Diseases of the Great Vessels

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Diseases of theGreat Vessels
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Diseases of the Great Vessels
Definition

• Ascending Aorta
• Transverse Aortic Arch
• Descending Thoracic Aorta
• Supra-Renal Aorta
• Infra-Renal Aorta

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Diseases of the Great Vessels

• Atherosclerosis
• Thromboangiitis
• Nonspecific Arteritis (Takayasus)
• Fibrodysplasia
• Uncommon Arteriopathies
• Marfan Syndrome
• Ehlers-Danlos Syndrome
• Cystic medial necrosis
• Aneurysms, Dissecting and True

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Aortic Dissection

• Epidemiology
• 3.5 per 100,000 person-years
• MaleFemale 51
• Peak incidence Type A 50 60 yrs.
• Peak Incidence Type B 60 70 yrs.
• 75 Hypertensive

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Aortic Dissection

• Etiology
• 7 -14 Bicuspid Aortic Valve with Aortic Root
  dilatation
• Coarctation, Arteritis, Aortic Ectasia, Turner or
  Noonan syndrome, Marfans, Ehlers-Danlos, Aortic
  Hypoplasia, Cocaine Use
• gt 40 y.o. Marfans syndrome most common
• gt 40 y.o. Callogen/Elastin Deterioration
• Women lt40 y.o., 50 during pregnancy
• Rupture of Intima and Media

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Aortic Dissection

• Complications
• Shock
• Malperfusion Syndrome

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Malperfusion Syndrome
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Malperfusion Syndrome

• 512 Dissections (Mayo Clinic)
• 29 strokes
• 11 arm ischemia
• 5 paralysis
• 19 mesentaric ischemia
• 23 renal failure
• 62 L.E. Ischemia
• 149 Total

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Aortic Dissection

• Diagnosis
• Chest Pain, Hemoptysis, Dysphagia, Hematemasis,
  Hoarseness
• Differential is large
• CXR
• Non-specific rarely diagnostic
• Angio
• Former gold standard
• 88 sensitive, 85 specific
• Time consuming invasive

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Aortic Dissection

• Diagnosis (cont)
• TEE
• 98 sensitive, 80 specific
• Bedside capable, easy to use
• Can be used in the O.R.
• Downside blind spots caused by trachea
  bronchus, cant see beyond diaphragm
• MRI
• 95 to 100 sensitive and specific
• Long exam time, cant monitor well
• Pacers, metal a problem

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Aortic Dissection

• CT scan
• 87 sensitive, 93 specific
• Should be done on all patients
• Spiral C.T. with contrast
• False lumen usually has thrombus

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Aortic Dissection
STANFORD B POSTERIOR
DISSECTION WITH ANEURYSMAL DILATATION
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Aortic Dissection
TRUE LUMEN
FALSE LUMEN
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Acute Aortic Dissection
STANFORD B POSTERIOR DISSECTION
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Aortic Dissection

• Principles of Treatment
• Type A Usually prompt repair
• Mortality 1 per hour
• Type B
• Rupture uncommon
• Medical Rx preferred unless perfusion syndrome
  present
• Endovascular stenting may have a role

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Aortic Dissection

• Medical Therapy
• Reduce Hemodynamic forces
• Beta BlockerEsmolol
• 500 microgram/Kg bolus
• Infuse 50 200 microgm/kg/min drip
• Then control systolic pressure if needed
• Nipride 20 800 microgm/min
• Serial CTs, Hb, creat, circ checks

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Great Vessels
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Aneurysms of the Great Vessels

• Definition
• Any artery that exceeds 150 the size of the
  normal vessel proximal or distal to it.
• Clinically Significant aneurysm
• Any artery that exceeds 200 the size of the
  normal vessel proximal or distal to it.

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Aneurysmal Disease Etiology

• Medial Matrix Degeneration or Disruption
• Aging
• Atherosclerosis
• Infection
• Inflammation
• Trauma
• Congenital Abnormalities
• Smoking
• Genetic predisposition
• Impaired Connective tissue repair
• Hemodynamic Factors

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Arterial Wall Layers
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Mechanism of Aneurysm Formation

• MMPs Matrix Metalloprotinases
• Family of connective tissue-degrading enzymes
  that affect tissue remodeling
• gt25 known MMPs
• MMP-2, MMP-7, MMP-9, MMP-12, degrade elastin
• Expressed by mesenchymal cells i.e. vascular
  endothelium, SMCs, fibroblasts
• Regulation of MMP activities prevents wide-spread
  tissue distruction

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Mechanism of Aneurysm Formation
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Aneurysms of the Descending Thoracic Aorta

