Bicuspid Aortic Valve Hemodynamic Consequences Hector I. Michelena, MD, FACC, FASE Associate Professor of Medicine Director, intra-operative Echocardiography
Disclosures None Happy and honored CP1293058-2
Life is a long lesson in humility James Barrie "Everything should be kept as simple as possible, but no simpler" Albert Einstein
BAV Disease is old
Leonardo 1452-1519
Leonardo s notes Royal collection, Queen Elizabeth II
Leonardo s notes Royal collection, Queen Elizabeth II
BAV Look at the valve!
Diagnosis: Clinical first // Ejection click 60-70% click, systolic-diastolic murmur Michelena et al. Circulation 2008;117:2776
Phenotyping Michelena et al. Circulation 2014;129:2691-2704
Heterogeneous Phenotypes 80% 20% 1%
True BAV 5%
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
Most common association Aortopathy Heterogeneous Phenotypes
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
AdultTHE BAV disease VALVE HEMODYNAMIC CONSEQUENCES Intrinsic dysfx Stenosis Insufficiency Interaction Valvulo aortopathy Aortic dilatation Dissection
Life is a long lesson in humility Am J Cardiol, 1970
BAV Intrinsically dysfunctional VALVULAR IMPLICATIONS
Excessive folding/creasing Extended areas leaflet contact Morphologic stenosis Asymmetrical flow/turbulence
Valve under stress Stress = early leaflet degeneration?precursor of calcific
Even before stress Collagen Elastin Proteoglycan VICs Pediatric BAV with stenosis without calcification Loss of compartmentalization Disorganized VICs
Stress effects Cultured VICs High matrix stiffness Myofibroblast transformation Calcific aggregates with apoptosis
2012
All stress?? genetics? Familial BAV and aortopathy RARE AND FAMILIAL Michelena et al. Circulation 2014;129:2691
BAV VALVULAR OUTCOMES
Clinical outcomes Aortic valve replacement 53±5% Aortic surgery total Aneursym Coarctation Dissection % 416 416 342 375 280 327 Years 160 196 83 104 29 40 Michelena et al. Circulation 2014;129:2691 Michelena et al. JAMA 2011;306:1104
NO RELATION BETWEEN BAV PHENOTYPE AND AS / AR (different in cross-sectional) Michelena et al. Circulation 2014;129:2691
AS related to RN fusion AR related to LR fusion
BAV VALVULAR OUTCOMES PREDICT??
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: 2776-84 1980-1999 212 patients Mean age 32 yr, 65% male
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
Aortic Valve Surgery by Degeneration Score: Clinical implications 100 80 60 DS 3 DS<3 75±10% P<0.0001 % 40 20 0 Years 0 No. at risk 28 184 17±4% 5 10 15 20 23 176 9 130 2 79 2 31 Michelena et al. Circulation 2008;117:2776
18 yo, echo 1981, DS=0 ( echo 2002 MG 10 mmhg)
45 yo, echo 1983, DS=4, ( echo 2005 MG 38 mmhg )
1995 DS= 5 1990 DS= 0 9 years later 2004 (AVR) 16 years later 2006 asymp
Valvular Outcomes CHF age 62±20 y vs 76±12 y (p 0.0002) 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
AdultTHE BAV disease VALVE HEMODYNAMIC CONSEQUENCES Intrinsic dysfx Stenosis Insufficiency Interaction Valvulo aortopathy Aortic dilatation Dissection
BAV Intrinsically dysfunctional AORTIC IMPLICATIONS
α
Mostly normal function valves AS increased shear stress
Right-anterior // AA Right-posterior // Ao and arch
AdultTHE BAV disease VALVE HEMODYNAMIC CONSEQUENCES Intrinsic dysfx Stenosis Insufficiency Interaction Valvulo aortopathy Aortic dilatation Dissection
Valvuloaortopathy
Root phenotype Ao/Arch phenotype
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
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
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
There is no disease more conducive to clinical humility than aneurysm of the aorta -William Osler
Bicuspid Aortic Valve michelena.hector@mayo.edu