Diseases of the Aorta

Cindy Chan, MD

Overview
Aortic structure Dilating disease Constricting disease Diagnosis Treatment

Aortic structure
Aortic layers: intima, media and adventitia Medial Lamellar units (layers)
Collagen/elastin composition Most collagen in adventitia (for tensile strength) Most elastin in media (for compliance)

Zatina, et al. J Vasc Surg 1:443, 1984.

Matrix degradation
Proteases
Matrix Metalloproteinases MMPs
Eg. MMP-1 (collagenase), MMP-2, MMP-9 (elastase)

Protease inhibitors
Eg. a-1 antitrypsin

Dilating disease of the aorta

Dilating disease of the aorta

Nonspecific Aneurysm Dissection Congenital
Eg. Marfans Disease, Ehlers-Danlos syndrome

Infection Trauma Unusual

Eg. Behets Disease

Nonspecific Aortic Aneurysm

Atherosclerotic - misnomer Genetic
Males

Inflammation
Inflammatory cell infiltrates

Matrix degradation
Increased collagenase and elastase activity

Defective remodeling
Cellular and matrix proliferation

Nonspecific Aortic Aneurysm

Location
Thoracic - rare (Marfans, chronic dissection) Thoraco-abdominal Abdominal (most)

Natural history
Unpredictable rate of increase Risk of rupture aneurysm diameter

Distribution of aneurysms in aorta

AAA, Ultrasound Imaging

Mural Thrombus Aortic Lumen

Aortic Diameter

Complications of aneurysms
Rupture Free, anterior rupture Retroperitoneal rupture Rupture into adjacent structures Inferior vena cava: aorto-cava fistula Duodenum: aorto-enteric fistula Thrombosis Acute arterial occlusion (of the aneurysm itself) Embolization Acute arterial occlusion (distally) Compression of adjacent structures Veins, nerves, bowel

Infrarenal AAA
Mortality is 75-90% 5 year rupture risk is related to transverse diameter
< 3 cm 3.5-4.9 cm > 5.0 cm > 6.0 cm > 8.0 cm ~ 0% ~ 5% ~ 25% ( ~20% at 2 years) ~ 40% 75-80%

5.0 cm is cut-off for surgical intervention

Dilating disease of the aorta

Dissection
Incidence 5-10 / 1,000,000 (~2x rupt AAA) Blood dissects through intima, separating intima from media Classification - Stanford A & B Acute dissection
High mortality if untreated
33% 50% 66% 90% 24 h 48 h 1 wk 1 mo

1% mortality rate / hour for the first 48 hours

Stanford Classification Of Aortic Dissection

Miller, et al., J Thorac Cardiovasc Surg 78:365, 1979.

Dilating disease of the aorta

Aortic Dissection
Stanford type A

~ 2/3 of aortic dissections Tear in ascending aorta or arch Younger, inherited connective tissue disease High mortality with medical Rx
~ 1/3 of aortic dissections Tear in descending aorta Older patients, chronic hypertension Equivalent early results with medical Rx

Stanford type B

Effects of aortic dissection on branch arteries

Complications of Aortic Dissection

Rupture of false lumen
Free rupture into chest or abdomen Pericardial tamponade exsanguination

Compression by false lumen

Aortic annulus Main aortic lumen Arterial branches

aortic insufficiency ischemia ischemia

Coronary myocardial infarction Bracheocephalic stroke Spinal paralysis Renal renal failure Mesenteric intestinal infarction Extremity gangrene

Gradual enlargement of false lumen

Chronic dissection false aneurysm Late rupture!

Aortic segmental and branch artery involvement in 450 aortic dissections (by autopsy)

From: Hurst and Gore, in Doroghazi and Slater (eds.): Aortic Dissection, McGrawHill, 1983, p 193.

Dilating disease of the aorta

Infection
Direct extension - rare - pancreatic abscess Embolomycotic
Septic emboli Bacterial endocarditis (Viridans Strep)

Cryptogenic (hematogenous)
Normal aorta:
Syphilitic (thoracic aorta) Salmonella (abdominal aorta)

Diseased aorta:
GM + organisms (strep / staph) Multiple organisms - fungus mycobacteria - immunocompromized patients

Trauma
IV drug use - direct inoculation Iatrogenic

Dilating disease of the aorta

Also..Pseudoaneurysm
Focal destruction of arterial wall Specific pathology
Trauma Infection Prior vascular repair

Saccular configuration Less predictable natural history Expectant management is not appropriate

Aortic Atherosclerotic Ulcers

From: Stanson AW, in Strandness and vanBreda (eds.): Vascular Diseases, Churchill Livingstone, 1994, p 600.

Perforation of the aorta

Constricting disease of the aorta

Constricting disease of the aorta

Congenital
Coarctation
Thoracic Abdominal (0.5-2% of coarcs)

Acquired
Atherosclerosis Radiation Takayasus disease Tumor

Constricting disease
Stenosis - limits maximal flow volume through an artery Symptoms result from ischemia of organs
Resting demands Increased demands
Exercise Emotional stress Digestion

Constricting disease of the aorta

Atherosclerosis
Location
Branch points Turbulent flow, decreased shear Abdominal > thoracic aorta

Arterial adaptation to atherosclerotic plaque

Glagov, et al., N Engl J Med 316:1371, 1987. Zarins, et al., J Vasc Surg 7:386, 1988.

Hypoplastic Aorta Syndrome

43 yo WF - Hyperlipidemic - Smoker - post menopausal - Rest pain

Calcified aortic (coral reef) atherosclerosis

Fibromuscular dysplasia (FMD) of the Iliac artery

Diagnosis & Treatment of Dilating & Contricting Disease

Diagnosis
Symptomatic presentation
Pain Hypotension Ischemia - visceral, extremity, cerebral

Physical Exam
Pulses / bruits Abdominal masses

Imaging
Lumenal - stenoses: - duplex U/S, arteriography Mass - aneurysm/ dissection: - CT, MR, U/S

Treatment
Type B Aortic Dissection
Treatment Goals:
Control propagation of dissection
Reduce arterial pressure (beta-blockade) Success judged by pain relief

Prevent and/or treat complications

Hemorrhage Branch artery occlusion with critical ischemia

Treatment
Dilating diseases
Replacement of weakened wall Control of infection?

Constricting disease
Medical therapy for rare cases Atherosclerosis
Risk factor management Bypass grafts

Endoluminal Grafting
- nitinol supports - thin wall seamless fabric

Meadox Vanguard

Endoluminal Aortic Grafting