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Guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation 2010;121. Introduction . Usually asymptomatic until complication-imaging required for detection and monitoring Risk of radiation and contrast related toxicity

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guidelines for the diagnosis and management of patients with thoracic aortic disease

Guidelines for the diagnosis and management of patients with thoracic aortic disease

Circulation 2010;121

  • Usually asymptomatic until complication-imaging required for detection and monitoring
  • Risk of radiation and contrast related toxicity
  • Results of treatment better in asymptomatic stage
  • Identification of genetic alterations
    • Potential for early detection
    • Targeted therapy

Aneurysm:permanentlocalized dilatation of an artery with at least 50% increase in diameter compared with the expected normal diameter.Covered by all 3 layers

  • Pseudoaneurysm:disruption of arterial wall&extravasation of blood contained by periarterial connective tissue

Aortic dissection:disruptionof media layer of aorta with bleeding within and along the wall of aorta

  • Intramural hematoma:clinical picture of dissection with hematoma in arterial wall,without an flow in false lumen
  • Penetrating atherosclerotic ulcer:atheroscleroticlesion with ulceration that penetrates the internal elastic lamina.
normal thoracic aortic diameter
Normal thoracic aortic diameter
  • Depends on
    • Age
    • Sex
    • Body size
    • Location of measurement
    • Method of measurement
  • Diameter increases by 0.12-o.29mm/yr at each level
imaging modalities classi
Imaging modalities(classI)
  • Measurements should be taken at reproducible anatomic landmarks,perpendicular to axis of flow
  • CT&MRI-ext.diameter taken
  • Echo-internal diameter
  • For aortic root-the widest diameter at mid sinus level
  • Abnormalities of aortic morphology reported separately
  • Minimise cumulative radiation exposure
  • classIIa-aortic diameter should be related to patient's age and body size

Selection of most appropriate imaging study

    • Patient factors-hemodynamic status,renalfn,contrast allergy
    • Availability
  • IRAD-initial imaging modality was CT(61%),TEE(33%),MRI(1%)
  • Advantages
    • Availability
    • Entire aorta imaged
    • Short time
    • Branch vessel invt.
  • Ecggating,MDCT
  • Advantage
    • No radiation
    • Identification of anatomic variants of AoD
    • branch vessel invt
  • Disadvantage
    • Prolonged duration
    • Pt.inaccessible to care providers
    • Gadolinium contrast not used in renal impairment
  • Proximal AoD-
    • TEE sensitivity 88-98%,specificity 90-95%
    • TTE 77-80% and 93-96%
  • Distal AoD-TEE better
  • TEE blind spot-distal ascending aorta&prox.arch-interposition of trachea &lt.main bronchus
acute aortic syndromes
Acute aortic syndromes
  • Consists of 3 conditions-aortic dissection,IMH,PAU
  • 15% of AoD have an IMH without an intimal tear
  • Incidence 2-3.5/1,00,000 person years
  • Mean age of presentation 63yrs
  • Male predominance-65%

Acute dissection-within 2 weeks of onset of pain

  • Subacute-b/w 2-6wks
  • Chronic->6 wks

DeBakey classification:

    • Type I: Dissection originates in Asc.aorta and propagates to include at least the aortic arch
    • Type II: Dissection originates in and confined to the Asc.aorta
    • Type III: Dissection originates in the desc.aorta and propagates most often distally
  • Stanford classification system
    • Type A:dissections involving the asc.aorta regardless of the site of origin
    • Type B:dissections that do not involve the asc.aorta

1.classic dissection

  • 2.IMH
  • 3.intimal tear without IMH
  • 4.PAU
  • 5.iatrogenic/traumatic
  • High risk conditions-(class I)
    • Genetic syndromes like Marfansyn.,Ehlers-Danlos
    • Connective tissue diseases
    • F/h of aortic dissection
    • Known aortic valve disease
    • Recent aortic manipulation
    • Known aortic aneurysm

High risk pain features(classI)

    • Chest,back or abdominal pain
    • Abrupt in onset
    • Severe intensity
    • Ripping,tearing,stabbing or sharp

High risk exam features(classI)

