bronchovascular structures with no evidence of dissection into the pleural space. Transthoracic echocardiography showed a dissection flap as well as a large pericardial effusion and right ventricular tamponade. The left ventricle was compressed with normal systolic function. Due to the patient’s chronic immunosuppression and general frailty surgical intervention was not deemed suitable thus supportive therapy and
comfort measures were instituted. The patient died six days later. Penetrating Aortic Ulcers (PAU) are a well defined component of the Acute Aortic Syndrome (AAS) with prevalence amongst symptomatic AAS patients of approximately 2.3-7.6% [1]. There are atherosclerotic lesions which ulcerate through the internal elastic lamina resulting in haematoma formation within the media of the aortic wall [2]. There is a higher incidence of PAU in the mid to distal descending thoracic aorta and they are rarely observed in the ascending aorta [3]. Haematoma extension may then evolve into either a double-barrelled or thrombosed aortic dissection with the former demonstrating a communication between the true and false lumina while the latter shows no opacification of the false lumen [3]. Patients presenting with these ulcerative lesions typically have a multitude of pre-existing vascular comorbidities including a history of hypertension, atherosclerosis, smoking, and other cardiovascular risk factors. Progression to rupture has been well-documented in the literature at rates varying from 4.9% to 38% [2]. CT and magnetic resonance imaging (MRI) are currently the preferred investigative tools for detecting PAUs. A classical presentation on CT demonstrates focal out-pouching and adjacent sub-intimal haematoma formation of the aortic wall. Thickening and enhancement of the surrounding wall is also usually present [3]. MRI has been observed to be
* Corresponding author. Nambour General Hospital, Hospital Road, Nambour, Queensland, 4560. Mobile: 0410237011., Emails: [email protected], [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
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Figure 1 A & B: Transverse (a) and coronal (b) sections of contrast CT depicting mid-ascending aortic penetrating ulcer (*) directed towards the pulmonary trunk with haemopericardium (H) and extension along the bronchovascular structures.
Figure 2 A & B: Transverse (a) and coronal (b) sections of contrast CT depicting a large penetrating ulcer (*) at the level of the left subclavian artery directed inferolaterally with an associated haemopericardium (H). Small volume gas within the left brachiocephalic vein is iatrogenic, related to contrast injection.
Figure 3 A reconstructed four chamber view demonstrating a large volume haemopericardium (H) causing compression of the right ventricle (RV) and right atrium (RA).
superior to CT in detecting the extent of and differentiating intramural thrombus from atherosclerotic plaque and chronic intraluminal thrombus [4]. CT, however, retains a high clinical relevance due to faster examination times, ease of access, and the ability to demonstrate complex spatial relationships including mural abnormalities and extraluminal pathologic conditions [3]. The differential diagnosis may include a mycotic or infected aneurysm of the aorta due to its similar appearance on CT. This lesion typically appears as a saccular form aneurysm with nodularity, irregular configuration, or air in the aortic wall [5]. It clinically presents with a fever and severe back, abdominal or thoracic pain, depending on site of aneurysm [5]. Leucocytosis, elevated C-reactive protein and positive blood cultures may also be present. Major causative factors include systemic bacteraemia, infective endocarditis or aortitis with common infective organisms being Stapholococcus, Salmonella, Kleibsiella, Streptococcus and Campylobacter species [5,6]. The immunosuppression in this patient may have predisposed to an infective aneurysm however given the lack of any infective symptoms, positive blood cultures, histological evidence, and combined with the presence of atherosclerosis, this diagnosis is unlikely.
e141
Ruptured penetrating aortic ulcer in an immunosuppressed patient
Management of PAU is divided into conservative non-interventional and interventional approaches. Asymptomatic patients with an incidental finding of PAU typically receive aggressive management regarding their risk factors for arterial rupture with continued follow-up monitoring of the lesion. Symptomatic patients generally present with atypical chest pain or referred pain and usually undergo intervention by either endovascular stent-grafting for uncomplicated descending thoracic PAU, or surgical intervention for complicated ruptured PAU including those involving the ascending aorta and aortic arch [7,8]. Prior to this case, no randomised controlled trials have been performed but several series results indicate that stent-grafting success rates approach 100% with low morbidity and mortality in uncomplicated cases [9]. Comparatively, mortality rates in traditional open surgical techniques of the descending thoracic aorta vary from 5% to 20% [7].
Conflict of Interest None declared.
