IMAGING OF AN ASCENDING AORTIC ANEURYSM

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MAY 2012 IMAGING OF AN ASCENDING AORTIC ANEURYSM, National and Kapodistrian University of Athens

Outline Definition and epidemiology of ascending aortic aneurysm Anatomy of the thoracic aorta and classification Etiology and pathogenesis of ascending aortic aneurysm Clinical presentation and complications of ascending aortic aneurysm Different modalities for the diagnosis and evaluation of ascending aortic aneurysm Companion Patient #1 : CXRs DDx of prominent aorta or aortic arch Index Patient : History and findings Companion Patient #2 : on transthoracic echocardiography Transthoracic echocardiography and transesophageal echocardiography imaging of ascending aortic aneurysm Index Patient : MRA Imaging Index Patient : CTA Imaging Comparison between different modalities Treatment References Acknowledgements 1

Definition and epidemiology of ascending aortic aneurysm Localized dilatation of the aorta 50% over the normal diameter Includes all three layers of the vessel (intima, media, adventitia) Ascending aortic aneurysms arise anywhere from the aortic valve to the innominate artery Incidence 3.6-6 cases per 100.000 pt. years Males - 2x-4x more commonly than females Woo Y Joseph., Mohler R. Emile, (Jan 29,2009).Clinical features and diagnosis of thoracic aortic aneurysm. Uptodate. Retrieved May 11, 2012, from http://www.uptodate.com/contents/clinical-features-and-diagnosis-of-thoracic-aortic 2

60% Anatomy of the thoracic aorta and classification 10% 40% 10% Aneurysm of the thoracic aorta can be classified into four anatomical categories Ascending aortic Aortic arch Descending aortic Thoracoabdominal Aneurysms can be: Fusiform or Saccular Massachusetts General Hospital Thoracic Aortic Center Drawing of the thoracic aorta anatomy. The arrows show the percentage of the thoracic aneurysm that involves each anatomical segment. 3

Etiology and Pathogenesis of ascending aortic aneurysm Pathophysiology: Cystic medial degeneration Risk factors are: Aging Hypertension Atherosclerosis (infrequent cause). Smoking Bicuspid valve Inflammatory/infectious disorders (eg. giant cell arthritis, syphilitic aortitis) When occurs in young patients, think: Marfan syndrome Ehlers- Danlos syndrome Other familiar (eg. mutation in TGF beta receptor 2 gene) Takayasu arthritis : Young females/males Isselbacher Eric M. Thoracic and Abdominal Aortic Aneurysms. Circulation 2005, 111:816-828. 4

Clinical presentation and complications of ascending aortic aneurysm. Most often asymptomatic Heart failure due to aortic regurgitation Myocardial ischemia or MI Rare presentations due to mass effect: Hoarseness, hemidiaphragmatic paralysis Wheezing, cough, hemoptysis, dyspnea pneumonitis Dysphagia SVC syndrome Chest or back pain due to bone compression Thromboembolic episodes Complications: Dissection, leakage, rupture, acute aortic regurgitation Woo Y Joseph., Mohler R. Emile, (Jan 29,2009).Clinical features and diagnosis of thoracic aortic aneurysm. Uptodate. Retrieved May 11, 2012, from http://www.uptodate.com/contents/clinical-features-and-diagnosis-of-thoracic-aortic 5

Different modalities for the diagnosis and evaluation of the ascending aortic aneurysm Chest X-ray Echocardiography CTA MRA Conventional angiography seldom used our days 6

Companion patient #1 : CXR (PA view) Possible findings on a CXR suggesting an ascending aortic aneurysm can be: 1. Widening of the mediastinum as a result of the prominence of the ascending aorta. 2. Mass effect (e.g. deviation of the trachea) can be an indicator of an ascending aortic aneurysm. There is no such finding in this companion patients CXR. 7

Companion patient #1: CXR (Lateral view) This is the companion patient (#1) lateral chest X-ray. Our findings are: Normal aortic arch Normal distal ascending aorta Dilated proximal ascending aorta 8

