Management of Aortic Aneurysms &Management of Aortic Aneurysms & Aortic Dissections.

Miss Indu DeglurkarMiss Indu Deglurkar

Consultant Cardiothoracic Surgeon

University Hospital of Wales

Cardiff

Aetiology of Ascending Aortic gy gAneurysms

Degenerative

BAV

Syndromic Syndromic

Marfans

Loeys- Dietz

Ehler- Danlos

Turner/Noonan

Familial

Aetiology of Ascending Aortic Aetiology of Ascending Aortic Aneurysms

-

Infections: Syphilis

-Bacterial infections

Auto-immune arthritis Auto immune arthritis

Takayasu

B h t di Behçet disease

Idiopathic aortitis

Post-traumatic

Chronic aortic dissection

Ascending Aortic AneurysmsAscending Aortic Aneurysms

Detection is essential because of the indolent nature & lethality of the disease.indolent nature & lethality of the disease.

Detection is difficult because thoracic aortic aneurysm is a silent disease – a ‘silent killer’silent killer

Management of Ascending Aortic g gAneurysms

Medical TreatmentMedical TreatmentFollow-up by Imaging TechniquesFollow-up by Imaging TechniquesPredictors of complicationspSurgical treatment: IndicationsType of surgery

Management of Ascending Aortic g gAneurysms: Medical Management

A i Aggressive blood pressure controlBeta blocker preferred

If contraindicated: ACEI/ARBsIf contraindicated: ACEI/ARBs

Follow-up of aortic size by close imaging techniques

N EngJ Med 1994 330.1335-1341

BiomarkersBiomarkers

We need a biomarker to identify this asymptomatic, indolent & lethal diseaseindolent & lethal disease

‘RNA Signature’ blood test may be suitable for g yapplication to at-risk populations,

Sh ll t iti it d ifi it Shows excellent sensitivity and specificity,

Overall accuracy of 80% Overall accuracy of 80%

Distinguishes familial from non-familial aneurysms and ascending from descending aneurysms.

Other BiomarkersOther Biomarkers

Matrix metalloproteinases

Inflammatory markers: CRP, CD4 count

Markers of collagen turnover: elastin peptidespeptides

Endothelins and hepatocyte growth p y gfactor

Follow-up Imaging TechniquesFollow-up Imaging Techniques

Aortic root: Echo image of dilated aortic Aortic root:

2D-echo annually

groot

MRI aorta2D echo annually &

every 6m when the bsol te si e >45

o

absolute size >45mm for significant AR

Tubular AA: MRI and CT to Tubular AA: MRI and CT to evaluate the entire aorta

Inherent level of resolution in current imaging technology

In clinical practice

Not significant change if <3-4mm

Frequent mistakes Frequent mistakes

-Not to use the same imaging technique

-Axial measurements may

exaggerate diameter in elongated ascending aorta

-Motion artefacts can adversely affect the resolution of CT images producing changes as great as 7.5% to 27.5%

Gated CT angiography-

Mean Age & Size at gpresentation

Age Size

Hypertension 64.2yrs 60mm

Marfans 24 5yrs 51mmMarfans 24.5yrs 51mm

BAV 49yrs 52mmy

Loeys-Dietz 19.8yrs 40-50mm

Ehler Danlos No data No data

Yearly risk of rupture, dissection or y p ,death related to thoracic aortic size

Risk of aortic rupture related to pdiameter and BSA

Risk of ruptureRisk of rupture

<4 cms 0%

4.0-5.9cms 16%

>6 31% >6 cms 31%

The one-, three-, and five-year survival of unoperated thoracic aneurysms was 65, 36, and 20 percent, respectively

“Hinge points” for lifetime natural history complications at various sizes of history complications at various sizes of

aorta

Aortic diameter >5.5cms is not a ood edicto of T e A Ao tic good predictor of Type A Aortic

DissectionDissection

Limited value of aortic size

<50mmsurgery would fail to prevent 40% of aortic dissection

Dilatation is only one manifestation of aortic wall disease

Circulation2007; 116:1120 Circulation2007; 116:1120

Marfans vs BAVMarfans vs BAV

Marfans has a high lifetime grisk (40%) of aortic dissection

BAV disease carries a 6.1% life time risk of aortic dissection (9 fold higher than general population)

