SHAPE Society

1. Aortic ulcer –
intramural hematoma-
aortic dissection:
a continuous spectrum
R Erbel, H Eggebrecht, D Baumgart, J Debatin
J Barkhausen,U Herold, H Jakob
Department of Cardiology Radiology and
Thoracic and Cardiovascular Surgery
University Essen, Germany

2. Classification of acute aortic
syndromes
Svensson LG et al.Circulation 99: 1331-6, 2000
1- Classic dissection
2- Intramural
hematoma
3- Discrete/subtitle
dissection
4- Plaque ulcer,
plaque rupture
5- Iatrogenic/traumatic
dissection
1 2 3
4 5
ESC TF Eur Heart J 22: 1642 81, 2001

3. History of IMH
• 1920 Krukenberg: Bleeding to the outer
layer of the media due to rupture
of vasa vasorum without tear.
• 1952 Gore,
• 1958 Hirst and 1982 Wilson: pathologic studies
• 1988 Yamada et al: 1st CT and MRI study
• 1991 Zotz et al: 1st IMH FU to AD by TEE
• 1994 Mohr-Kahaly: 1st TEE clinical study and FU
• 2000 v Kodolitsch et al: „Hemorrhagic stroke of the
aortic wall“

4. Cystic Media Necrosis
Collagen Fiber Rupture

5. Cystic Media Necrosis
Collagen Fiber Rupture and Intramural Hemorrhage

6. Desc. Aorta SAX at 35 cm
Intramural Hematoma Typ I
N = 17
X = 64 years
3 – 20cm length
0.7 – 3 cm W Th
35% echolucent zones
Mohr-Kahaly et al JACC 23:658 – 64, 1994

7. Intramural
Hematoma Type II
with Vessel Wall
Layering and
Shearing
N = 10
-Age 70 years
-Aortic ectasia,aneurysm
-Calcium displacement
-3 – 23 cm length
-0.7 – 4 cm W Th
- 70 % echolucent zones
Mohr-Kahaly et al JACC 23:658 – 64, 1994

8. - Hematoma formation within the aortic wall in the absence of a
detectable intimal tear (wall thickening)
- Due to spontaneous rupture of vasa vasorum
- Potential precursor of overt dissection class 1
- Class 2 aortic dissection
Intramural hematoma (IMH)
Erbel R, EHJ 2001
Vilacosta, Am Heart J 1997

9. - Displacement of intimal calcifications
- Affects long segment of the aorta
Intramural hematoma, Class 2 AD (IMH)
Differentiation against thrombosed aneurysm

10. Meta-Analysis1
(143 patients):
- 5-20% of patients with acute aortic syndromes
- 61% men, mean age 68 yrs.
- 53% hypertension
- Rare: traumatic (motor vehicle accident)
- 80% chest pain
- ~ 21% mortality
Intramural hematoma (IMH)
1
Maraj et al,, Am J Cardiol 2000

11. Outcome1
:
IMH- Outcome
1
Mara et al,, Am J Cardiol 2000

12. Intramural Hematoma
Aortography
IVUS
Class 2 AD type B
Intravascular Ultrasound

13. Pericardial tamponade, progression to dissection, rupture
within one week despite
RR control
IMH- Complications

14. History of PAU Reports
• 1935 Shennan T 4/218 cases AD begin
in the
base of AU
• 1941 Willius /Cragg „some of AD
accociated with
ulcerating
atheromatous
abscesses“Vilacosta et al JACC 32:83 – 9,1998

15. - Elderly, hypertensive patients
- Symptomatic vs. asymptomatic (incidental finding)
- Most common site: mid/distal descending thoracic aorta
- Strong association with concomitant abdominal aneurysm
Penetrating Atherosclerotic Ulcer (PAU)
Atheroma Plaque erosion
Intimal ulcer PAU+IMH Pseudoaneurysm Rupture
Von Kodolitsch, Z Kardiol 1998

