Diseases of the Aorta Seoul National University Hospital



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Diseases of the Aorta

  • Seoul National University Hospital

  • Department of Thoracic & Cardiovascular Surgery


Anatomy of Aorta

  • Aortic root

    • aortic valve, sinus of Valsalva, coronary artery
  • Ascending aorta

    • aortic root ~ innominate artery
  • Aortic arch

    • proximal, distal
  • Descending thoracic aorta

    • distal to LSCA ~ 12th ICS
  • Thoracoabdominal aorta

    • descending thoracic aorta & abdominal aorta


Properties of Aorta & Major Conduit

  • 1. Aorta

    • Compliant vessel (Windkessel function)
    • ; transforms pulsatile hydraulic energy into a more steady flow by elastic distension & contraction
  • 2. Synthetic conduit

    • Noncompliant
    • ; must result in alteration of arterial hemodynamics & LV load (increased impedance & afterload)


Diseases of Thoracic Aorta

  • Aortic aneurysm

  • Aortic dissection

  • Obstructive disease of branches

  • of the thoracic aorta

  • Traumatic aortic rupture



Pathophysiology of Aortic Aneurysm

  • Definition

    • localized or diffuse dilatation > 50% of normal diam.
    • Most common aortic disease that require surgery
  • Etiology

    • Atherosclerosis ( + underlying weakness)
    • Chronic aortic dissection
    • Annuloaortic ectasia (Marfan syndrome)
    • Trauma
    • Infection
    • Associated with aortic valve disease


Histopathology of Ascending Aortic Aneurysm

    • 1. Cystic medial necrosis by pooling of mucoid material
    • 2. Elastin fragmentation by disruption of elastin lamellae
    • 3. Fibrosis as an increase in collagen at the expense of smooth muscle cells
    • 4. Medionecrosis as areas with apparent loss of nuclei


Pathophysiology of Ascending AA

  • Marfan syndrome

    • Incidence
      • 1 / 5,000
    • Annuloaortic ectasia is very common
    • Associated defects
      • Aortic regurgitation, mitral valve prolapse, dysrhythmia
      • Tall stature, long limbs and digits, anterior chest deformity, joint laxity, vertebral column deformity
      • High arched palate, lens disorder


Marfan’s Syndrome

  • * Definition

    • A heritable disorder (AD) of connective tissue involving biochemical abnormality of extracellular matrix by a mutation in fibrillin gene on chromosome 15 (Fibrillin-1, 350-KD glycoprotein : integral structural component of 10-nm noncollagenous microfibrils of extracellular matrix in most tissue)
    • The absence of structural integrity of skeletal, ocular, & cardiovascular system
    • Adult patients demonstrate abnormal elastic properties manifested by decreased aortic distensibility & increased stiffness index


Marfan’s Syndrome

  • Clinical manifestations

  • Cardiovascular

  • Ocular

  • Skeletal abnormality

  • Cardiovascular manifestations

  • Progress with time

  • Mitral valve prolapse in 100%

  • Aortic root dilatation in 80%

  • Rarely atrial septal aneurysm



Manifestations of Marfan’s Syndrome

  • 1. Patterns of aortic dilatation

    • 1) 80% of the patients shows aortic dilation
    • 2) more commonly generalized form than localized form
    • 3) more commonly aortic regurgitation in generalized form
  • 2. Natural prognosis

    • 1) Life expectancy is significantly reduced (40~50) as a
    • consequence of aortic dilatation & its complications
    • (aortic dissection, fatal rupture, AR, heart failure)


Marfan’s Syndrome in Children

  • 1. Diagnosis can be made at any age with marked variation

  • in clinical expression.

  • 2. Patients without family history (in one third of patients

  • of all age) have more severe manifestation probably

  • due to sporadic mutation.

  • 3. Surgery should be carried out even in asymptomatic

  • patients, once the diameter of the aortic root or

  • ascending aorta reaches 5 to 6cm as in adults.

  • 4. Mitral valve prolapse is as common as aortic root

  • dilatation and progression can cause significant

  • morbidity & mortality.