BAV is 100 times more common than Marfans

S i l ith Tho cic A e s sSurvival with Thoracic Aneurysms

Elefteriades et al Radiology 199,211:889

I dices fo o lisi di ete sIndices for normalising diameters

Aortic root diameter ratio Aortic root diameter ratio:

Observed/maximum predicted>1.3

AA area/height>10

AA diameter/BSA > 2.75 mm/cm2

SurgerySurgery

Indications for elective surgery g yin Ascending Aortic Aneurysm

Aortic diameter >55mm>55mm

Evaluate comorbidities

Risk of surgery

Age

Indications for elective surgery in g ythe Bicuspid Aortic valve

Aortic diameter > 55mm Aortic diameter > 55mm

Aortic diameter> 50mm if: -Aortic coarctation

-First degree family relative with AoD/rupture

- Small body size:

Aortic diameter/BSA > 2.75cm/m2

- Severe AS or AR without surgical criteria

Expansion rate> 2mm/yr- Expansion rate> 2mm/yr

Aortic diameter > 45mm with concomitant indication for elective AVR

Indications for elective surgery g yin Marfans syndrome

Ascending aortic diameter > 50mmg->45mm with risk factors

First degree with ascending aortic dissection/rupture-First degree with ascending aortic dissection/rupture.

-Concomitant indication for AV surgery

-Ratio of aortic diameter to BSA >2.75cm/m2

-Expansion rate > 2mm/yr

>40mm if pregnancy is desired and AV repair not required.repair not required.

SurgerySurgery

Aortic root with a composite valved graft

Ascending aortic replacement/ hemiarch

Valve sparing root replacement.p g p

A di & h i h l tAscending & hemiarch replacement

ConclusionsConclusions

Although Marfan’s & BAV present wall abnormalities that cause aortic wall weakness and its progressive dilatation, the risk of p g ,dissection/rupture is higher in Marfans syndrome.

In patients with Marfans with risk factors surgery should be In patients with Marfans with risk factors, surgery should be considered when diameter is 45mm.

In BAV without severe dysfunction of the valve timing of In BAV, without severe dysfunction of the valve, timing of ascending aorta surgery( 50 vs 55mm) should be individualised considering the presence of aortic coarction, body size, progressive dilatation, age & comorbidities. With concomitant indication of AVR aortic surgery should be performed when diameter is >45mm diameter is >45mm

HistoricallyHistorically

The first case of aortic dissection described was in the post-mortem examination of King George II of Great Britain in 1760.

Surgery for aortic dissection was introduced in the 1950s. Since DeBakey first reported his surgery for y p g yaneurysm the techniques have steadily advanced.

Since the 1990s Endovascular Repair has been used in Since the 1990s Endovascular Repair has been used in specific cases.

Risk factors for aortic dissectionRisk factors for aortic dissection

Medial degeneration. Marfan syndrome,g y ,

Loeys-Dietz syndrome,

Vascular form of Ehlers-Danlos syndrome, inflammatory diseases of the aorta,

Turner’s syndrome,

Bicuspid aortic valve Bicuspid aortic valve,

Familial thoracic aortic aneurysm and dissection dsyndrome

Risk factors for aortic dissectionRisk factors for aortic dissection

Increases wall stress: Hypertension

h h pheochromocytoma,

cocaine use

coarctation.

Physical trauma: weightlifting, deceleration injury in motor vehicle accidents

Smoking also increases dissection risk by affecting TGF-β.

Prior cardiac surgery

ClassificationClassification

class 1 =Classic aortic dissection class 1 Classic aortic dissection

class 2= Intramural haematoma/haemorrhage

class 3= Subtle-discrete aortic dissection

class 4= Plaque rupture/ulceration class 4= Plaque rupture/ulceration

class 5=Traumatic/iatrogenic aortic dissection

Operative indications for acute and pchronic Type A & B dissections

Acute Type A : Presence Acute Type A : Presence

Type B: Rupture, malperfusion, progressive dissection failure of medical managementdissection, failure of medical management

Chronic: type A: Symptoms related to dissection

- CCF, AR, pain, stroke, angina

- Type B: Symptoms, malperfusion, aneurysm.

Controversies in management gof AoD

>80 f >80 yrs of age

Neurological injuriesNeurological injuries

Late presentationsLate presentations

Previous cardiac surgeryPrevious cardiac surgery

5 tenets of Type A AoD repair

M di l t ti Myocardial protection

Cerebral protection Cerebral protection

Restoration of competent aortic valve

Excision of intimal tear site

Elimination of flow in false lumen blood flow and maintenance of true lumen blood flow

Goals of repair of AoDGoals of repair of AoD

P i l f A D C t ith Primary goal of AoD: Come out with a live patient

Not to obliterate the false lumen!