    • Pulse deficit
    • Syst.BP limb diff.>20mmHg
    • Focal neurological deficit
    • Murmur of AR(new)
    • shock
recommendations for screening tests classi
Recommendations for screening tests(classI)
  • STEMI in ECG should be treated as primary cardiac event without delay for aortic imaging unless pt. is high risk
  • Low and intermediate risk patients should be screened by CXR-
    • Findings s/o aortic d/s-definitive aortic imaging
    • Alternate diagnosis-manage accordingly
  • High risk patients should directly undergo TEE,CT or MRI
  • Class III- negative CXR should not delay definitive aortic imaging in high risk pt
diagnostic imaging studies classi
Diagnostic imaging studies(classI)
  • Selection of imaging modality based on pt variables and institutional capabilities
  • If high clinical suspicion exists in spite of negative initial imaging a second study should be performed
recommendations for management
Recommendations for management
  • BP mesured in both arms-treatment strategy based on highest reading
  • Patient not in shock-i.v drugs for rate and B.P control
class i rec for medical management
Class I rec.for medical management
  • i.v beta blockade titrated to HR<60/min
  • CCB alternative if beta blocker contra.
  • After HR controlled,i.v vasodilators to reduce B.P to a level that maintains organ perfusion
  • Beta blocker used cautiously in setting of a/c AR
  • Vasodilator should not be given prior to rate control(classIII)
recommendation for definitive management classi
Recommendation for definitive management(classI)
  • Ascending aorta involved-emergent surgical repair
  • Desc. Aorta involved-managed medically unless lifethreateningcomplications
    • Malperfusion syndrome
    • Progression of dissection
    • Enlarging aneurysm
    • Inability to control BP or symptoms
recommendations for surgical management classi
Recommendations for surgical management(classI)
  • Ascending ao.dissection-aneurysmal aorta and proximal extent of dissection should be resected
  • Prtially dissected root-repaired with valve resuspension
  • Extensive dissection of aortic root-aortic root replacement with a composite graft or with a valve sparing root replacement
  • DeBakey type II-entire dissected aorta should be replaced
class iia
Class IIa
  • IMH-treated similar to aortic dissection in the corresponding segment

Endovascular interventions-not approved for dissection involving asc.aorta or arch

  • Intimal defect without IMH-
    • Asc.aorta-emergency surgery
    • Desc.aorta-endograft
  • Intimal defect with IMH
    • Stable pt-delay def.Rx until IMH resorbs
    • Desc.aorta-endovascular Rx
thoracic aortic aneurysms
Thoracic aortic aneurysms
  • Rec.for medical Rx:classI
    • Antihypertensives to a goal<140/90(goal<130/80 for DM&CKD)
    • Beta blocker in Marfanssyn.&aortic aneurysm
    • Smoking cessation
  • classIIa-
    • Reduce B.P to the lowest point pt can tolerate with beta blocker and ACEI or ARB
    • ARB (losartan) for pt with Marfans syn.
    • Treatment with statin to target LDL-C<70mg%

Use of beta blockers slowed aortic root dilatation(Shores J et al;nejm 1994,Ladouceur M et al;Am J Cardiol 2007)

  • Beta blockers reduced aortic dilatation in c/c type B dissection(Genoni M et al;2001)
  • ACEI perindopril-reduced aortic root dilatation in marfans(Ahimastos AA et al;JAMA2007)
  • ARB-reduced aortic root dilatation in Marfanssyn(Brooks BS et al;nejm2008)
  • Statin use a/w decreased long term mortality in abd aortic aneurysm(Diehm N et al;2008)
surgical management asymptomatic pt classi
Surgical management-asymptomatic pt.(classI)
  • Ascending aortic aneurysm degenerative etiology-surgical repair if diameter>5.5cm
  • Genetic syn.-elective surgery at diameter 4.0 to 5.0 cm
  • Growth rate>0.5cm/yr
  • Patients undergoing AVR at a diameter>4.5cm
rec for surgery classii a
Rec.for surgery-classII a
  • Genetic syn.-ratio of aortic root area(cm²)devided by height (m)>10-surgery indicated
  • Loeys Diets syn,TGFBR1&2 mutation-diameter >4.2 by TEE,or >4.4 by CT or MRI

Patients with symptoms s/o expansion of aneurysm-prompt surgical intervention(classI)

  • Endovascular stent grafts have not been approved by the US FDA for treatment of aneurysms or other conditions of asc. aorta.
rec for open surgery asc aortic aneurysm class i
Rec.for open surgery(asc.aortic aneurysm)-class I
  • Separate valve and asc.aortic replacement in patients without significant root dilatation
  • Patients with significant root dilatation-excision of sinuses&modified David reimplantation or root replacement with valved graft conduit
rec for arch aneurysms classii
Rec. for arch aneurysms-classII
  • Partial arch replacement-asc.aortic aneurysm involves proximal arch
  • Replacement of entire aortic arch-
    • A/c or c/c dissection in an aneurysmal arch
    • Aneurysm of entire arch
    • Distal arch aneurysm that involves prox.desc.aorta
    • Asymptomatic pts. With diameter>5.5cm
  • Annual reimaging-aneurysms<4cm
  • Endovascular stent graft not approved
recommendations for desc thoracic aorta classi
Recommendations for desc.thoracic aorta(classI)
  • Endovascular stent grafting-
    • Degenerative or traumatic aneurysm>5.5cm
    • Saccular aneurysm
    • Post op.pseudoaneurysm
  • Open repair
    • c/c dissection
    • a/w connective tissue d/s
    • Desc.thoracic aorta>5.5 cm

