Diseases of the Aorta
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Transcript of Diseases of the Aorta
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Diseases of the AortaDiseases of the Aorta
Seoul National University HospitalDepartment of Thoracic & Cardiovascular Surgery
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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 & abd
ominal aorta
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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)
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Diseases of Thoracic Aorta
Aortic aneurysm
Aortic dissection
Obstructive disease of branches
of the thoracic aorta
Traumatic aortic rupture
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Pathophysiology of Aortic AneurysmDefinition
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
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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
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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
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Marfan’s Syndrome * Definition
1) A heritable disorder (AD) of connective tissue involving biochemical
abnormality of extracellular matrix by a mutation in fibrillin gene on chr
omosome 15 (Fibrillin-1, 350-KD glycoprotein : integral structural comp
onent of 10-nm noncollagenous microfibrils of extracellular matrix in
most tissue)
2) The absence of structural integrity of skeletal, ocular, & cardiova
scular system
3) Adult patients demonstrate abnormal elastic properties manifest
ed by decreased aortic distensibility & increased stiffness index
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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
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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)
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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.
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Patterns of Aortic Aneurysm
Locations of Aneurysm
Ascending aorta 45 %
Aortic arch 10 %
Descending thoracic aorta 35 %
Thoracoabdominal aorta 10 %
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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 %
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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
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Indications for Aortic Aneurysm
Aneurysm diameter 5cm Aneurysm with documented enlargementSymptomatic aneurysm
― chest pain or back pain indicating expansion― significant aortic regurgitation
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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
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Pathophysiology of Aortic Dissection
Malperfusion Reentry
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Predisposing Factors of DA
Hypertension Cystic medial necrosis Marfan syndrome AAE(annuloaortic ectasia) Bicuspid aortic valve Coarctation Pregnancy Chest trauma
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Classification of Dissection
Standford Type A
Involvement of the a-Ao ( arch or d-Ao) regardless of site of primary intimal tear
Type BAll others without involvement of a-Ao
DeBakey I, II, IIIAccording to the location of intimal tear
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Classification of Aortic Dissection
A B
II I III
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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
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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
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Clinical Presentation of DA
Type A Type B Frequency Pain anterior substernal posterior, midscapular,
abdominalSyncope +++ rareDyspnea + ―Blood pressure elevated 50%, low 20% elevated 80%Asymmetric pulses upper, lower extremity lower extremity 30-50%Diastolic murmur 50% 10%Pericardial effusion +++ rarePleural effusion ± +++Hemiparesis or plegia + ― 5-6%Paraparesis or plegia + + 2-6%Renal, intestinal infarction + + 3-5%Myocardial infarction + rare 10 %
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Principle of Treatment in DA
Type A acute aortic dissection Emergent operation
Type B acute aortic dissection Medical Tx and observation unless life threateni
ng Surgical indication
– Persistent pain– Aneurysmal dilatation ( 5cm)– End organ (kidney, bowel) or limb ischemia– Evidence of retrograde dissection to the a-Ao
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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)
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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
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MRI Noninvasive study Do not require contrast medium Better than CT at detecting aortic root dila
tation Disadvantages
cost required time (esp, in acute dissection)
Contraindication pacemaker, claustrophobia
Diagnostic Studies for DA
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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, atheroscl
erosis in the thoracic aorta Limitation
– requires a skilled cardiologist
Diagnostic Studies for DA
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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
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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)
Surgery of Type A Dissection
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Operation of Type A Dissection
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Type A Dissection
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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
Surgery of Acute Type B Dissection
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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 Early mortality : 25~50 % (cf. medical treatment : 7~32 %) Major complications : ischemic spinal cord injury
Surgical Results of DA
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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
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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 )
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Aortic Root, Ascending Aorta, Aortic Arch
Adjuncts for brain protection Reintroduction of antegrade cere
bral perfusion (Frist, 1987)
Retrograde cerebral perfusion (Ueda, 1989)
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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
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Valve-sparing Operation
Resorting aortic root dimensions in an aortic valve-sparing operation when aortic annulus is normal and sinotubular junctio
n is enlarged
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Valve-sparing Operation
Resorting aortic root dimensions when aortic annulus & sinotubular junction are normal, as in aortic dissection
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Valve-sparing Operation
Resorting aortic root dimensions when aortic annulus and sinotubular junction are enla
rged, as in anuloaortic ectasia with Marfan syndrome
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Valve-sparing Operation
Reconstructing aortic root using a graft with the aortic valve placed within it
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Separate valve/graft replacement For older patients with mild to moderate sinus
dilatation
Aortic Root – Wheat Technique
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Bentall technique Coronary artery reattachment
side-to-side anastomosis Disadvantage
bleeding d/t anastomosis tension → pseudoaneurysm (7~25%)
Aortic Root – Composite Valve Graft
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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
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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
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Button technique Coronary artery reattachment Carrel patch for both coronary a.