16. - Ulceration of aortic atherosclerotic plaque penetrating
through the internal elastic lamina into the media
- Class 4 aortic dissection
- 2.3 - 7.6% in symptomatic patients with acute aortic
syndromes
Penetrating Atherosclerotic Ulcer (PAU)
CTIVUS
Erbel R, EHJ 2001

17. Plaque Rupture class 4 AD
Ao
Fibrous
cap
Ulcer
core
1 cm
Erbel R Heart 2001
IVUS
MRI Imaging

18. PAU- Complications
- Intramural hematoma :
• 10 – 100% 1,2
•due to erosion of vasa vasorum
• upredictor of adverse outcome
IMH
IMH
(Ganaha et a. Circulation 2002)
1. Vilacosta et al JACC 1998
2. Kazerooni et al Radiology 1992

19. Ruptured Plaque with Floating Fibrous Cap
Tear
Fibrous Cap
Ulcer

20. PAU- Complications
- Pseudoaneurysm : 0- 50%1,2
Growth rate: 0,31 cm/ year
1
Yucel, Radiology 1990
2
Harris, J Vasc Surg 1994
- Embolism: rare

21. PAU- Complications
- 0- 44%1,2
rupture
1
Stanson, Ann Vasc Surg 1986
2
Harris, J Vasc Surg 1994
3
Coady, J Vasc Surg 1998
- 40% for PAU vs. 3.6% for classic type B dissection3
- Risk factors : symptomatic patient, aortic diameter,
*
type-A PAU

22. Impending Perforation of Plaque
Rupture of descending Aorta
Pleura
effusion
Plaque-
rupture
Aortic sclerosis
class 4 AD

24. IMH with /without PAU
• Age/year 71 67
• Male/% 44 61
• Ao asc/% 9 26
• Ao desc/% 91 74
• WTH mm 16 _ 5 13 _ 4
• Stable 25% 91%
• Ao rupture 16% 4%
• Ao dissection 12% 4%
Pt group IMH with PAU without PAU
Ganaha et al Circulation 106:342 – 8, 2002

25. Indicators of Disease Progression
• Age/years 71 72
• Male/% 58 23
• Pain persistence/% 75 7
• Pl effusion /% 75 0
• PAU diameter/mm 21 12
• PAU depth /mm 14 7
• PAU number 1.2 1.5
• Ao diameter/mm 48 46
• WTh /mm 17 14
• IMH segments 3.3 3.9
Clinical Signs Progression Stable Course
Ganaha et al Circulation 106:342 – 8, 2002

30. Media Necrosis Erdheim Gsell Aortic Disease
Entry Tear
IMH Aortic dissection
class 2 AD
Aortic rupture
Healing
No continuity: PAU, IMH, dissection

31. Arteriosclerosis Progression
Stary IV – V Atherom, Fibroatherom
Plaque Rupture
Ulcer Hematoma Mural Thrombosis
VIa VIb VIc
Yes: PAU/ IMH/ Aortic Dissection
can be a continuity in atherosclerosis

32. Aortic Diseases
Aortic
rupture
Aortic Disease
-congenital
-degenerative
-arteriosclerotic
-inflammatory
-traumatc,toxic
Healing
Trauma
Class 5
Plaque rupture
Class4
Discrete/subtitle
Dissection
Class 3
Intramural
Haematoma
Haemorrhage
Class 2
Aortic dissection
Class 1
Communicating/no
n communicating
AD
ESC Task Force EHJ 2001

33. IMHwith PAU
MRI:
Contained rupture of the descending
thoracic aorta due to penetrating (PAU)
atherosclerotic ulcer (class IV type B) with
IMH

34. Arteriosclerosis and Aneurysm Formation
Preexisting atherosclerosis not required
-absence in animals
-Proteolytic activity different (MMPs)
-Disparity in characteristics of pts
Reed et al Circulation 85:205-11,1992