References [1] Roldan CJ. Penetrating atherosclerotic ulcerative disease of the aorta: Do emergency physicians need to worry? J Emerg Med 2012;43(1):196–203. [2] Nathan DP, Boonn W, Lai E, Wang GJ, Desai N, Woo EY, et al. Presentation, complications, and natural history of penetrating atherosclerotic ulcer disease. J Vasc Surg 2012;55(1):10–5. [3] Hayashi H, Matsuoka Y, Sakamoto I, Sueyoshi E, Okimoto T, Hayashi K, et al. Penetrating atherosclerotic ulcer of the aorta: Imaging features and Disease concepts. Radiographics 2000;20:995–1005. [4] Yucel EK, Steinberg FL, Egglin TK, Geller SC, Waltman AC, Athanasoulis CA. Penetrating aortic ulcers: diagnosis with MR imaging. Radiology 1990;177(3):779–81. [5] Ishizaka N, Sohmiya K, Miyamura M, Umeda T, Tsuji M, Katsumata T, et al. Infected aortic aneurysm and inflammatory aortic aneurysm – In search of an optimal differential diagnosis. J Card 2012;59:123–31. [6] Chen IM, Chang HH, Hsu C, Lai ST, Shih CC. Ten year experience with surgical repair of mycotic aortic aneurysms. J Chin Med Assoc 2005;68 (6):265–71. [7] Brinster DR, Wheatley GH, Williams J, Ramaiah VG, Diethrich EB, Rodriguez-Lopez JA. Are penetrating aortic ulcers best treated using an endovascular approach? Ann Thorac Surg 2006;82:1688–91. [8] Pauls S, Orend K-H, Sunder-Plassmann L, Kick J, Schelzig H. Endovascular repair of symptomatic penetrating atherosclerotic ulcer of the thoracic aorta. Eur J Vasc Endovasc Surg 2007;34:66–73. [9] Patatas K, Shrivastava V, Ettles DF. Penetrating atherosclerotic ulcer of the aorta: A continuing debate. Clin Rad 2013;68:753–9.
Heart, Lung and Circulation (2014) 23, e139–e141 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2013.12.011
Type A Aortic Dissection Secondary to Ruptured Penetrating Ascending Aortic Ulcer in an Immunosuppressed Patient Vinesh Appadurai, MBBSa,b*, Ahmad Al-Hindawi, MBBSc, Paul Leschke, MBBSc, Kim Greaves, MD, FACC, FRCPa,b a
Department of Cardiology, Nambour General Hospital, Hospital Road, Nambour, Queensland 4560 School of Medicine, The University of Queensland, St Lucia, Queensland 4072 Department of Radiology, Nambour General Hospital, Hospital Road, Nambour, Queensland 4560
b c
Received 26 October 2013; received in revised form 15 December 2013; accepted 29 December 2013; online published-ahead-of-print 22 January 2014
Keywords
Cardiac Imaging Penetrating Aortic Ulcer Cardiac tamponade Aortic Dissection Vascular Intervention
A 79 year-old female presented after a syncopal episode on a background of 12 hours of lethargy, dry cough and severe left shoulder pain. A liver transplant was performed 20 years ago for primary biliary cirrhosis with a daily immunosuppressive regimen of Prednisolone, Cyclosporine and Azathioprine. Further history included hypertension and osteoarthritis. On physical examination the patient was hypotensive and required high flow oxygen to maintain oxygen saturations above 95%. General clinical examination was unremarkable except for a delayed response to verbal commands. Laboratory investigations revealed an elevated white cell count of 16.5109/L and reduced glomerular filtration rate of 57 ml/min/1.73m2, otherwise all other values were within normal limits. Contrast enhanced computed tomography (CT) of the chest and abdomen revealed three penetrating aortic ulcers of varying size along the ascending and descending aorta (Figs. 1–3) complicated by type A dissection and haemopericardium along the central bronchovascular structures with no evidence of dissection into the pleural space. Transthoracic echocardiography showed a dissection flap as well as a large pericardial effusion and right ventricular tamponade. The left ventricle was compressed with normal systolic function. Due to the patient’s chronic immunosuppression and general frailty surgical intervention was not deemed suitable thus supportive therapy and
comfort measures were instituted. The patient died six days later. Penetrating Aortic Ulcers (PAU) are a well defined component of the Acute Aortic Syndrome (AAS) with prevalence amongst symptomatic AAS patients of approximately 2.3-7.6% [1]. There are atherosclerotic lesions which ulcerate through the internal elastic lamina resulting in haematoma formation within the media of the aortic wall [2]. There is a higher incidence of PAU in the mid to distal descending thoracic aorta and they are rarely observed in the ascending aorta [3]. Haematoma extension may then evolve into either a double-barrelled or thrombosed aortic dissection with the former demonstrating a communication between the true and false lumina while the latter shows no opacification of the false lumen [3]. Patients presenting with these ulcerative lesions typically have a multitude of pre-existing vascular comorbidities including a history of hypertension, atherosclerosis, smoking, and other cardiovascular risk factors. Progression to rupture has been well-documented in the literature at rates varying from 4.9% to 38% [2]. CT and magnetic resonance imaging (MRI) are currently the preferred investigative tools for detecting PAUs. A classical presentation on CT demonstrates focal out-pouching and adjacent sub-intimal haematoma formation of the aortic wall. Thickening and enhancement of the surrounding wall is also usually present [3]. MRI has been observed to be
* Corresponding author. Nambour General Hospital, Hospital Road, Nambour, Queensland, 4560. Mobile: 0410237011., Emails: [email protected], [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
e140
V. Appadurai et al.
Figure 1 A & B: Transverse (a) and coronal (b) sections of contrast CT depicting mid-ascending aortic penetrating ulcer (*) directed towards the pulmonary trunk with haemopericardium (H) and extension along the bronchovascular structures.
