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Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome
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ARTICLE IN PRESS IHJ Cardiovascular Case Reports (CVCR) xxx (2017) xxx–xxx
Contents lists available at ScienceDirect
IHJ Cardiovascular Case Reports (CVCR) journal homepage: www.elsevier.com/locate/ihjccr
Case report
Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome Sonali Avinash Inamdar, Bijulal Sasidharan ∗ , Deepa S. Kumar, Jayadevan ER Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, 695011, India
a r t i c l e
i n f o
Article history: Received 7 December 2016 Received in revised form 18 June 2017 Accepted 14 August 2017 Available online xxx Keywords: Middle aortic syndrome Takayasu’s arteritis balloon angioplasty aortic dissection pseudoaneurysm
a b s t r a c t Balloon angioplasty was done successfully in a young girl with Takayasu’s arteritis (TA). Non flow limiting dissection was not stented. On 4th day of procedure, patient presented with obstructing dissection and pseudoaneurysm. Biomarkers of disease activity were negative. Transmural fibrosis in TA usually resists dissections in response to angioplasty but underlying inflammation can make the aorta vulnerable. Balloon angioplasty in TA needs to be done after a thorough search for persisting disease activity. © 2017 Published by Elsevier B.V. on behalf of Cardiological Society of India. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction Balloon angioplasty has emerged as an effective treatment for middle aortic syndrome secondary to Takayasu’s arteritis (TA) with low incidence of complications. In children and adolescents, stenting of the segment after angioplasty is done when the dissection is extensive. Non flow limiting dissections are usually left alone, as stenting is associated with reduced growth potential of the segment. Also, the need for re-dilation and stenting requires placement of large sized sheaths. Stenting of lower thoracic segments is associated with risk of spinal cord ischemia as well. We describe a complicated balloon angioplasty of the descending thoracic aorta (DTA) and abdominal aorta (AA) in a 10 year old girl with known TA. Case report A 10 year old girl was diagnosed as a case of TA (Type V) when she presented with heart failure and hypertension one year ago. The duration of her heart failure symptoms had been since two weeks. She also had a year long history of both lower limb (LL) claudication. She was found to have absent pulses in the left upper limb and both LL. Blood pressure recorded in the right upper limb was 140/90 mmHg. 2D echocardiogram revealed severe left ven-
∗ Corresponding author. E-mail address: [email protected] (B. Sasidharan).
tricular (LV) dysfunction with LV dimensions being 56 mm (end diastole)/50 mm (end systole), ejection fraction (EF) of 25% along with mild mitral regurgitation. Computed Tomography angiogram (CTA) done one year ago at the time of diagnosis, revealed long segment stenosis of the descending thoracic aorta (DTA), infrarenal abdominal aorta (AA), distal arch of aorta including the left subclavian artery. There was “double ring” sign on CTA post-contrast images suggestive of active inflammatory disease. (Fig. 1) Her erythrocyte sedimentation rate (ESR) and C- reactive protein (CRP) levels were raised. A possibility of myocarditis was considered as a cause of LV dysfunction leading to heart failure especially on the background of active inflammation, however her Troponin T levels were normal and her ECG was suggestive of left ventricular hypertrophy with repolarization abnormalities. She was treated with a combination of azathioprine and prednisolone as well as antihypertensives and heart failure therapy. Her inflammatory markers normalized after about 12 months later, however left ventricular dysfunction persisted. She subsequently underwent balloon angioplasty with an option of stenting only if strongly indicated. Aortography measured diameter of normal segment of DTA to be 12 mm and AA to be 7 mm (Fig. 2). Stenotic segments measured 4.63 and 4.1 mm respectively. There was pressure gradient of 30 mm Hg across the stenotic segment in DTA and 20 mm Hg across the segment in AA. A 7 mm diameter cutting balloon (Boston Scientific, USA) was used and both segments were dilated upto 5 atmosphere (atm). Both lesions yielded well. Pressure gradient across the DTA segment was still >20 mmHg. So this segment was dilated with 10 mm diameter balloon (Advance balloon, Cook Inc) at 4 atm. Pres-
http://dx.doi.org/10.1016/j.ihjccr.2017.08.003 2468-600X/© 2017 Published by Elsevier B.V. on behalf of Cardiological Society of India. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Inamdar SA, et al, Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome, IHJ Cardiovasc Case Rep. (2017), http://dx.doi.org/10.1016/j.ihjccr.2017.08.003
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ARTICLE IN PRESS S.A. Inamdar et al. / IHJ Cardiovascular Case Reports (CVCR) xxx (2017) xxx–xxx
she was managed by insertion of stents. (Fig. 3) A short covered stent (10 mm diameter) was placed across the pseudoaneurysm and a longer non covered stent was used to cover the extended dissection flap. She made complete clinical recovery and her renal function became normal.
