1. Guidelines for the diagnosis and management of patients with thoracic aortic disease
Circulation 2010;121

2. Introduction
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

3. Aneurysm:permanent localized 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

4. Aortic dissection:disruption of 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 intimal disruption.no flow in false lumen
Penetrating atherosclerotic ulcer:atherosclerotic lesion with ulceration that penetrates the internal elastic lamina.

5. 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

7. Aortic diameter at various levels by CT

8. Aortic root dimensions by echo

9. 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

12. Selection of most appropriate imaging study
Patient factors-hemodynamic status,renal fn,contrast allergy
Availability
IRAD-initial imaging modality was CT(61%),TEE(33%),MRI(1%)

13. CT Advantages Ecg gating,MDCT Availability Entire aorta imaged
Short time
Branch vessel invt.
Ecg gating,MDCT

14. MRI Advantage Disadvantage 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

15. Echocardiography Proximal AoD- Distal AoD-TEE better
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

16. 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%

17. Acute dissection-within 2 weeks of onset of pain
Subacute-b/w 2-6wks
Chronic->6 wks

18. 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

20. Classes of intimal tears

21. 1.classic dissection
2.IMH
3.intimal tear without IMH
4.PAU
5.iatrogenic/traumatic

22. Evaluation High risk conditions-(class I)
Genetic syndromes like Marfan syn.,Ehlers-Danlos
Connective tissue diseases
F/h of aortic dissection
Known aortic valve disease
Recent aortic manipulation
Known aortic aneurysm

23. High risk pain features(classI)
Chest,back or abdominal pain
Abrupt in onset
Severe intensity
Ripping,tearing,stabbing or sharp

24. High risk exam features(classI)
Pulse deficit
Syst.BP limb diff.>20mmHg
Focal neurological deficit
Murmur of AR(new)
shock

26. 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

27. 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

28. 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

30. 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)

31. Recommendation for definitive management(classI)
Ascending aorta involved-emergent surgical repair
Desc. Aorta involved-managed medically unless lifethreatening complications
Malperfusion syndrome
Progression of dissection
Enlarging aneurysm
Inability to control BP or symptoms

32. 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

33. Class IIa
IMH-treated similar to aortic dissection in the corresponding segment

34. Intimal defect without IMH-
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

35. Thoracic aortic aneurysms
Rec.for medical Rx:classI
Antihypertensives to a goal<140/90(goal<130/80 for DM&CKD)
Beta blocker in Marfans syn.&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%

36. 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 Marfans syn(Brooks BS et al;nejm2008)
Statin use a/w decreased long term mortality in abd aortic aneurysm(Diehm N et al;2008)

37. 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

38. 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

41. 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.

42. 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

43. 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

44. 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

45. Thoraco abdominal aneurysm-surgery when diameter >6cm
End organ ischemia-additional revascularisation procedure recommended

46. 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,dilatation of asc.aorta.
If any abnormalities-annual imaging
Otherwise-repeat 5 to 10yr

47. Class IIa
Marfan syn planning pregnancy-replacement of aortic root and asc.aorta if >4.5cm

49. 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

50. classIIa
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

52. 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

53. Recommendations for takayasu arteritis &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

54. Recommendations in pregnancy
classI
Marfans syn 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

55. ClassIIa Class IIb CS for pts with significant aortic dilatation
If progressive aortic dilatation-prophylactic surgery recommended

56. 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

57. Aortic arch atheroma classIIa-Rx with a statin
Class IIb-oral anticoagulation with warfarin or antiplatelets in stroke pts with atheroma≥ 4 mm

58. Pre op evaluation for CAD
Class I-
thoracic aortic d/s undergoing intervention-evaluated for CAD
unstable cor syn-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

59. 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

60. 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 %
Total arch replacement-2-6%mortality,2-7% risk of stroke

61. Post op surveillance