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

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Presentation on theme: "Guidelines for the diagnosis and management of patients with thoracic aortic disease Circulation 2010;121."— Presentation transcript:

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

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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

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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 – Availability – Entire aorta imaged – Short time – Branch vessel invt. Ecg gating,MDCT

14 MRI 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

15 Echocardiography 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 <.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

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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

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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

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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 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

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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

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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

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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 – CS for pts with significant aortic dilatation Class IIb – If progressive aortic dilatation-prophylactic surgery recommended

56 Aortic dissection in pregnancy Type A- – 1 st or 2 nd TM-urgent surgical repair and fetal monitoring – 3 rd 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


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