Discussion
Fig. 1. Computed Tomography angiogram (CTA) post-contrast images done one year prior to balloon angioplasty: Enhancing wall thickening with “double ring sign” (arrowhead) depicting active inflammation is seen which involved distal arch of aorta, descending thoracic and abdominal aorta. The inner ring represents the poorly enhanced edematous intima whereas the enhanced outer ring corresponds to the media and adventitia.
sure gradient after balloon angioplasty was < 10 mm Hg. There was non-flow limiting dissection at this segment after angioplasty. It was not stented as the dissection was not extensive. Patient was started on aspirin, remained clinically stable and went home. On day four post-procedure she developed acute paraparesis (grade 3 weakness) with anuric renal failure (creatinine 5 mg/dl). Magnetic resonance angiography revealed aortic occlusion at the DTA level with pseudoaneurysm (26 × 15 mm) adjacent to the flap in the inferolateral aspect. Angiography confirmed the findings and
This case highlights the difference in the histology and thereby the behavior of the aorta to angioplasty in an inflammatory coarctation (such as in TA) in contrast to the usual non-inflammatory congenital (native) coarctation. Native coarctation of aorta consists of a thick intimal and medial ridge which protrudes posterolaterally into the lumen. There is associated medial degeneration in the form of mucoid extracellular matrix accumulation (MEMA) along with fragmentation of elastin fibres.1 Both features are substrates for late dissections and aneurysm formation after balloon angioplasty which is reportedly high. In case of inflammatory coarctation, there is panarteritis with edema, infiltrates and granulomatous giant cells in the acute phase. Late phase consists of chronic inflammation, transmural fibrosis and disruption of elastic fibres.2 The intima becomes thicker than media thereby obliterating the lumen. Persistent acute inflammation causes vessel dilatation and formation of aneurysm, but stenosis is more commonly seen in the chronic phase. Wall fibrosis makes the vessel less likely to dissect after balloon angioplasty but higher balloon inflation pressures and multiple and prolonged dilatations may be required for the lesion to yield. This high pressure dilation can result in aortic dissection and rupture.3 The transmural scarring can be resistant to plain balloon angioplasty
Fig. 2. Angiographic images of index procedure. Panel A: Diffuse tight stenosis of DTA. Panel B: Balloon expansion of the lesion. Panel C & D: Acute lumen gain with minimal residual stenosis, non flow limiting dissection flap on either side of DTA. (Black and white arrowheads) Note: The dissection flap on lateral aortic wall is in the direction of the blood flow (White arrowhead).
Fig. 3. Angiographic images of second procedure. Panel A: Pseudoaneurysm arising from the dissected segment over the lateral aortic wall with compromised distal aortic blood flow. Panel B: Result after implantation of multiple stents.