Patterns of Aortic Aneurysm

  • Locations of Aneurysm

    • Ascending aorta 45 %
    • Aortic arch 10 %
    • Descending thoracic aorta 35 %
    • Thoracoabdominal aorta 10 %


Natural History of AA

  • Aortic aneurysm

    • Incidence
      • 5.9 new aneurysms / 100,000 person-years
    • Life time probability of rupture : 75~80%
    • 5-yr untreated survival rate : 10~20%
    • Median time to rupture : 2~3 yrs
    • Size Risk of rupture within 1yr
    • < 5 cm 4 %
    •  6 cm 43 %
    •  8 cm 80 %


Clinical Presentation of AA

  • Symptoms & signs

    • Asymptomatic
    • Compressive symptoms
      • recurrent laryngeal n. or vagus n. : hoarseness
      • tracheobronchial tree : dyspnea
      • pulmonary a. : fistula, bleeding  pulmonary HT & edema
      • esophagus : dysphagia
      • stomach : sensation of satiety  wt. loss
    • Pain  aneurysmal expansion
    • Intestinal angina, renovascular HT
    •  associated atherosclerotic obstructive disease (5% in TAAA)
  • Physical finding - usually unremarkable

    • Wide pulse pressure, diastolic murmur  AR


Indications for Aortic Aneurysm

  • Aneurysm diameter  5cm

  • Aneurysm with documented enlargement

  • Symptomatic aneurysm

    • ― chest pain or back pain indicating expansion
    • ― significant aortic regurgitation


Dissecting Aortic Aneurysm

  • Catastrophic event

  • Intimal tear

  • False channel

    • in the outer half of the media
    • highly susceptible to rupture
  • Acute dissection

    • < 2 wks from Sx onset
  • Chronic dissection

    • > 2 wks from Sx onset


Pathophysiology of Aortic Dissection



Predisposing Factors of DA

  • Hypertension

  • Cystic medial necrosis

  • Marfan syndrome

  • AAE(annuloaortic ectasia)

  • Bicuspid aortic valve

  • Coarctation

  • Pregnancy

  • Chest trauma



Classification of Dissection

      • Standford
        • Type A
          • Involvement of the a-Ao ( arch or d-Ao)
          • regardless of site of primary intimal tear
        • Type B
          • All others without involvement of a-Ao
      • DeBakey I, II, III
        • According to the location of intimal tear




Natural History of DA

  • Annual incidence

    • 5~10 / million
  • Sex ratio

    • M:F = 2:1 ~ 5:1
  • Acute dissection

    • Median time to rupture : 3 days
    • Mortality rate ; 50 % within 2 days
    • 75 % within 2 wks
  • Chronic dissection

    • Median time to rupture : 1~3 Yrs
    • Follows patterns of non-dissecting aneurysm


Clinical Presentation of DA

  • Acute dissection

    • Excruciating pain
      • abrupt onset
      • sudden rise to peak
      • Chest pain
        • 2/3 of a-Ao dissection
      • Back pain
        • dissection distal to aortic arch
      • Pain may migrate as the dissection moves distally.
    • Various extent of peripheral & central vessel occlusion
      • from progression of dissection through the false lumen
    • Failure of diagnosis : major problem


Clinical Presentation of DA

    • Type A Type B Frequency
  • Pain anterior substernal posterior, midscapular, abdominal

  • Syncope +++ rare

  • Dyspnea + ―

  • Blood pressure elevated 50%, low 20% elevated 80%

  • Asymmetric pulses upper, lower extremity lower extremity 30-50%

  • Diastolic murmur 50% 10%

  • Pericardial effusion +++ rare

  • Pleural effusion ± +++

  • Hemiparesis or plegia + ― 5-6%

  • Paraparesis or plegia + + 2-6%

  • Renal, intestinal infarction + + 3-5%

  • Myocardial infarction + rare 10 %



Principle of Treatment in DA

  • Type A acute aortic dissection

    • Emergent operation
  • Type B acute aortic dissection

    • Medical Tx and observation unless life threatening
    • Surgical indication
      • Persistent pain
      • Aneurysmal dilatation ( 5cm)
      • End organ (kidney, bowel) or limb ischemia
      • Evidence of retrograde dissection to the a-Ao


Medical Management of DA

  • Initial management

    • Immediate ICU care
    • BP control & Monitoring
      • Central line, arterial line, urine output
    • Imaging studies
      • Daily Chest X-ray, weekly CT scan during hospitalization
  • Pharmacologic therapy

    • Vasodilator : Sodium nitroprusside
    • β-blocker : Esmolol (β-1 selective & short acting)


Diagnostic Studies for DA

  • CT & CT angiography

    • Aneurysm size, location, extent, intimal tear site
    • Other pathologies in the chest & abdomen
    • Follow-up study : aneurysm growth
    • Limitation
      • unreliable detection of root enlargement
    • Contraindication
      • renal insufficiency, allergy to contrast agents


Diagnostic Studies for DA

  • MRI

  • Noninvasive study

  • Do not require contrast medium

  • Better than CT at detecting aortic root dilatation

  • Disadvantages

    • cost
    • required time (esp, in acute dissection)
  • Contraindication

    • pacemaker, claustrophobia


Diagnostic Studies for DA

  • Transesophageal Echocardiography (TEE)