Thoraco abdominal aneurysm-surgery when diameter >6cm

  • End organ ischemia-additional revascularisation procedure recommended
recommendations for genetic syn
Recommendations for genetic syn.
  • classI-
    • Marfan syn.-echo to assess aortic root and asc.aorta at diagnosis &6 months after to determine rate of growth
      • Annual imaging if stable and <4.5cm
      • More frequent imaging otherwise
    • Loeys-Dietz syn,other gen. mutations –complete aortic imaging at diagnosis,6months
    • Loeys-Dietz syn-annual MRI from cerebrovasc.circulation to pelvis
    • Turner syn-imaging at diagnosis for BAV,CoA,dilatationof asc.aorta.
      • If any abnormalities-annual imaging
      • Otherwise-repeat 5 to 10yr

Class IIa

    • Marfansyn planning pregnancy-replacement of aortic root and asc.aorta if >4.5cm
recommendations for familial thoracic aortic aneurysms
Recommendations for familial thoracic aortic aneurysms
  • ClassI
    • Aortic imaging for first degree relatives of pts with thoracic aortic aneurysm or dissection
    • If a mutant gene a/w aneurysm identified-first degree relatives should undergo genetic screening-those with genetic mutation undergo imaging


    • If one or more first degree relatives of a patient affected-imaging of second degree relatives
    • Sequencing of ACTA2 gene in patients with f/h of thoracic aortic aneurysm or dissection
recommendations for bav class i
Recommendations for BAV (class I)
  • First degree relatives of pts with BAV,premature onset of thoracic aortic d/s,familial form of aortic aneurysm and dissection-should be evaluated for presence of BAV&aortic d/s
  • All pts with BAV should be evaluated for aortic dilatation
recommendations for takayasu arteritis gca classi
Recommendations for takayasuarteritis &GCA(classI)
  • Initial evaluation should include CT or MRI of thoracic aorta and branches
  • Initial therapy-corticosteroids at high dose
  • Periodic evaluation-physical examination&ESR or CRP
  • Elective revasc.after a/c inflammatory state is quiescent
  • Class II a –use of anti inflammatory agents
recommendations in pregnancy
Recommendations in pregnancy
  • classI
    • Marfanssyn and aortic d/s-counselled about risk of dissection,heritable nature
    • Strict BP control for pts with predisposition to dissection
    • Aortic root or asc.aortic dilatation-monthly echo
    • Arch,descending or abd.aortic dilatation-MRI


    • CS for pts with significant aortic dilatation
  • Class IIb
    • If progressive aortic dilatation-prophylactic surgery recommended
aortic dissection in pregnancy
Aortic dissection in pregnancy
  • Type A-
    • 1st or 2nd TM-urgent surgical repair and fetal monitoring
    • 3rd TM-urgent CS f/b aortic repair
  • Type B-medical therapy preferred
aortic arch atheroma
Aortic arch atheroma
  • classIIa-Rx with a statin
  • Class IIb-oral anticoagulation with warfarin or antiplatelets in stroke pts with atheroma≥ 4 mm
pre op evaluation for cad
Pre op evaluation for CAD
  • Class I-
    • thoracic aortic d/s undergoing intervention-evaluated for CAD
    • unstable corsyn-undergo revascularisation prior to at time of aortic surgery or intervention
  • classIIa-
    • Asc.aortic or arch d/s undergoing surgery-concomitant CABG for stable significant CAD
  • Class IIb-
    • Descending thoracic aortic d/s-stable significant CAD-benefits not certain
organ protection
Organ protection
  • Class II a-
    • deep hypothermic circulatory arrest for brain protection
    • Optimisation of spinal cord perfusion pressure and moerate systemic hypothermia
  • Class II b-preop.hydration and intra op.mannitol for preservation of renal fn during open repairs of desc.aorta
mortality risk
Mortality risk
  • Composite valve graft,AVR with asc.aortic repair-1-5%
  • Valve sparing aortic root reconstruction-less than1.5%
  • BAV &asc.aorta repair-1.5%
  • a/c AoD-3.5-10%
  • Total arch replacement-2-6%mortality,2-7% risk of stroke