DDx of prominent ascending aorta or aortic arch Congenital Aortic arch anomaly (e.g., double aortic arch, right aortic arch PDA Tetralogy Fallot Coarctation of aorta; pseudocoarctation Acquired Aneurysm of aorta Aortic regurgitation Aortic valve stenosis Aortitis (eg, syphilitic, giant cell, rheumatoid, Takayasu s) Atherosclerosis (tortuosity, elongation, unfolding, and/or dilatation of aorta) Hypertensive heart disease Medial degeneration of aorta (eg, Marfan S., Ehlers-Danlos S.) Mediastinal mass simulating large aorta (eg, lymphoma) Reeder M. M., Bradley G. W., Jr. (1993). Reeder and Felson s Gamuts in Radiology. (3 rd edition). New York: Springer-Verlag 9

Index Patient: History and findings 25 year old male presenting to the student clinic for a check up. PMH: No Findings: Hypertensive (SP 150 mmhg) Transthoracic Echocardiography was performed to evaluate for hypertrophic left ventricle. Findings: bicuspid valve and ascending aortic aneurysm. 10

Companion patient #2 : on transthoracic echocardiography This is a parasternal long-axis view in a companion patient (#2) showing a dilated aortic root and ascending aorta (white arrows). Ilenia Foffa, Pier Luigi Festa, Lamia Ait-Ali3, Annamaria Mazzone, Stefano Bevilacqua and Maria Grazia Andreassi http://www.cardiovascularultrasound.com/content/7/1/34 11

Transthoracic Echocardiography (TTE) and Transesophageal Echocardiography (TEE) imaging of ascending aortic aneurysm 2003 ACC/AHA guidelines : Echocardiography for the diagnosis 2006 ACC/AHA guidelines : CT or MRI for quantification of dilatation TTE Preferred procedure. Effective for imaging the aortic root (eg. In patients with Marfan syndrome), generally not be used for sizing thoracic aortic aneurysms. TEE Can visualize the entire thoracic aorta well, semi-invasive not favored for routine imaging. Aortic root or ascending aortic diameter > 4 cm and bicuspid aortic valve further evaluation (size morphology of root and ascending aorta) by echo, CT or MR yearly. So our index patient needs further CT or MR evaluation. Woo Y Joseph., Mohler R. Emile, (Jan 29,2009).Clinical features and diagnosis of thoracic aortic aneurysm. Uptodate. 12

Index Patient: MRA imaging (levels of sinus of Valsalva and main pulmonary artery Multiplanar T1 and T2 weighted MR images were acquired in order to evaluate the aortic root and ascending aorta dilatation. Axial view, C+, thoracic MRA Level of the sinuses of Valsalva Axial view, C+, thoracic MRA Level of the main pulmonary artery Aortic diameters 13

Axial view, C+, thoracic MRA Level of the right pulmonary artery Index Patient: MRA imaging (levels of right pulmonary artery and aortic arch) Axial view, C+, thoracic MRA Level of the aortic arch Aortic diameters 14

Index Patient: MRA imaging (Sagittal view) Sagittal view, C+,thoracic MRA 3D MR reformation of the aorta demonstrating a dilated ascending aorta. Measurement of the ascending aorta was taken at the level of the left pulmonary artery (42,9 mm). Another measurement was taken at the level of the aortic arch (23,8 mm). Findings: The aortic root and ascending thoracic aorta are dilated. 15

CTA images were then acquired to evaluate the aortic valve for calcification and the ascending aneurysm dimensions. Aortic valve has a bicuspid morphology with a tiny calcific speck. 3D reformation, C+, Thoracic CTA Index Patient: CTA imaging Axial view, C+, Thoracic CTA 16

Index Patient : CTA imaging 3D Reformations, Axial, Oblique views, C+, CTA Aortic valve level At the aortic valve level, a maximum diameter of 27,7 mm is measured in the oblique view (bottom right corner). 17

Index Patient : CTA imaging imaging 3D Reformations, Axial, Oblique views, C+, CTA Sinus of Valsalva level At the Sinus of Valsalva level, a maximum diameter of 52,3 mm is measured in the oblique view (bottom right corner). 18

Index Patient :CTA imaging Axial view, C+, CTA Sinus of Valsalva level At the Sinus of Valsalva level, a maximum diameter of 48.33 mm is measured in the axial view. Maximum aortic diameter (Valsalva l.) Bicuspid Valve Left Ventricle Outflow Track Left Atrium Descending aorta 19

Index Patient : CTA imaging 3D Reformations, Axial, Oblique views, C+, CTA Aortic root level At the aortic root level, a maximum diameter of 40,6 mm is measured in the oblique view (bottom right corner). 20