Majority will have a patent false lumen

O ti lt f A t T A/ BOperative results of Acute Type A/ B

Post operative mortality for Type A: 24%

5 yr survival: 55% - 75%

10 i l 32% 65% 10 yr survival: 32% - 65%

Post operative mortality for Type B: 28 – 65%p y yp

5 yr survival: 48%

10 yr survival: 28%

Results of Chronic Type A/B yprepair

Post operative mortality for Chronic Type A: 10-17%%17%%

5 yr survival: 59% - 75%

10 yr survival: 45%

P t ti t lit f h i T B 11% Post operative mortality for chronic Type B: 11% -15%

5 yr survival: 48%

10 yr survival: 28% 10 yr survival: 28%

Median diameter at time of rupture

Ascending aorta 5.9cms

Descending aorta 7.2cms

Long-Term Survival in Patients Long Term Survival in Patients Presenting With Type B Acute Aortic Di iDissection

Thomas T. Tsai (Circulation. 2006;114:2226-2231.

242 consecutive patients discharged alive with acute type B aortic dissection enrolled in the International Registry of Acute Aortic Dissection (IRAD) between 1996 and 2003

Contemporary follow-up mortality in patients who survive to hospital discharge with acute p gtype B aortic dissection is high, approaching 1 in every 4 patients at 3 yearsin every 4 patients at 3 years.

Endovascular Repair of Type B Aortic DissectionEndovascular Repair of Type B Aortic DissectionLong-Term Results of the Randomized Investigation of Stent Grafts in Aortic Dissection TrialStent Grafts in Aortic Dissection TrialChristoph A. Nienaber, MD, PhD, Stephan Kische, MD,Hervé Rousseau, MD, PhD,Holger Eggebrecht, MD, Tim C. Rehders, MD,Guenther Kundt, MD, PhD,Aenne Glass, MA,Dierk Scheinert, MD, PhD, Martin Czerny, MD, PhD,

il l i f ld kh i f l i b ll i Tilo Kleinfeldt, MD,Burkhart Zipfel, MD,Louis Labrousse, MD,Rossella Fattori, MD, PhD, Hüseyin Ince, MD, PhD,

TEVAR in addition to optimal medical treatment is associated with improved 5-year survival and delayed disease progression.

Randomized Comparison of Strategies for Type B Aortic Dissection The INvestigation of STEnt Grafts in Aortic Dissection (INSTEAD) Trial

Christoph A et al

Circulation December 22/29, 2009

In the first randomized study on elective stent-graft In the first randomized study on elective stent graft placement in survivors of uncomplicated type B aortic dissection, TEVAR failed to improve 2-year aortic dissection, TEVAR failed to improve 2 year survival and adverse event rates despite favorable aortic remodelingaortic remodeling.

E d l f l i Endovascular stent graft placement in thoracic aortic aneurysms and ydissectionsIssued: June 2005NICE

is a suitable alternative to surgery in is a suitable alternative to surgery in appropriately selected patients.

Th k YThank You

Pathophysiology of Aortic p y gyAneurysms

Mechanisms

-Congenital aortic fragility from genetic predisposition-Congenital aortic fragility from genetic predisposition

-Mechanical stress

Aortic media affected by damage and repair events

-Excessive injury: Valve dysfunction, hypertension, age

Impaired repair: Connective tissue disorders

International Registry of Acute g yAortic Dissection (IRAD)2000

12 International referral centres 6 countries

Coordination centre at University of Michigan

A total of 464 patientsp

62.3% had type A dissection

Common symptomsy p(IRAD)

Chest pain → 84.8%

Aortic regurgitation → 31.6%

Pulse Deficit → 15.1%

Hypertension → 70.1% (in type B)

Diagnosis g(IRAD)

Chest X-Ray → No Abnormality in 12.4%

ECG → No Abnormality in 31.3%

CT Scan → used in 61.1% of cases

Echocardiography →used in 32.7%

Outcome (IRAD) ( )Overall in hospital 27.4% Mortality

Type A

S (72% ) 26%Surgery (72% )→ 26%

Medical → 58% advanced age ,comorbidity

Type B

S rger (20%) 31 4%Surgery (20%) → 31.4%

Medical → 10.7%

Cause of DeathCause of Death

Type A

A i d 41 6%Aortic rupture or tamponade → 41.6%

Visceral Ischemia → 13.9%

Type B

Aortic r pt re 38 5%Aortic rupture → 38.5%

Visceral Ischemia →15.4%

Initial diagnostic steps in the g pemergency room

ECG must be acquired in all patients. 20% of patients with type A dissection have ECG evidence of acute ischaemia or ypacute myocardial infarction