Please cite this article in press as: Inamdar SA, et al, Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome, IHJ Cardiovasc Case Rep. (2017), http://dx.doi.org/10.1016/j.ihjccr.2017.08.003
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ARTICLE IN PRESS S.A. Inamdar et al. / IHJ Cardiovascular Case Reports (CVCR) xxx (2017) xxx–xxx
as there is residual stenosis due to recoil. Hence cutting balloons are used to overcome the residual stenosis. Diffuse adjacent disease (versus discrete stenosis in native non-inflammatory coarctation), eccentric stenosis and calcification predict bad outcomes of balloon angioplasty in TA in the form of dissection and/or obstruction. In our patient, even though multiple sustained dilatations were given to the DTA after using a cutting balloon, the pressures used were not high (4 atm). Non flow limiting dissection extended to pseudoaneurysm over a short time span. When we reviewed the images, we observed that the dissection flap on the lateral aortic wall was downward looking, in the direction of aortic forward flow and the extension of dissection beyond the vessel media and the direction of flow would have resulted in rapid progression and formation of pseudoaneurysm. How much role vessel inflammation plays in the progression is also worth investigating. Commonly used inflammatory markers such as ESR and CRP were normal in our patient before procedure. However these are not perfect markers of disease activity in TA. Active disease can persist in the absence of elevated biomarkers. Several biomarkers have been proposed for measurement of disease activity like NF-alpha, IL-6, IL-18 and pentraxin-3 (PTX-3) levels.4 O’Connor et al have reported various imaging modalities that are useful for monitoring remission, relapse, and subclinical progression of disease as well as for demonstrating the effectiveness of therapy. They include CT and MR angiography and positron emission tomography (PET). Our patient had “double ring” sign on the CTA done one year ago during active disease, where inner ring represents the poorly enhanced swollen intima and the outer enhanced ring indicates active inflammation in media and adventitia.5 We hypothesize that the presence of lurking inflammation could have contributed to the rapid progression of minor dissection in this patient. CT angiography immediately before intervention would have probably given a clue of persisting inflammation despite the negative biomarkers, which was not done in our case. A few cases of aortic rupture following angioplasty of TA have been described previously as reported by Mehta et al, wherein the majority of cases were re-do cases with a prior history of intervention either in the form of surgery or angioplasty. The index case (with negative inflammatory markers) described by Mehta et al was an aortic rupture that occurred on table at the time of first
3
intervention. In our case too, the complication occurred after the first interventional procedure; albeit four days later in the form of obstructing dissection and pseudoaneurysm presenting as paraparesis and acute renal shutdown. Conclusion Rapid progression of minor dissection occurring after balloon angioplasty is possible in inflammatory aortoarteritis, leading to aortic occlusion. Disclosures The authors have no competing interests, funding or financial relationships to disclose. References [1]. Halushka MK, Angelini A, Bartoloni G, et al. Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association For European Cardiovascular Pathology: II. Noninflammatory degenerative diseases — nomenclature and diagnostic criteria. Cardiovasc Pathol. 2016;25(May-June (3)):247–257. [2]. Stone JR, Bruneval P, Angelini A, et al. Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases. Cardiovasc Pathol. 2015;24(September-October (5)):267–278. [3]. Mehta V, Pandit BN, Yusuf J, et al. Aortic rupture during aortoplasty in Takayasu arteritis −A rare complication: Case report and review of literature. Indian Heart J. 2014 May;66(3):350–354. [4]. O’Connor TE, Carpenter HE, Bidari S, et al. Role of inflammatory markers in Takayasu arteritis disease monitoring. BMC Neurol. 2014;14(1):62. Mar 28. [5]. Zhu FP, Luo S, Wang ZJ, et al. Takayasu arteritis: imaging spectrum at multidetector CT angiography. Br J Radiol. 2012;85(December (1020)):e1282–92.