• Etiology Elastin Disruption
• Atherosclerosis
• Infection
• Trauma
• Dissection
• 5 yr. Rupture Rate _at_ 6cm.80
• Enlarge and rupture _at_ greater rate than AAA
• Sx Asymptomatic, Chest pain, Hoarseness,
  Hemoptysis, Dysphagia, Hematemasis
• Rx Surgery Open or Endovascular

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Thoracoabdominal Aortic Aneurysm
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TAAA Etiology

• Matrix Degeneration 82
• Dissection 17
• Marfans
• Ehlers-Danlos 1
• Mycotic
• Takayasus

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TAAA Distribution
Crawford classification
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TAAA (Type B) Symptoms at presentation
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TAAA Diagnosis

• Pain
• CXR
• CT Scan
• MRI
• Angio

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TAAA Natural History without Repair
94 pts
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TAAA Natural History with Repair
604 pts
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TAAA Mortality

• Acute Dissection gt Chronic Dissection
• Patent False Lumen gt Thrombosed Lumen
• Prox. Lt. Subclavian gt Distal Lt. Subclavian

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TAAA Surgical Complications

• Pulmonary 33.0
• Death 9.6
• M.I. 7.2
• Paralysis 6.0
• CVA 4.8
• Renal Failure 2.4

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TAAA Spinal Cord Injury

• CSF Pressure lt 10 mm Hg.
• Steroids
• Mannitol

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Infra-renal Aortic Aneurysms
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Aortic Aneurysm Classification

• True Aneurysms
• Saccular
• Fusiform
• False Aneurysms
• Not all layers of the arterial wall ( intima,
  media, adventitia) are present
• One or more layers of the arterial wall have been
  disrupted

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Anatomy Pathophysiology True Aneurysm
Classification
Fusiform
Saccular
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Risk Factors

• Males (80)
• People over age 55
• Smokers (Smoked more than 100 packs in a
  lifetime)
• 7x more likely than non-smoker to have AAA
• Caucasians
• People with
• Family history of AAA (20)
• High blood pressure
• Diabetes
• High cholesterol

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Statistics - US Incidence/Prevalence

• 200,000 new patients diagnosed with non-ruptured
  aneurysms each year
• More than 1.5 to 2 million are estimated to have
  an undiagnosed AAA
• Up to 50 of patients with untreated aneurysms
  gt5.5 cm will die of rupture in a 5-year period
• Over 15,000 deaths each year

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Aortic Aneurysm Natural History

• Mortality of Rupture 35-75
• Unchanged over past 4 decades
• Worse with COPD, Multiple co-morbidities
• Average rate of growth
• 0.4 cm/year
• 10 per year
• Accelerated by hypertention

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Abdominal Aortic Aneurysm
J Vasc Surg 2003371106-17
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Rupture Risk of Untreated Aneurysms
Infrarenal Aortic Aneuryms, in Vascular Surgery
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AAA Symptoms

• Most nonruptured AAA patients asymptomatic at
  diagnosis
• Vague abdominal pain with back painmost common
  complaint
• Constant or throbbing
• Rapid abdominal expansion may cause intense pain
• AAA should be considered for any elderly patient
  with abdominal, flank or back pain
• GI symptoms (uncommon)
• Early satiety, nausea, weight loss may indicate
  intestinal compression

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AAA Diagnosis

• History
• Physical exam
• Palpable, pulsating mass
• Not effective in obese patients
• Abdominal tenderness over Aorta
• Bruit over Aorta
• Abdominal Ultrasound
• Good Screening Test
• gt80 accurate
• Spiral C.T. (3mm cuts)
• Angiography ?IVUSnot for diagnosis

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AAA Treatment Options

• Watch and Wait
• AAA lt5cm, asymptomatic
• Surgical Risks gt Risk of Rupture
• Lifestyle changes cannot reduce the size of the
  AAA
• Open Surgical Repair
• Endovascular Repair

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Elective Open Surgical Repair

• Major surgical procedure
• Mortality 2 to 8
• Complications
• Pseudoaneurysms (3)
• Erectile dysfunction (gt80)
• Aortoenteric fistula (1-2)
• Graft thrombosis (2)
• Graft infection (1-2)
• Recovery period 6 weeks to 4 months

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Some patients never really bounce back to preop
functional status following surgical repair
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Mayo Study on Open RepairEarly and Late
Graft-related complications

• 307 patients underwent AAA repair
• Anastomotic aneurysm 9 (3.0)
• Graft thrombosis 6 (2.0)
• Graft-enteric erosion/fistula 5 (1.6)
• Graft infection 4 (1.3)
• Anastomotic hemorrhage 4 (1.3)
• Colon ischemia 2 (0.7)
• Tissue loss 1 (0.3)
• Atheroembolism 1 (0.3)