    • Accuracy in imaging intimal tear : 90%
    • Assessment of cardiac structure & function
    • Highly sensitive in aortic pathology diagnosis
      • aortic valve disease, aortic dilatation, dissection, thrombi, atherosclerotic disease
    • Intraoperative monitoring
      • check cardiac function, aortic valve competency, atherosclerosis in the thoracic aorta
    • Limitation


Diagnostic Studies for DA

  • Aortography

    • Geography of the aorta & condition of smaller vessels
    • Previous gold standard in dissection
      • double lumen, tear site, extent
    • Indication
      • renovascular HT, intermittent claudication, atherosclerotic occlusive abdominal aorta, symptoms of carotid artery occlusion
    • Disadvantages
      • invasive procedure using radiopaque dyes
  • Cardiac cath & coronary angiography

    • Evaluation of the concomitant coronary artery disease


Surgery of Type A Dissection

  • Principles of Surgical Tx in Acute Dissection

    • Resection of aortic segment containing intimal tear
    • Obliteration of false lumen in both end of remained aorta
    • Graft replacement of resected aortic segment
  • Techniques

    • Median sternotomy
    • Femoral-femoral bypass
    • Trendelenburg position
    • Circulatory arrest with deep hypothermia
    • Retrograde cerebral perfusion
    • Reinforcement of the intima & adventitia together (sandwich technique)


Operation of Type A Dissection



Type A Dissection



Surgery of Acute Type B Dissection

  • Techniques

    • Similar to the techniques for aneurysm
    • Rechanneling blood into the true lumen
    • Ligation of all intercostal arteries in acute dissection
  • Surgical indications

    • Persistent pain
    • Aneurysmal dilatation ( 5cm)
    • End organ(kidney, bowel) or limb ischemia
    • Evidence of retrograde dissection to the a-Ao


Surgical Results of DA

  • Acute Type A Dissection

    • Early mortality : 20~30 %
    • Main cause of death  underlying end-organ injury
    • Major complications  stroke (9%)
    • Major risk factors for postop. stroke
      • pump time, episode of severe hypotension
  • Acute Type B Dissection



Surgical Treatment of AA Aneurysm : Aortic Root, a-Ao, Aortic Arch

  • Historical evolution

    • 1950s : Cardiopulmonary Bypass (Gibbon)
    • 1955 : 1st successful a-Ao repair (Cooley & DeBakey)
    • 1964 : 1st successful replacement of entire a-Ao (Wheat)
      • CPB, coronary perfusion, myocardial cooling, cold cardiac arrest
    • 1968 : Composite valve graft (Bentall & de Bono)
    • 1975 : Replacement of entire aortic arch (Griepp)
      • profound hypothermia & circulatory arrest


Aortic Root, Ascending Aorta, Aortic Arch

    • Limitation of profound hypothermia
      • < 30 min : safe duration
      • > 45 min : increased incidence of stroke
      • > 65 min : increased incidence of death
    • Calculated safe duration of hypothermic circulatory arrest
  • Temperature Cerebral Metabolic Rate Safe Duration of HCA

  • (C) (% of baseline) (min)

  • 37 100 5

  • 30 56 ( 52 ~ 60 ) 9 ( 8 ~ 10 )

  • 25 37 ( 33 ~ 42 ) 14 ( 12 ~ 15 )

  • 20 24 ( 21 ~ 29 ) 21 ( 17 ~ 24 )

  • 15 16 ( 13 ~ 20 ) 31 ( 25 ~ 38 )

  • 10 11 ( 8 ~ 14 ) 45 ( 36 ~ 62 )



Aortic Root, Ascending Aorta, Aortic Arch

  • Adjuncts for brain protection

    • Reintroduction of antegrade cerebral perfusion (Frist, 1987)
    • Retrograde cerebral perfusion (Ueda, 1989)


Aortic Root - Techniques

    • Median sternotomy
    • Antegrade and/or retrograde
    • cardioplegic perfusion
    • Techniques for aortic root
      • Wheat
      • Composite graft (esp, for Marfan)
        • Bentall
        • Cabrol
        • modified Cabrol
        • button
      • Homograft
      • Valve sparing procedure
      • Choice of tube graft ; diameter of 10% smaller than the length of the free
      • margin of the aortic leaflet


Valve-sparing Operation



Valve-sparing Operation



Valve-sparing Operation



Valve-sparing Operation



Aortic Root – Wheat Technique

    • Separate valve/graft replacement
    • For older patients with mild to moderate sinus dilatation