Please cite this article in press as: Inamdar SA, et al, Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome, IHJ Cardiovasc Case Rep. (2017), http://dx.doi.org/10.1016/j.ihjccr.2017.08.003
ARTICLE IN PRESS IHJ Cardiovascular Case Reports (CVCR) xxx (2017) xxx–xxx
Contents lists available at ScienceDirect
IHJ Cardiovascular Case Reports (CVCR) journal homepage: www.elsevier.com/locate/ihjccr
Case report
Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome Sonali Avinash Inamdar, Bijulal Sasidharan ∗ , Deepa S. Kumar, Jayadevan ER Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, 695011, India
a r t i c l e
i n f o
Article history: Received 7 December 2016 Received in revised form 18 June 2017 Accepted 14 August 2017 Available online xxx Keywords: Middle aortic syndrome Takayasu’s arteritis balloon angioplasty aortic dissection pseudoaneurysm
a b s t r a c t Balloon angioplasty was done successfully in a young girl with Takayasu’s arteritis (TA). Non flow limiting dissection was not stented. On 4th day of procedure, patient presented with obstructing dissection and pseudoaneurysm. Biomarkers of disease activity were negative. Transmural fibrosis in TA usually resists dissections in response to angioplasty but underlying inflammation can make the aorta vulnerable. Balloon angioplasty in TA needs to be done after a thorough search for persisting disease activity. © 2017 Published by Elsevier B.V. on behalf of Cardiological Society of India. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction Balloon angioplasty has emerged as an effective treatment for middle aortic syndrome secondary to Takayasu’s arteritis (TA) with low incidence of complications. In children and adolescents, stenting of the segment after angioplasty is done when the dissection is extensive. Non flow limiting dissections are usually left alone, as stenting is associated with reduced growth potential of the segment. Also, the need for re-dilation and stenting requires placement of large sized sheaths. Stenting of lower thoracic segments is associated with risk of spinal cord ischemia as well. We describe a complicated balloon angioplasty of the descending thoracic aorta (DTA) and abdominal aorta (AA) in a 10 year old girl with known TA. Case report A 10 year old girl was diagnosed as a case of TA (Type V) when she presented with heart failure and hypertension one year ago. The duration of her heart failure symptoms had been since two weeks. She also had a year long history of both lower limb (LL) claudication. She was found to have absent pulses in the left upper limb and both LL. Blood pressure recorded in the right upper limb was 140/90 mmHg. 2D echocardiogram revealed severe left ven-
∗ Corresponding author. E-mail address: [email protected] (B. Sasidharan).
tricular (LV) dysfunction with LV dimensions being 56 mm (end diastole)/50 mm (end systole), ejection fraction (EF) of 25% along with mild mitral regurgitation. Computed Tomography angiogram (CTA) done one year ago at the time of diagnosis, revealed long segment stenosis of the descending thoracic aorta (DTA), infrarenal abdominal aorta (AA), distal arch of aorta including the left subclavian artery. There was “double ring” sign on CTA post-contrast images suggestive of active inflammatory disease. (Fig. 1) Her erythrocyte sedimentation rate (ESR) and C- reactive protein (CRP) levels were raised. A possibility of myocarditis was considered as a cause of LV dysfunction leading to heart failure especially on the background of active inflammation, however her Troponin T levels were normal and her ECG was suggestive of left ventricular hypertrophy with repolarization abnormalities. She was treated with a combination of azathioprine and prednisolone as well as antihypertensives and heart failure therapy. Her inflammatory markers normalized after about 12 months later, however left ventricular dysfunction persisted. She subsequently underwent balloon angioplasty with an option of stenting only if strongly indicated. Aortography measured diameter of normal segment of DTA to be 12 mm and AA to be 7 mm (Fig. 2). Stenotic segments measured 4.63 and 4.1 mm respectively. There was pressure gradient of 30 mm Hg across the stenotic segment in DTA and 20 mm Hg across the segment in AA. A 7 mm diameter cutting balloon (Boston Scientific, USA) was used and both segments were dilated upto 5 atmosphere (atm). Both lesions yielded well. Pressure gradient across the DTA segment was still >20 mmHg. So this segment was dilated with 10 mm diameter balloon (Advance balloon, Cook Inc) at 4 atm. Pres-
http://dx.doi.org/10.1016/j.ihjccr.2017.08.003 2468-600X/© 2017 Published by Elsevier B.V. on behalf of Cardiological Society of India. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/).
