Aortic ulcer – intramural hematoma- aortic dissection: a continuous spectrum

R Erbel, H Eggebrecht, D Baumgart, J DebatinJ Barkhausen,U Herold, H Jakob

Department of Cardiology Radiology and Thoracic and Cardiovascular Surgery

University Essen, Germany

Classification of acute aortic syndromes

Svensson LG et al.Circulation 99: 1331-6, 20001- 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

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“

Cystic Media Necrosis

Collagen Fiber Rupture

Cystic Media Necrosis

Collagen Fiber Rupture and Intramural Hemorrhage

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

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

- 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

- Displacement of intimal calcifications

- Affects long segment of the aorta

Intramural hematoma, Class 2 AD (IMH)

Differentiation against thrombosed aneurysm

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)

1Maraj et al,, Am J Cardiol 2000

Outcome1:

IMH- Outcome

1Mara et al,, Am J Cardiol 2000

Intramural Hematoma

Aortography

IVUS

Class 2 AD type B

Intravascular Ultrasound

Pericardial tamponade, progression to dissection, rupture

within one week despite

RR control

IMH- Complications

History of PAU Reports

• 1935 Shennan T 4/218 cases AD begin in the

base of AU

• 1941 Will ius /Cragg „some of AD accociated with

ulcerating atheromatous

abscesses“• 1973 Gore/Hirst < 4% cause of AD• 1986 Stanson: Penetrating aortic ulcer

PAU

Vilacosta et al JACC 32:83 – 9,1998

- 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

- 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)

CTIVUSErbel R, EHJ 2001

Plaque Rupture class 4 AD

Ao

Fibrous cap

Ulcer core

1 cm

Erbel R Heart 2001

IVUS

MRI Imaging

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

Ruptured Plaque with Floating Fibrous Cap

Tear

Fibrous Cap

Ulcer

PAU- Complications

- Pseudoaneurysm : 0- 50%1,2

Growth rate: 0,31 cm/ year

1Yucel, Radiology 19902Harris, J Vasc Surg 1994

- Embolism: rare

PAU- Complications

- 0- 44%1,2 rupture

1Stanson, Ann Vasc Surg 19862Harris, J Vasc Surg 19943Coady, J Vasc Surg 1998

- 40% for PAU vs. 3.6% for classic type B dissection3

- Risk factors : symptomatic patient, aortic diameter,

*

type-A PAU

Impending Perforation of Plaque Rupture of descending Aorta

Pleuraeffusion

Plaque-rupture

Aortic sclerosisclass 4 AD

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

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

Media Necrosis Erdheim Gsell Aortic Disease

Entry Tear

IMH Aortic dissection

class 2 AD

Aortic rupture

Healing

No continuity: PAU, IMH, dissection

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

Aortic Diseases

Aortic rupture

Aortic Disease-congenital-degenerative-arteriosclerotic-inf lammatory-traumatc,toxic

Healing

TraumaClass 5

Plaque rupture Class4

Discrete/subtit leDissection Class 3

IntramuralHaematomaHaemorrhage Class 2

Aort ic dissection Class 1Communicating/non communicating AD

ESC Task Force EHJ 2001

IMH with PAUMRI:

Contained rupture of the descending thoracic aorta due to penetrating (PAU)

atherosclerotic ulcer (class IV type B) with IMH pleural effusion

IMH

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

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

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

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

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

Prognosis of PAU Total Type A Type BAortic 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

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

FOLLOW UP IMH

Ascending aorta:n= 3 1surgery 1ruptur 1 dissectionDescending aorta:n=24 4 dissection 3 surgery 3 healing 6 death

Assessment of the true and false lumen Ao desc 23 cm

1.19 cm

Visualisation of Intimal Tearusing 3D-Echocardiography

Non communicating dissection type B 38 cm

Aortic dissection classification

Morphology of False Lumen

WL

FL

WL

FL

WL

FL

Pitfalse

Artefacts

Explanation: Reverberationof the aortic wall, chest wallNot integrated in the anatomy of the aorta

Intramural Hematoma class 2 AD

Transesophageal Echocardiography

Erbel R, Heart 2001

Intramural Hematoma

No Intimal flap! circular or half mond-thickening of Aortic wall >7mmCalcification of intima

Mohr - Kahaly et al JACC 1993

class 2 AD Dissection

Drohende Perforation bei Plaqueruptur in der descendierenden Aorta

thoracalis

Pleuraerguß

Plaque-rupture

Aortensklerose

Klasse 4 AD

Case 2

Angio-Spiral CT mit KM

Aortendissektion Klasse 2

Diagnostik von Aortenerkrankungen

Magnetresonanztomographie

Aortendissektion Aneurysma

Klasse 1

Aortographie

TL

FL

Aortendissektion Klasse 1

Svensson LG et al. Circulation 1999

Begrenzte Aortendissektion Klasse 3

Intravaskulärer Ultraschall (IVUS)

Plaqueruptur(Klasse 4)

Plaquerupturder Aorta Abdominalis(Klasse 4)

IntramuralesHämatom(Klasse 2)

Eggebrecht H, et al., Heart 2001

Angio-Spiral CT

Case 2

• 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 over left lower area

• CK 90 U/l; Troponine I 0,1 ng/ml; CRP: 8,4 mg/dl

Case 3

• 69 year-old female patient

• History : Arterial hypertension >10 y

IDDM

Atrial fibrillation

• Severe thoracic back pain

Case 3

• EKG: atrial fibrillation, ST depression II,III

• CK 33 U/l, Troponine I 0.0 ng/ml

Case 3TEE:

Case 3Intravascular ultrasound (IVUS, Manual Pullback)

2D Longitudinal reconstruction

Intramural hematoma of the descending aorta (class 2 dissection)

Case 3

Antihypertensive treatment: Beta-blockerACE-inhibitorDiureticsCa-antagonist

RR controlled around 110/80 mmHg

After 10 days (just before discharge) :

recurrent severe back pain at rest

Case 3

Progression to overt dissection

Case 3

Progression to overt dissection

Case 3

Additional pleural effusion as a sign of impending rupture

FLTL

Case 3Therapy: Endovascular stent-graft placement

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 (?)

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

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 (?)

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

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