The chest X-ray is not sufficient to rule out aortic dissection

Guidelines for the Diagnosis and M t f P ti t With Management of Patients With Thoracic Aortic Disease

March 2010ACC/AHA Guideline

Class IClass I

Aortic imaging is recommended for first-degree relatives g g gof patients with thoracic aortic aneurysm and/or dissection to identify those with asymptomaticdisease.

Initial ManagementInitial Management

Class I

Initial management of thoracic aortic dissection should be directed at decreasing aortic wall stress by controlling heart rate and blood pressurerate and blood pressure

Management of haemodynamically unstable g y ypatients with suspected aortic dissection

RecommendationsRecommendations

1. Profound haemodynamic instability: intubation and ventilation I C

2. Transoesophageal echocardiography as the sole diagnostic procedure — IIC

3. Surgery-based on findings of cardiac tamponade by transthoracicechocardiography II C

4. Pericardiocentesis (lowers intrapericardial pressure (recurrent bleeding!) III C

6.Reduction of systolic blood pressure using beta-blockers (i.v. propranolol, metoprolol, esmolol or labetalol) → IC

7. Transfer to intensive care unit → I C

8. In patients with severe hypertension additional vasodilator (i.v. sodium nitroprusside to titrate BP to 100–120 mmHg) → IC

9. In patients with obstructive pulmonary disease, blood pressure lowering with calcium channel blockers → IIC

Long term survival type ALong-term survival type A

Surgically managed patients had a follow-up mortality of 13.9%, mean survival 2.5 years %, y

medically managed patients had a mortality of 36.7%, mean survival 2 1mean survival 2.1

10. Imaging in patients with ECG signs of ischaemia before thrombolysis if aortic pathology is suspected → IICbefore thrombolysis if aortic pathology is suspected → IIC

11 Chest X ray → III C11. Chest X-ray → III C

Cl Class I

A l di h ld b b i d ll i An electrocardiogram should be obtained on all patients who present with symptoms that may represent acute h i i di ithoracic aortic dissection

h l f h i h l i f ibl The role of chest x-ray in the evaluation of possible thoracic aortic disease should be directed by the

i ’ i k f dipatient’s pretest risk of disease

Subsequent ManagementSubsequent Management

Class I

Acute thoracic aortic dissection involving theg

ascending aorta should be urgently evaluated for emergent surgical repair because of the high risk of associated life-surgical repair because of the high risk of associated lifethreatening complications such as rupture.

Class I

For patients with ascending thoracic aortic dissection, all aneurysmal aorta and the proximal , y pextent of the dissection should be resected. A partially dissected aortic root may be repaired with p y y paortic valve resuspension.Extensive dissection of the aortic root should be treated with aortic root replacement with a composite graft or with a valve sparing root replacement. If a DeBakey Type II p g p y ypdissection is present, the entire dissected aorta should be replacedp

Class IIa

It is reasonable to treat intramural hematoma similar to aortic dissection in the corresponding segment of the in the corresponding segment of the aorta

Follow upFollow-up

Pathology Interval Study Study

Acute dissection Before discharge, 1, 6 mo, yearly CT orMR, TTE

Chronic dissection Before discharge, 1 y, 2 to3 y CT ,MR, TTE

Aortic root repair Before discharge, yearly TTE

AVR plus ascending Before discharge, yearly TTE

Aortic arch Before discharge, 1 y, 2 3 y CT ,MR, chest

Thoracic aortic stent Before discharge, 1, 2, 6 mo, yearly CXR,CT

Acute IMH/PAU Before discharge, 1, 3, 6 mo, yearly CT , MR

Recommendations for Thoracic Ste t G ft I se tioStent Graft Insertion

1-Penetrating ulcer/intramural hematoma

Asymptomatic III

Symptomatic IIa

2-Acute traumatic I

3-Chronic traumatic IIa

4-Acute Type B dissection

Ischemia I

No ischemia IIb

5-Subacute dissection IIb

Chronic dissection IIb