Please cite this article in press as: Inamdar SA, et al, Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome, IHJ Cardiovasc Case Rep. (2017), http://dx.doi.org/10.1016/j.ihjccr.2017.08.003
G Model IHJCCR-37; No. of Pages 3 2
ARTICLE IN PRESS S.A. Inamdar et al. / IHJ Cardiovascular Case Reports (CVCR) xxx (2017) xxx–xxx
she was managed by insertion of stents. (Fig. 3) A short covered stent (10 mm diameter) was placed across the pseudoaneurysm and a longer non covered stent was used to cover the extended dissection flap. She made complete clinical recovery and her renal function became normal.
Discussion
Fig. 1. Computed Tomography angiogram (CTA) post-contrast images done one year prior to balloon angioplasty: Enhancing wall thickening with “double ring sign” (arrowhead) depicting active inflammation is seen which involved distal arch of aorta, descending thoracic and abdominal aorta. The inner ring represents the poorly enhanced edematous intima whereas the enhanced outer ring corresponds to the media and adventitia.
sure gradient after balloon angioplasty was < 10 mm Hg. There was non-flow limiting dissection at this segment after angioplasty. It was not stented as the dissection was not extensive. Patient was started on aspirin, remained clinically stable and went home. On day four post-procedure she developed acute paraparesis (grade 3 weakness) with anuric renal failure (creatinine 5 mg/dl). Magnetic resonance angiography revealed aortic occlusion at the DTA level with pseudoaneurysm (26 × 15 mm) adjacent to the flap in the inferolateral aspect. Angiography confirmed the findings and
This case highlights the difference in the histology and thereby the behavior of the aorta to angioplasty in an inflammatory coarctation (such as in TA) in contrast to the usual non-inflammatory congenital (native) coarctation. Native coarctation of aorta consists of a thick intimal and medial ridge which protrudes posterolaterally into the lumen. There is associated medial degeneration in the form of mucoid extracellular matrix accumulation (MEMA) along with fragmentation of elastin fibres.1 Both features are substrates for late dissections and aneurysm formation after balloon angioplasty which is reportedly high. In case of inflammatory coarctation, there is panarteritis with edema, infiltrates and granulomatous giant cells in the acute phase. Late phase consists of chronic inflammation, transmural fibrosis and disruption of elastic fibres.2 The intima becomes thicker than media thereby obliterating the lumen. Persistent acute inflammation causes vessel dilatation and formation of aneurysm, but stenosis is more commonly seen in the chronic phase. Wall fibrosis makes the vessel less likely to dissect after balloon angioplasty but higher balloon inflation pressures and multiple and prolonged dilatations may be required for the lesion to yield. This high pressure dilation can result in aortic dissection and rupture.3 The transmural scarring can be resistant to plain balloon angioplasty
Fig. 2. Angiographic images of index procedure. Panel A: Diffuse tight stenosis of DTA. Panel B: Balloon expansion of the lesion. Panel C & D: Acute lumen gain with minimal residual stenosis, non flow limiting dissection flap on either side of DTA. (Black and white arrowheads) Note: The dissection flap on lateral aortic wall is in the direction of the blood flow (White arrowhead).
Fig. 3. Angiographic images of second procedure. Panel A: Pseudoaneurysm arising from the dissected segment over the lateral aortic wall with compromised distal aortic blood flow. Panel B: Result after implantation of multiple stents.