Aortic Root – Composite Valve Graft

  • Bentall technique

    • Coronary artery reattachment
      • side-to-side anastomosis
    • Disadvantage
      • bleeding d/t anastomosis tension
      • → pseudoaneurysm (7~25%)


Aortic Root – Composite Valve Graft

  • Cabrol technique

    • Coronary artery reattachment
      • a small graft to the both coronary arteries
      • side-to-side anastomosis of the small graft & composite graft
    • Advantage
      • ↓anastomosis tension
    • Disadvantage
      • kinking at the anastomosis sites


Aortic Root – Composite Valve Graft

  • Modified Cabrol technique

    • Coronary artery reattachment
      • a small graft to the LCA
      • end-to-side anastomosis of the small graft & composite graft
      • button attachment of the RCA
    • Advantage
      • ↓kinking


Aortic Root Composite valve graft

      • Button technique
        • Coronary artery reattachment
        • Carrel patch for both coronary a.
          • Direct anastomosis to the composite graft


Composite Valve Graft



Surgery of Aortic Root

  • Results

    • Early mortality : 2~15%
    • Early complications : thromboembolism, bleeding
    • Late complications : endocarditis, thromboembolism
    • pseudoaneurysm


Surgery of Aortic Root

  • Results



Ascending Aorta & Arch

  • Closed technique

    • Limited to a-Ao
    • Aorta cross clamp


Elephant Trunk Technique (by Borst, 1988)

  • Elephant Trunk Technique (by Borst, 1988)

    • for extensive aortic aneurysm (“mega-aorta”)


Elephant Trunk Technique (Staged op.)

  • Elephant Trunk Technique (Staged op.)



Surgery of Ascending Aorta & Arch

  • Results

    • Major complications
      • stroke, encephalopathy
    • Major risk factors
      • circulatory arrest time, transverse arch involvement


Descending Thoracic & Thoracoabdominal Aorta

  • Spinal protection

    • Arterial radicularis magna (Adamkiewicz a.)
    • Technique
      • Shunt
      • Hypothermic circulatory arrest
      • Spinal cord cooling
      • Pharmacologic agent
      • Sequential aortic clamp
      • Distal aortic perfusion
      • CSF drainage
      • Intercostal artery reattachment (T9~12)


Endovascular Stent Graft

    • Indications
      • Poor surgical candidates for thoracic aneurysm
      • Expected survival time < 5 yrs
    • Problem
      • Endoleaks (→ graft migration)
      • Exclusion of intercostal arteries
      • Lack of long-term data
    • Results
      • Early mortality : 9%
      • Complications
        • stroke (7%)
        • paraplegia (3%)
        • early endoleak (24%)
        • reintervention (5%)


Thoracoabdominal Aorta

  • Modified Crawford’s classification for TAAA



Thoracoabdominal Aorta

      • Technique
        • Thoracoabdominal incision
        • Descending thoracic aorta involvement
          • Distal aortic perfusion
          • CSF drainage
          • Intercostal artery reattachment (T9~12)
        • Celiac axis, SMA, IMA, renal arteries
          • Visceral perfusion
          • Carrel patch or bypass graft


Thoracoabdominal Aorta



Descending Thoracic & Thoracoabdominal Aorta Results

    • Risk Factors for poor outcome
      • aneurysm extent (type II)
      • preop. renal dysfunction
      • aortic cross clamp time


Abdominal Aortic Aneurysm

  • 1. Type Fusiform : most

  • Sacciform

  • Dissecting : rare

  • False

  • 2. Etiology Atherosclerosis : 90%

  • Traumatic

  • Syphilitic

  • Congenital

  • Infected

  • Pregnancy related

      • Anastomotic


Pathophysiology of Abdominal Aorta

    • Nature of the aortic wall
      • 1) Contain more elastin, deposition of
      • cholesterol and calcium
      • 2) Stress factor and turbulent flow due to
      • origin of major branches
      • 3) Stability of proximal abdominal aorta and
      • presence of large bifurcation
    • Hemodynamic factor
    • Physical factor


Procedures for Abdominal AA

  • Heparin 1mg/kg IV

  • Mannitol 0.5g/kg in suprarenal clamp

  • Inferior mesenteric artery occlusion

  • Lumbar arteries oversewn

  • Proximal and distal anastomosis

  • Reimplantation of inferior mesenteric

  • artery



Operative Complications

  • Division of parasympathetic and

  • sympathetic nerves crossing the

  • proximal common iliac arteries

  • Peripheral embolism

  • Paralytic ileus

  • Aortoenteric fistula



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