35. Characteristics of PAU Patients
No Sex Age Co morbidity Ao D Location FU
1 F 68 EH 4.4 IIIa IMH,R
2 M 65 EH,CABG 2.9 IIIa free
3 M 66 EH, 2-VD 1.9 IIIb free
4 F 75 EH, CABG 3.0 IIIa IMH,Pseu
5 M 71 EH, 1-VD 3.0 IIIa free
6 M 69 EH,AF 2.9 IIIa free
7 M 78 EH, 3-VD 2.8 IIIa IMH,R
8 M 72 CABG, PVD 3.9 Arch Pseudoan
9 M 72 EH 2.0 II IMH,>1PAU

36. PAU – Graft Stenting
• Stent diameter/mm 34 _ 7 24 – 46
• Stent length /mm 90 _17 60 – 130
• Fluoroscopy time
/min 12 _ 6 5 - 21
• Contrast material
/ml 244 _ 115 50 - 450
• Neurological deficit none
• Late FU 1/9 ex for renal stenosis
• Mortality 0
x _ s range

38. PAU References
• Stanson 86 16 81% 44% 44%
• Yucel 90 7 100% 14% 43%
• Kazeroni 92 16 81% 56% 19%
• Harris 94 18 22% - 6%
• Coady 98 15 80% 20% 27%
• Vilacosta 98 12 100% 17% 42%
• Hayoshi 00 12 - - 33%
• Quint 01 38 58% 16% -
x 134 66% 21% 20%
Author year N Sympt Rupture Surgery

39. PAU References
• Stanson 86 16 - - - 44%
• Yucel 90 7 - - 0% 0%
• Kazeroni 92 16 6% 11% - 31%
• Harris 94 18 - 0% 50% -
• Coady 98 15 20% 27% - -
• Vilacosta 98 12 17% 0% - 0%
• Hayoshi 00 12 17% 0% 0% 0%
• Quint 01 38 0% 0% 16% 16%
Author Year N Mortality Delayed Progress S/stent
Rupture to Aneury in FU

40. Prognosis of PAU
Total Type A Type B
Aortic dissection 16 % 57 % 12 %
Rupture 12 % 57 % 5 %
Stable without
surgery 54 % 0 % 75 %
Mortality surgery 13 % 0 % 13 %
med Th 26 % 100 % 11 %
total mortality 19 % 57% 14 %
v. Kodolitsch et al Z Kardiol 87:917 – 27,1998

41. Clinical Features of PAU
• Age > 65 years sex: M 60%
• 15 % Type A, Type B 85 %
• RF: EH 85 %, Smoking 72 %, HLP 35 %
• 85 % Single PAU, 4 % two, > 2 PAUs 11 %
• 73 % IMH
• 16 % AD, 4 % typical class 1AD
• 27 % Pseudoaneurysm
• 19 % Fusiforme Aneurysm
• 12% Rupture
v. Kodolitsch et al Z Kardiol 87:917 – 27,1998
93 References, nearly all case reports

42. FOLLOW UP IMH
Ascending aorta:
n= 3 1surgery
1ruptur
1 dissection
Descending aorta:
n=24 4 dissection
3 surgery
3 healing
6 death

43. Assessment of the true and false lumen
Ao desc 23 cm
1.19 cm

44. Visualisation of Intimal Tear
using 3D-Echocardiography

45. Non communicating dissection type B
38 cm
Aortic dissection classification

46. Morphology of False Lumen
WL
FL
WL
FL
WL
FL

47. Pitfalse
Artefacts
Explanation: Reverberation
of the aortic wall, chest wall
Not integrated in the
anatomy of the aorta


48. Intramural Hematoma
class 2 AD
Transesophageal Echocardiography
Erbel R, Heart 2001

49. Intramural Hematoma
No Intimal flap!
circular or half mond-
thickening of
Aortic wall >7mm
Calcification of intima



Mohr - Kahaly et al JACC 1993
class 2 AD Dissection

52. Drohende Perforation bei Plaqueruptur
in der descendierenden Aorta
thoracalis
Pleura
erguß
Plaque-
rupture
Aortensklerose
Klasse 4 AD

