Priv.-Doz. Dr. med. Michael Kindermann

CaritasKlinikum Saarbrücken, St. Theresia Interventionelle Kardiologie und Angiologie

Aortic Regurgitation and Aneurysm:

Epidemiology and Guidelines

Homburg, Wednesday, May 14th, 2014

Workshop: Reconstruction of the

Aortic Valve and Root

cts CaritasKlinikum Saarbrücken St. Theresia

cts Some numbers - on aortic regurgitation

• Prevalence: 5 out of 10,000 people

• Third most frequent single left-sided valve disease (13%) in

patients admitted to medical or surgical cardiology departments

• Among single native left-sided valve disease, pure AR accounts

for 13% of all valve interventions

Iung B.: Eur Heart J;2003:24:1231.

Aortic valve replacement

with mechanical or bioprosthesis 94.1%

Replacement by homograft 02.5%

Replacement by autograft 01.7%

Aortic valve repair 01.7%

Interventions on AR in 2001 (Euro Heart Survey on Valvular Heart Disease):

cts Aortic regurgitation - Etiology

Abnormalities of the cusps Abnormalities of the aortic root Abnormalities of cusps & root

- Bicuspid valve (1-2% popul.)

- Unicuspid, quadricuspid valve

- Osteogenesis imperfecta

- Marfan syndrome

- Loeys-Dietz syndrome

Genetic - Ehlers-Danlos syndrome

- Familial thoracic aortic aneurysm

and dissection syndrome

- Idiopathic cystic medial necrosis

- Pseudoxanthoma elasticum

- Rheumatic valve disease

- Libman Sacks endocarditis (SLE)

Inflammatory

- Ankylosing spondylitis

- Reiter´s syndrome

- Behcet´s disease

- Syphilitic aortitis

- Takayasu arteritis

- Giant cell arteritis

Degenerative - Primary myxomatous degeneration

- Degenerative calcification

Abnormal loading - Hypertensive aortic root dilatation

Valve destruction - Infectious endocarditis

- Traumatic tear/avulsion of aortic cusp

- Traumatic aortic dissection

Drug side effects - Dopamine agonists

- Anorectic drugs

Degenerative 50%

Rheumatic 15%

Congenital 15%

Endocarditis 08%

Most frequent etiologies 2001 (Euro Heart Survey)

cts Aortic Regurgitation – Natural history

Bonow RO.: Circulation;2008:118:e523.

> 25% of patients who die or develop systolic dysfunction do so before

the onset of warning symptoms!

LV end-systolic dimension Death/symptoms/LV-dysfunction per year

< 40 mm 00%

40-50 mm 06%

> 50 mm 19%

LVESD = most important outcome predictor in patients with preserved EF

Asymptomatic % per year

• Normal LV function (good prognosis)

– Progression to asymptomatic LV dysfunction < 3.5 %

– Progression to symptoms or LV dysfunction < 6 %

– Sudden death rate < 0.2 %

• Abnormal LV function (poor prognosis)

– Progression to cardiac symptoms > 25 %

Symptomatic (poor prognosis)

– Mortality > 10 %

cts

Incidences:

TAA: 10.4 per 100.000 per year (m:f ∼ 1.7:1)

TAD: 2.9 per 100.000 per year (m:f ∼ 4:1 to 1:1)

Isselbacher EM in Baliga, Nienaber, Isselbacher, Eagle (eds): Aortic Dissection and Related Syndromes, Springer 2007.

Thoracic aortic aneurysms & dissections - Epidemiology

Causes:

Congenital connectice tissue disorders:

- Syndromes (Marfan, Loeys-Dietz, Ehlers-Danlos, Turner)

- Familial thoracic aortic aneurysm syndrome

Bicuspid aortic valve

Aortitis:

- Noninfective: Takasu´s arteritis, giant cell arteritis

- Infective: Syphylitic aortitis, mycotic aneurysm

Hypertension

Atherosclerosis

60%

10%

40%

10%

Pattern of involvement of TAAs:

Annual risk of rupture or dissection:

TAA < 5 cm → 2%

TAA 5.0-5.9 cm → 3%

TAA ≥ 6.0 cm → 7%

cts Valvular Heart Disease – International Guidelines

20

14

20

10

2

01

2

cts

Vahanian A.: Eur Heart J;2012:33:2451.

