Bicuspid Aortic Valve Hemodynamic Consequences
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1 Bicuspid Aortic Valve Hemodynamic Consequences Hector I. Michelena, MD, FACC, FASE Associate Professor of Medicine Director, intra-operative Echocardiography
2 Disclosures None Happy and honored CP
3 Life is a long lesson in humility James Barrie "Everything should be kept as simple as possible, but no simpler" Albert Einstein
4 BAV Disease is old
5 Leonardo
6 Leonardo s notes Royal collection, Queen Elizabeth II
7 Leonardo s notes Royal collection, Queen Elizabeth II
8 BAV Look at the valve!
9 Diagnosis: Clinical first // Ejection click 60-70% click, systolic-diastolic murmur Michelena et al. Circulation 2008;117:2776
10 Phenotyping Michelena et al. Circulation 2014;129:
11 Heterogeneous Phenotypes 80% 20% 1%
12 True BAV 5%
13 Heterogeneous pheno + genotype BAV-- Innocent bystander? Coarctation Left-side obstruction; Shone s Turner Loeys-Dietz Milewicz DM et al. Genomics Hum Genet 2008;9: 283 Cedars A, Braverman AC. Progress in Pediatric Cardiology 2012;34:91
14 Most common association Aortopathy Heterogeneous Phenotypes
15 Heterogeneity Clinical correlate? Complex congenital Syndromic Pediatric Accelerated AS Fernandes et al. JACC 2007;49:2211 Young Adult AS / AR Aorta complic Adult BAV disease Michelena et al. Circulation 2014;129:2691
16 AdultTHE BAV disease VALVE HEMODYNAMIC CONSEQUENCES Intrinsic dysfx Stenosis Insufficiency Interaction Valvulo aortopathy Aortic dilatation Dissection
17 Life is a long lesson in humility Am J Cardiol, 1970
18 BAV Intrinsically dysfunctional VALVULAR IMPLICATIONS
19 Excessive folding/creasing Extended areas leaflet contact Morphologic stenosis Asymmetrical flow/turbulence
20 Valve under stress Stress = early leaflet degeneration?precursor of calcific
21 Even before stress Collagen Elastin Proteoglycan VICs Pediatric BAV with stenosis without calcification Loss of compartmentalization Disorganized VICs
22 Stress effects Cultured VICs High matrix stiffness Myofibroblast transformation Calcific aggregates with apoptosis
23 2012
24 All stress?? genetics? Familial BAV and aortopathy RARE AND FAMILIAL Michelena et al. Circulation 2014;129:2691
25 BAV VALVULAR OUTCOMES
26 Clinical outcomes Aortic valve replacement 53±5% Aortic surgery total Aneursym Coarctation Dissection % Years Michelena et al. Circulation 2014;129:2691 Michelena et al. JAMA 2011;306:1104
27 NO RELATION BETWEEN BAV PHENOTYPE AND AS / AR (different in cross-sectional) Michelena et al. Circulation 2014;129:2691
28 AS related to RN fusion AR related to LR fusion
29 BAV VALVULAR OUTCOMES PREDICT??
30 Natural History of Asymptomatic Patients with Normally Functioning or Minimally Dysfunctional Bicuspid Aortic Valve in the Community Hector I. Michelena, Valerie A. Desjardins, JeanFrançois Avierinos, Antonio Russo, Vuyisile T. Nkomo, Thoralf M Sundt, Patricia A. Pellikka, A. Jamil Tajik, Maurice Enriquez-Sarano Circulation 2008; 117: patients Mean age 32 yr, 65% male
31 Methods Valve degeneration score (DS) Score of 3 was considered valve degeneration at diagnosis Thickening Calcification Mobility Minimum DS= 0 Maximum DS= 9 0 points 1 point 2 points 3 points No or minimal abnormality mild or mod-1leaflet Mild-2 leaflets moderate- 2 leaflets Severe- 1 leaflet Severe- 2 leaflets Michelena et al. Circulation 2008;117:2776
32 Aortic Valve Surgery by Degeneration Score: Clinical implications DS 3 DS<3 75±10% P< % Years 0 No. at risk ±4% Michelena et al. Circulation 2008;117:2776
33 18 yo, echo 1981, DS=0 ( echo 2002 MG 10 mmhg)
34 45 yo, echo 1983, DS=4, ( echo 2005 MG 38 mmhg )
35 1995 DS= DS= 0 9 years later 2004 (AVR) 16 years later 2006 asymp
36 Valvular Outcomes CHF age 62±20 y vs 76±12 y (p ) AVR incidence 1370 vs 19 per 100,000 AVR age 49±20 y vs 67±16 y (p<0.0001) Michelena et al. Circulation 2008;117:2776
37 AdultTHE BAV disease VALVE HEMODYNAMIC CONSEQUENCES Intrinsic dysfx Stenosis Insufficiency Interaction Valvulo aortopathy Aortic dilatation Dissection
38 BAV Intrinsically dysfunctional AORTIC IMPLICATIONS
39 α
40 Mostly normal function valves AS increased shear stress
41 Right-anterior // AA Right-posterior // Ao and arch
42 AdultTHE BAV disease VALVE HEMODYNAMIC CONSEQUENCES Intrinsic dysfx Stenosis Insufficiency Interaction Valvulo aortopathy Aortic dilatation Dissection
43 Valvuloaortopathy
44 Root phenotype Ao/Arch phenotype
45 Most common dilatation irrespective of BAV type-aa Root dilatation linked to R-L fusion, male gender (p=0.0001) R-L fusion annulus/root/stj enlargement AR related to root progression Dr Delphine Detaint
46 Further evidence Root phenotype related to L-R fusion, males // AR AA phenotype related to R-N fusion, related to AS AR correlates with annular dilatation and increased root stiffness 21 patients S/P AVR for AR with large root followed for 10 years 2 dissections Della Corte et al. Eur J Cardiothorac Surg 2014 e118 Grotenhuis et al. JACC 2007;49:1660 Girdauskas et al. Eur J Cardiothorac Surg 2012;42:832
47 A conclusion is the place where you got tired of thinking BAV is intrinsically dysfunctional: Hemodynamic abnormalities: Stress-calcification // aortopathy AVR-AS is by far the most important complication //baseline degeneration is predictive R-L fusion related AR + proximal Ao disease (root phenotype) // male gender more malignant phenotype? R-N fusion related AS + mid-distal (arch) Ao disease
48
49 There is no disease more conducive to clinical humility than aneurysm of the aorta -William Osler
50 Bicuspid Aortic Valve
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