9.4 of patients had major graft-related
complications
J Vasc Surg 199725277-86
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Endovascular AAA Repair

• Benefits of Endovascular Repair compared to Open
  Surgery
• Ability to treat patients unfit for open repair
• Reduction in morbidity
• Reduced blood loss
• Shorter hospital stay
• Earlier return to function

J Vasc Surg 200133S135-45
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Types of AAA Stent Grafts
Cook Zenith
Gore Excluder
Medtronic AneuRx
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Candidates for Endovascular Repair

• Neck size lt28mm
• Neck Length gt1.5cm
• Neck Angulation lt60 degrees
• Acceptable Neck Calcium and Thrombus
• Acceptable Iliac Tortuosity
• Iliac aneurysmal disease treatable
• Iliac size Acceptable
• Able to be converted????

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Example of an Introduction of Device into Aorta
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Orientation of Contralateral Gate
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Proximal Deployment Initial Positioning
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Proximal Deployment Final positioning
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Distal Deployment
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Contralateral Limb Implantation
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Alignment of Contralateral Limb in Gate
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Deployment of Distal Contralateral Limb
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Implantation and Deployment Complete
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• Completion angiogram shows aneurysm exclusion
• CT demonstrates thrombosis of aneurysm sac with
  arterial blood flow through stent graft

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Endographs Risks and Complications

• Risks
• Radiation exposure
• Contrast agents
• Potential future interventions
• Complications
• Endoleaks
• Migration
• Infection

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Endoleaks

• Type I Graft Related ( usually at an attachment
  site)
• Type II - Retrograde Leak, not graft related
  (most common)
• Type III Fabric Tear
• Type IV Graft Porosity
• Type V endotension

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Endoleak angio
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Endoleak CT scan
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Endoleak Treatment
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Endoleak-Post embolization
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Recommended Follow Up
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Diseases of the Great Vessels

• Takayasus Disease
• Usually involves subclavian arteries pulseless
  disease

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Aneurysms of the Ascending Aorta
Etiology

• Medial Degeneration
• Dissection
• Poststenotic Dilatation
• False Aneurysm
• Mycotic Aneurysm
• Atherosclerosis

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0
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Aneurysms of the Ascending Aorta
Medial Degeneration

• Mucoid Degeneration
• Myxomatous
• Cystic Medial Necrosis

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Aneurysms of the Ascending Aorta
Complications

• Rupture
• Within Pericardial Sac
• Cardiac Tamponade
• Tubular Arch
• Severe Hemorrhage
• Aortic Insufficiency
• Death or Disability

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Aneurysms of the Ascending Aorta
Treatment

• Stabilize Medically
• Surgical Repair
• Valve Replacement usually necessary

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Dissecting Aneurysms of the Ascending Aorta

• 62 of Thoracic Dissections
• Most dissect Distally
• Acute death with rupture into pericardium, Aortic
  valve insufficiency or Coronary vessel involvement

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Dissecting Aneurysms of the Ascending Aorta
Clinical Manifestations

• Pain
• Substernal or back
• Variable B.P.
• CXR-Widened Mediastinum

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Dissecting Aneurysms of the Ascending Aorta
Diagnosis

• Clinical Exam and History
• CXR.
• CT Scan
• Aortography

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Dissecting Aneurysms of the Ascending Aorta
Surgical Survival

• 30 Day - 80
• 5 yr. - 57
• 10 yr. - 32
• 20 yr. - 5

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Aneurysms of the TransverseAortic Arch
Etiology

• Medial Degeneration
• Usually in association with proximal and/or
  distal aneurysmal disease

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Aneurysms of the TransverseAortic Arch
Symptoms

• Compression of adjacent structures
• Airway Obstruction
• Venous Obstruction
• Recurrent Laryngeal Nerve Hoarseness
• Chest Wall Pain

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Aneurysms of the TransverseAortic Arch
Diagnosis

• CXR
• CT Scan
• Angio

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Aneurysms of the TransverseAortic Arch
Treatment

• Replacement 78 3yr. survival

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Diseases of the Great Vessels

• Atherosclerosis
• Intimal changes with focal accumulation of
  lipids, blood products, fibrous tissue, and
  calcium
• Involves Large Vessels, not Medium and Small
  Vessels

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Aortic Dissection
Type A
Type B
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TAAA

• Classifications
• Crawford
• DeBakey
• Stanford
• Najafi
• University of Alabama
• Mass. General Hospital