Management of AR – ESC Guidelines 2012

Significant enlargement of ascending aorta

No Yes

Other cardiac surgery (IC) LVEF 50% (IB) or LVEDD > 70 mm (IIaC) or LVESD > 50 mm (IIaC) or LVESD/BSA > 25 mm/m² (IIaC)

No

No Yes

Whatever the severity of AR

Cutoff for surgery dependent on maximal ascending aortic diameter

• Marfan syndrome - without risk factors 50 mm (IC) - with risk factors 45 mm (IIaC)

• Bicuspid aortic valve

- without risk factors 55 mm (IIaC)

- with risk factors 50 mm (IIaC)

• Others 55 mm (IIaC)

AR severe

No

Follow-Up Surgery

Yes

Symptoms

Yes

(IB)

cts

Bicuspid aortic valve with significant enlargement of ascending aorta

No Yes

Other cardiac surgery (IC) LVEF < 50% (IB) or LVESD > 50 mm (IIaB) or LVESD/BSA > 25 mm/m² (IIaB) or LVEDD > 65 mm & low risk (IIbC)

No

No Yes

Whatever the severity of AR - Without risk factors* 55 mm (IB) - With risk factors* 50 mm (IIaC) With severe AS or AR - No risk factors required 45 mm (IIaC)

*Risk factors:

Family history of aortic dissection

Increase rate ≥ 5 mm per year

AR severe

No

Follow-Up Surgery

Yes

Symptoms

Yes

(IB)

Management of AR – AHA/ACC Guidelines 2014

Nishimura RA.: Circulation 2014.

moderate AR & other cardiac surgery (IIaC)

cts AHA/ACC Guidelines 2010

Indications for aortic root repair in Marfan syndrome (IIaC):

Maximal aortic diameter 50 mm

Hiratzka LF.: Circulation;2010:121:e266.

unless - family history of aortic dissection at < 5 cm

- rapid expanding aneurysm (> 5 mm/year)

- significant valvular regurgitation

cts AHA/ACC Guidelines 2010

Indications for aortic root repair in Marfan syndrome (IIaC):

Maximal aortic diameter 50 mm

Hiratzka LF.: Circulation;2010:121:e266.

unless

Indications for aortic repair in Loeys-Dietz syndrome (IIaC)*:

Maximal aortic inner diameter (TEE measure) 42 mm Maximal aortic external diameter (CT, MRI) 44 - 46 mm *same recommendation for familial TAAD syndrom with TGFBR-mutations

Maximal aortic cross sectional area (d²/4) in cm² divided by height in meters > 10 applicable also to other genetic diseases or bicuspid aortic valves

- family history of aortic dissection at < 5 cm

- rapid expanding aneurysm (> 5 mm/year)

- significant valvular regurgitation

Prophylactiv aortic repair in women contemplating pregnancy if aortic diameter > 40 mm

NB.: Current AHA/ACC Valvular Disease Guidelines 2014 do not

recommend adjustments of aortic diameter for body size!

cts

Neither genetic syndrom, nor bicuspid valve:

Indications (IC) for aortic root repair or

replacement of the ascending aorta

(Class IC recommendations):

Diameter or aortic root or ascending aorta > 55 mm

Rate of diameter increase 5 mm/year

or

If AVR is indicated anyway,

cutoff diameter for aortic root/ascending aorta is > 45 mm

or

AHA/ACC Guidelines 2010

Hiratzka LF.: Circulation;2010:121:e266.

cts Medical therapy – AHA/ACC Guidelines 2010 & 2014

Thoracic aortic aneurysms

Stringent control of hypertension (IB), statins (IIaA), smoking

cessation (IB)

ß-blockers in Marfan syndrome (IB)

ARBs (losartan) in Marfan syndrome (IIaB)

ß-blockers and ACEIs/ARBs in all patients with thoracic aortic

aneurysms (IIaB)

Hiratzka LF.: Circulation;2010:121:e266. Nishimura RA.: Circulation 2014.

Aortic regurgitation

Stringent control of hypertension with calcium channel blockers

and/or ACEIs/ARBs (IB)

Heart failure treatment including ß-blockers and ACEIs/ARBs in

symptomatic severe AR when surgery is not an option (IIaB)

No routine vasodilator therapy in chronic asymptomatic AR with

normal LV function (III)

Thank you

for your

attention!

Akademisches Lehrkrankenhaus der Universität des Saarlandes

cts CaritasKlinikum Saarbrücken St. Theresia

cts

Surgery recommended with aortic diameter at or above 2.75 cm/m² Davies RD.: Ann Thorac Surg 2006;81:169.

What to do in small persons? – Aortic size index