Figure 2 A & B: Transverse (a) and coronal (b) sections of contrast CT depicting a large penetrating ulcer (*) at the level of the left subclavian artery directed inferolaterally with an associated haemopericardium (H). Small volume gas within the left brachiocephalic vein is iatrogenic, related to contrast injection.
Figure 3 A reconstructed four chamber view demonstrating a large volume haemopericardium (H) causing compression of the right ventricle (RV) and right atrium (RA).
superior to CT in detecting the extent of and differentiating intramural thrombus from atherosclerotic plaque and chronic intraluminal thrombus [4]. CT, however, retains a high clinical relevance due to faster examination times, ease of access, and the ability to demonstrate complex spatial relationships including mural abnormalities and extraluminal pathologic conditions [3]. The differential diagnosis may include a mycotic or infected aneurysm of the aorta due to its similar appearance on CT. This lesion typically appears as a saccular form aneurysm with nodularity, irregular configuration, or air in the aortic wall [5]. It clinically presents with a fever and severe back, abdominal or thoracic pain, depending on site of aneurysm [5]. Leucocytosis, elevated C-reactive protein and positive blood cultures may also be present. Major causative factors include systemic bacteraemia, infective endocarditis or aortitis with common infective organisms being Stapholococcus, Salmonella, Kleibsiella, Streptococcus and Campylobacter species [5,6]. The immunosuppression in this patient may have predisposed to an infective aneurysm however given the lack of any infective symptoms, positive blood cultures, histological evidence, and combined with the presence of atherosclerosis, this diagnosis is unlikely.
e141
Ruptured penetrating aortic ulcer in an immunosuppressed patient
Management of PAU is divided into conservative non-interventional and interventional approaches. Asymptomatic patients with an incidental finding of PAU typically receive aggressive management regarding their risk factors for arterial rupture with continued follow-up monitoring of the lesion. Symptomatic patients generally present with atypical chest pain or referred pain and usually undergo intervention by either endovascular stent-grafting for uncomplicated descending thoracic PAU, or surgical intervention for complicated ruptured PAU including those involving the ascending aorta and aortic arch [7,8]. Prior to this case, no randomised controlled trials have been performed but several series results indicate that stent-grafting success rates approach 100% with low morbidity and mortality in uncomplicated cases [9]. Comparatively, mortality rates in traditional open surgical techniques of the descending thoracic aorta vary from 5% to 20% [7].
Conflict of Interest None declared.
References [1] Roldan CJ. Penetrating atherosclerotic ulcerative disease of the aorta: Do emergency physicians need to worry? J Emerg Med 2012;43(1):196–203. [2] Nathan DP, Boonn W, Lai E, Wang GJ, Desai N, Woo EY, et al. Presentation, complications, and natural history of penetrating atherosclerotic ulcer disease. J Vasc Surg 2012;55(1):10–5. [3] Hayashi H, Matsuoka Y, Sakamoto I, Sueyoshi E, Okimoto T, Hayashi K, et al. Penetrating atherosclerotic ulcer of the aorta: Imaging features and Disease concepts. Radiographics 2000;20:995–1005. [4] Yucel EK, Steinberg FL, Egglin TK, Geller SC, Waltman AC, Athanasoulis CA. Penetrating aortic ulcers: diagnosis with MR imaging. Radiology 1990;177(3):779–81. [5] Ishizaka N, Sohmiya K, Miyamura M, Umeda T, Tsuji M, Katsumata T, et al. Infected aortic aneurysm and inflammatory aortic aneurysm – In search of an optimal differential diagnosis. J Card 2012;59:123–31. [6] Chen IM, Chang HH, Hsu C, Lai ST, Shih CC. Ten year experience with surgical repair of mycotic aortic aneurysms. J Chin Med Assoc 2005;68 (6):265–71. [7] Brinster DR, Wheatley GH, Williams J, Ramaiah VG, Diethrich EB, Rodriguez-Lopez JA. Are penetrating aortic ulcers best treated using an endovascular approach? Ann Thorac Surg 2006;82:1688–91. [8] Pauls S, Orend K-H, Sunder-Plassmann L, Kick J, Schelzig H. Endovascular repair of symptomatic penetrating atherosclerotic ulcer of the thoracic aorta. Eur J Vasc Endovasc Surg 2007;34:66–73. [9] Patatas K, Shrivastava V, Ettles DF. Penetrating atherosclerotic ulcer of the aorta: A continuing debate. Clin Rad 2013;68:753–9.
