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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