Direct anastomosis to the composite graft
Aortic Root – Composite valve graft
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Composite Valve Graft
A; aortic valve is excised B; composite prosthetic valve conduit is
attached to annulus of aortic valve
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Results Early mortality : 2~15% Early complications : thromboembolism, bleeding Late complications : endocarditis, thromboembolism pseudoaneurysmTechnique Major Complications 30-Day Survival (%) 5-Yr Survival (%)
Wheat Endocarditis (5%) 85 70
Bentall Thromboembolism (5~10%), endocarditis (5%)
85~90 70~85
Cabrol 90 75 Button Thromboembolism (2~10%),
endocarditis (5%) 85~95 70~85
Surgery of Aortic Root
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Surgery of Aortic Root Results
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Closed technique Limited to a-Ao Aorta cross clamp
Ascending Aorta & Arch
Open techniqueArch involvementDeep hypothermia & circulatory arrest
–EEG monitoring–Retrograde cerebral perfusion
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Elephant Trunk Technique (by Borst, 1988) for extensive aortic aneurysm
(“mega-aorta”)
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Elephant Trunk Technique (Staged op.)
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Results
Major complications – stroke, encephalopathy
Major risk factors – circulatory arrest time, transverse arch involvement
Technique Major Complications 30-Day Survival (30%)
Normothermia Not reported 25
+ Antegrade cerebral perfusion Not reported 75
TCA with profound hypothermia Stroke (2~10%) 85~90
+ Antegrade cerebral perfusion Stroke (5~6%) 80~100
+ Retrograde cerebral perfusion Stroke (3%) 95
Surgery of Ascending Aorta & Arch
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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)
Descending Thoracic & Thoracoabdominal Aorta
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Indications – Poor surgical candidates for t
horacic 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%)
Endovascular Stent Graft
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Modified Crawford’s classification for TAAA
Thoracoabdominal Aorta
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–Technique Thoracoabdominal incision Descending thoracic aorta involvement
Distal aortic perfusionCSF drainageIntercostal artery reattachment
(T9~12) Celiac axis, SMA, IMA, renal arteries
Visceral perfusionCarrel patch or bypass graft
Thoracoabdominal Aorta
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Thoracoabdominal Aorta
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Descending Thoracic & Thoracoabdominal Aorta Results
Risk Factors for poor outcome– aneurysm extent (type II)– preop. renal dysfunction– aortic cross clamp time
Technique Major Complications 30-Day Survival 5-Yr SurvivalDescendingthoracic
Neurologic deficit (2~15%),renal failure (14%) 50~80%
TAAA type I, II& IV
Neurologic deficit (0~15%),renal failure (5~25%) 90~95 % 60~75%
TAAA type II,no adjuncts
Neurologic deficit (30~40%),renal failure (17%) 78% 35%
TAAA type II,with adjuncts
Neurologic deficit (12%), renalfailure (7%) 90% 60~70%
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Abdominal Aortic Aneurysm1. Type Fusiform : most Sacciform Dissecting : rare False
2. Etiology Atherosclerosis : 90%
Traumatic Syphilitic Congenital Infected Pregnancy related
Anastomotic
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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
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Procedures for Abdominal AA
1 Heparin 1mg/kg IV2 Mannitol 0.5g/kg in suprarenal clamp3 Inferior mesenteric artery occlusion4 Lumbar arteries oversewn5 Proximal and distal anastomosis6 Reimplantation of inferior mesenteric artery
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Operative Complications
1 Division of parasympathetic and sympathetic nerves crossing the proximal common iliac arteries 2 Peripheral embolism3 Paralytic ileus4 Aortoenteric fistula