53. Case2

55. Angio-Spiral CT mit KM
Aortendissektion Klasse 2
Diagnostik von Aortenerkrankungen

56. Magnetresonanztomographie
Aortendissektion Aneurysma
Klasse 1

57. Aortographie
TL
FL
Aortendissektion
Klasse 1
Svensson LG et al. Circulation 1999
Begrenzte Aortendissektion
Klasse 3

58. Intravaskulärer Ultraschall (IVUS)
Plaqueruptur
(Klasse 4)
Plaqueruptur
der Aorta
Abdominalis
(Klasse 4)
Intramurales
Hämatom
(Klasse 2)
Eggebrecht H, et al., Heart 2001

59. Angio-Spiral CT

70. Case2
• Physical examination: percussion sound dullness over
left lower chest and 2/6 systolic murmur heard best over the
2nd
intercostal space at the right parasternal line
• ECG: Sokolov-index elevated, slight ST-depression
V3-V5
• X-ray: Elongation of the ascending aorta and
shadowing overleft lowerarea
• CK90 U/l; Troponine I 0,1 ng/ml; CRP: 8,4 mg/dl

71. Case3
• 69 year-old female patient
• History : Arterial hypertension >10 y
IDDM
Atrial fibrillation
• Severe thoracic backpain

72. Case3
• EKG: atrial fibrillation, ST depression II,III
• CK33 U/l, Troponine I 0.0 ng/ml

73. Case3
TEE:

74. Case3
Intravascularultrasound (IVUS, Manual Pullback)
2DLongitudinal reconstruction
Intramural hematoma of the descending aorta
(class 2 dissection)

75. Case3
Antihypertensive treatment: Beta-blocker
ACE-inhibitor
Diuretics
Ca-antagonist
RR controlled around 110/80 mmHg
After 10 days (just before discharge) :
recurrent severe backpain at rest

76. Case3
Progression to overt
dissection

77. Case3
Progression to overt
dissection

78. Case3
Additional pleural effusion as a sign of impending rupture
FL
TL

79. Case3
Therapy: Endovascular stent-graft placement

80. PAU- Therapeutic approach
- Ascending aorta - Descending aorta
Surgery
Type-A PAU Type-B PAU
symptomatic asymptomatic
Medical Tx
Risk factors:
• Aortic diameter
• Recurrent pain
• IMH
• (Pseudoaneurysm)
No risk factors
Stent-Graft (?)

81. Diagnostic Aims
• Confirmation of diagnosis
• Classification, extent
• Differentiation TL/FL
• Tear localisation (entry , reentry)
• Side brnch involvement
• Aortic regurgitation (Grading, etiology, valve
morphology)
• Signs of emergency: periaortic -, mediastinal hematoma,
pleural, pericardial effusionOP / Stent - Graft-Stent / medical therapyOP / Stent - Graft-Stent / medical therapy
II IIII

82. IMH- Therapeutic approach
- Ascending aorta - Descending aorta
Surgery
Type-A IMH Type-B IMH
No risk factors
Medical Tx
Risk factors:
• Recurrent pain
• Progression to dissection
• Pleural effusion
Stent-Graft (?)

86. Definition of IMH
• Wall thickening < 7 (5) mm
• Segmental/crescentic wall thickening
• Thrombus – like appearance
• Wall layering,layer shifting
• Absence of tear(s) and flow
• Echolucent zones (+/-),high signal intensity
• Central calcium displacement
Mohr-Kahaly et al JACC 23:658 – 64, 1994
Mohr-Kahly JACC 37:1611- 13, 2001

87. TYPE I INTRAMURAL HEMATOMA
• smooth luminal surface
• circular thickening of the wall
• aortic diameter normal (3.5 cm)
•irregular luminal surface
• extensive arteriosclerotic plaques
• ectatic aorta (4,5 cm)
TYPE II INTRAMURAL HEMATOMA
Mohr-Kahaly et al JACC 23:658 – 64, 1994