Please cite this article in press as: Inamdar SA, et al, Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome, IHJ Cardiovasc Case Rep. (2017), http://dx.doi.org/10.1016/j.ihjccr.2017.08.003
G Model IHJCCR-37; No. of Pages 3
ARTICLE IN PRESS S.A. Inamdar et al. / IHJ Cardiovascular Case Reports (CVCR) xxx (2017) xxx–xxx
as there is residual stenosis due to recoil. Hence cutting balloons are used to overcome the residual stenosis. Diffuse adjacent disease (versus discrete stenosis in native non-inflammatory coarctation), eccentric stenosis and calcification predict bad outcomes of balloon angioplasty in TA in the form of dissection and/or obstruction. In our patient, even though multiple sustained dilatations were given to the DTA after using a cutting balloon, the pressures used were not high (4 atm). Non flow limiting dissection extended to pseudoaneurysm over a short time span. When we reviewed the images, we observed that the dissection flap on the lateral aortic wall was downward looking, in the direction of aortic forward flow and the extension of dissection beyond the vessel media and the direction of flow would have resulted in rapid progression and formation of pseudoaneurysm. How much role vessel inflammation plays in the progression is also worth investigating. Commonly used inflammatory markers such as ESR and CRP were normal in our patient before procedure. However these are not perfect markers of disease activity in TA. Active disease can persist in the absence of elevated biomarkers. Several biomarkers have been proposed for measurement of disease activity like NF-alpha, IL-6, IL-18 and pentraxin-3 (PTX-3) levels.4 O’Connor et al have reported various imaging modalities that are useful for monitoring remission, relapse, and subclinical progression of disease as well as for demonstrating the effectiveness of therapy. They include CT and MR angiography and positron emission tomography (PET). Our patient had “double ring” sign on the CTA done one year ago during active disease, where inner ring represents the poorly enhanced swollen intima and the outer enhanced ring indicates active inflammation in media and adventitia.5 We hypothesize that the presence of lurking inflammation could have contributed to the rapid progression of minor dissection in this patient. CT angiography immediately before intervention would have probably given a clue of persisting inflammation despite the negative biomarkers, which was not done in our case. A few cases of aortic rupture following angioplasty of TA have been described previously as reported by Mehta et al, wherein the majority of cases were re-do cases with a prior history of intervention either in the form of surgery or angioplasty. The index case (with negative inflammatory markers) described by Mehta et al was an aortic rupture that occurred on table at the time of first
3
intervention. In our case too, the complication occurred after the first interventional procedure; albeit four days later in the form of obstructing dissection and pseudoaneurysm presenting as paraparesis and acute renal shutdown. Conclusion Rapid progression of minor dissection occurring after balloon angioplasty is possible in inflammatory aortoarteritis, leading to aortic occlusion. Disclosures The authors have no competing interests, funding or financial relationships to disclose. References [1]. Halushka MK, Angelini A, Bartoloni G, et al. Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association For European Cardiovascular Pathology: II. Noninflammatory degenerative diseases — nomenclature and diagnostic criteria. Cardiovasc Pathol. 2016;25(May-June (3)):247–257. [2]. Stone JR, Bruneval P, Angelini A, et al. Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases. Cardiovasc Pathol. 2015;24(September-October (5)):267–278. [3]. Mehta V, Pandit BN, Yusuf J, et al. Aortic rupture during aortoplasty in Takayasu arteritis −A rare complication: Case report and review of literature. Indian Heart J. 2014 May;66(3):350–354. [4]. O’Connor TE, Carpenter HE, Bidari S, et al. Role of inflammatory markers in Takayasu arteritis disease monitoring. BMC Neurol. 2014;14(1):62. Mar 28. [5]. Zhu FP, Luo S, Wang ZJ, et al. Takayasu arteritis: imaging spectrum at multidetector CT angiography. Br J Radiol. 2012;85(December (1020)):e1282–92.
Please cite this article in press as: Inamdar SA, et al, Aortic occlusion after balloon angioplasty in inflammatory middle aortic syndrome, IHJ Cardiovasc Case Rep. (2017), http://dx.doi.org/10.1016/j.ihjccr.2017.08.003