Index Patient : CTA imaging 3D Reformations, Axial, Oblique views, C+, CTA Ascending aorta; level of right pulmonary artery At the right pulmonary artery level, a maximum diameter of 39,4 mm is measured in the oblique view (bottom right corner). 21

MRA and CTA findings Maximum Diameter measurements in CTA and MRA (in mm). The upper normal limit of Intra-luminal AAOD, is 35.6 for males in age group 20 to 40.* Level of Aorta CTA MRA Sinus of Valsalva 52.3 51 Aortic root 40,6 42 (Asc.) Right pulmonary artery 39.4 43 Aortic arch - 24 (Des.) Right main pulmonary artery 23 - Bicuspid aortic valve with fusiform dilatation of the ascending aorta with a maximum changes appreciated in the sinus of Valsalva. *Song Shou Mao, MD, Nasir Ahmadi, MPH, Birju Shah, M.B.B.S, Daniel Beckmann, BS, Annie Chen, BS, Luan Ngo, BS, Ferdinand R Flores, BS, Yan lin Gao, MD, and Matthew J Budoff, M.D. 22

Comparison between different modalities TTE TEE CXR CTA MRA 1 st choice - - Suitable for emergency No contrast, no radiation exp. Non invasive No contrast, no radiation exp. Semiinvasive, low risk No contrast, exposure to radiation Contrast, exposure to radiation (renal failure?) Time consuming (x2 CTA) Contrast, no radiation exp. Non invasive Non invasive Non invasive Low cost Low cost Low cost Expensive Most expensive Only images aortic root and ascending aorta Provides additional info TTE Not diagnostic (64% sensitivity of wid. Med. Sign. thoracic dis.) Good in diagnosing and detecting dimensions (92% accuracy for all th.abs.) - - - Images thrombus and calcification better Good in diagnosing and detecting dimensions Images aortic root better 23

Medical Beta blocker Treatment Surgical: Index patient has a dilation of 51mm, one risk factor and a bicuspid valve, thus recommended for surgery Laplace law (T=p*r/2*t) T: Aortic wall tension P: Intraluminal (blood) pressure r: radius of aorta t: aortic wall thickness A decrease in blood pressure (b-blocker) decreases the aortic wall tension thus decreasing the aneurysms rate of growth (the vessel does not compensate by increasing its radius) and thus the possibility of dissection. Lavall Daniel, Schäfers Hans-Joachim, Böhm Michael, Laufs Ulrich http://www.aerzteblatt.de/int/archive/article?id=124325 24

References Reeder M. M., Bradley G. W., Jr. (1993). Reeder and Felson s Gamuts in Radiology. (3 rd edition). New York: Springer-Verlag. Isselbacher Eric M. Thoracic and Abdominal Aortic Aneurysms. Circulation 2005, 111:816-828. Guo D, Hasham S, Kuang S-Q, Vaughan CJ, Boerwinkle E, Chen H, Abuelo D, Dietz HC, Basson CT, Shete SS, Milewicz DM. Familial thoracic aortic aneurysms and dissections. Circulation. 2001;103: 2461 2468. Woo Y Joseph., Mohler R. Emile, (Jan 29,2009).Clinical features and diagnosis of thoracic aortic aneurysm. Uptodate. Retrieved May 11, 2012, from http://www.uptodate.com/contents/clinicalfeatures-and-diagnosis-of-thoracic-aortic aneurysm?source=search_result&search=aortic+aneurysm&selectedtitle=2%7e150 Miller, WT. Thoracic Aortic Aneurysms: Plain Film Findings. Seminars in Roentgenology 2001 Oct; 36(4): 288-294. Song Shou Mao, MD, Nasir Ahmadi, MPH, Birju Shah, M.B.B.S, Daniel Beckmann, BS, Annie Chen, BS, Luan Ngo, BS, Ferdinand R Flores, BS, Yan lin Gao, MD, and Matthew J Budoff, M.D. Normal Thoracic Aorta Diameter on Cardiac Computed Tomography in Healthy Asymptomatic Adult; Impact of Age and Gender. Acad Radiol. 2008 July ; 15(7): 827 834 Lavall Daniel, Schäfers Hans-Joachim, Böhm Michael, Laufs Ulrich. Aneurysms of the Ascending Aorta Dtsch Arztebl Int. 2012 March; 109(13): 227 233 25

Acknowledgements Gillian Lieberman, MD Emmanouel Grigoriou Guangzu Gao Gagandeep Singh Ashton Lehmann Hailu Tilahun Claire Odom 26