biscupid aortic valve · 30 significant aortic regurg shone`s complex 60-85 left-ided obstructive...
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Bicuspid aortic valve
BAV
ZBIGNIEW GĄSIORKATEDRA i KLINIKA KARDIOLOGII SUM KATOWICE
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BAV
• Pathogenesis of the BAV is unknown
• NOTCH-1 mutations
• Unproper structure of the extracellular protein matrix, fibrilin-1 i fibulin deficiency, accelerated apoptosis of the smoth muscle cells, ↑ metaloproteinase, impaired arterial wall structure
• Familial prevalence (possible – 9%): autosomal dominant pattern with with reduced penetrance
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BAV - prevalence
• Approximately 1% of the population
• M / K 3 : 1
• Symptoms usually late (after 40 ys)
Diagnostics:
• TTE sensitivity 78%; specificity 96%
• TEE sensitivity 87%; specificity 91%
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BAV and associated cv conditionsCondition Incidence of BAV Comments
Coarctation of the aorta
50 Increased risk of aorticcomplications
Turner syndrome 30 Most frequent cardiacabnormality
Sinus of Valsalvaaneurysm
15-20 Frequently asympt; most commonly RCC
Ventricular septaldefect
30 Significant aorticregurg
Shone`s complex 60-85 Left-ided obstructivelesion
Ascending aorticdilatation
common BAV most common
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Prognosis
90+3% during 20 yrs (Olmstead study – 212 pat)
96+1% during 10 yrs (Toronto study – 642 pat)
Complications 25-40% persons in the age 44-52 andsevere/moderate stenosis or insufficiency
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Michelena HI et al. Circulation 2008; 117: 2776-84.
N=212 asymptomatic BAV, LVEF >50%, AR or AS absent or mild
20-year survival after diagnosis (90%) – similar to the general population
Independent predictors of CVE: age ≥ 50 years, valve degeneration after diagnosis
Baseline ascending aorta ≥ 40mm independently predicted surgery for aorta dilatation
Prognosis in BAV
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Michelena HI et al. Circulation 2008; 117: 2776-84.
N=212 asymptomatic BAV, LVEF >50%, AR or AS absent or mild
Early valve degeneration – risk factor for aortic valve surgery and medical events
Baseline ascending aorta ≥ 40mm independently predicted surgery for aorta dilatation
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Clinical forms of the BAV
• BAV without disturbances of flow
• Aortic regurgitation
• Valvular stenosis
• Aortic dilatation
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Clinical forms of the BAV
• BAV without disturbances of flow
• Aortic regurgitation
• Valvular stenosis
• Aortic dilatation
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Clinical forms of the BAV
• BAV without disturbances of flow
• Aortic regurgitation
• Valvular stenosis
• Aortic dilatation
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MSCT
Aortic root dilatation – max. 44mm asc. ao. – max. 67mm
Clinical forms of the BAV
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BAV: risk of aortic complication
• Expansion rate > 5mm/year
• Aortic stenosis
• Severe valvular dysfunction
• Hypertension
• Aortic coarctation
• Family history of aortic aneurysm complications
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Morfologic pattern BAV
Anterior – posterior (86%)
Right – left (12%)
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• Right-left pattern
• Anterior-posterior pattern
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Anterior-posterior pattern
TEE
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Right-left pattern
TEEAS + AR
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Summary
• BAV – one of the most common CHD affecting approximately 1% of the population
• Familial occurence in 9% of first-degree relatives
• Most pts with BAV will require surgical therapy for the valve and/or the aorta during their lifetime
• After BAV replacemment the patient is still at risk for aortic root complications