The Bicuspid Aortic Valve: New Frontiers in Genetics and Interventions
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1 The Bicuspid Aortic Valve: New Frontiers in Genetics and Interventions Westfälische Wilhelms-Universität Münster Helmut Baumgartner Adult Congenital and Valvular Heart Disease Center Dept. of Cardiology and Angiology University Hospital Muenster Muenster, Germany
2 Bicuspid Aortic Valve Most frequent congenital heart defect Prevalence (2)% Male predominance (2-3 : 1) Most frequently fusion of right and left coronary cusp
3 Bicuspid Aortic Valve Most frequent congenital heart defect Prevalence (2)% Male predominance (2-3 : 1) Most frequently fusion of right and left coronary cusp (type 1)
4 Bicuspid Aortic Valve Fusion the right and noncor. cusps (type 2): earlier dysfunction/complications Rarely left and noncoronary cusp fusion, symmetric or without raphe
5 Bicuspid Aortic Valve GENETICS Heritable component to BAV generally accepted
6 Bicuspid Aortic Valve GENETICS Heritable component to BAV generally accepted Prevalence in first degree relatives appr. 10% Huntington K et al J Am Coll Cardiol 1997;30: Cripe L et al J Am Coll Cardiol 2004;44: In families with 1 member with BAV appr. 25% Glick BN and Roberts WC Am J Cardiol 1994;73:400-4
7 Bicuspid Aortic Valve GENETICS Heritable component to BAV generally accepted Prevalence in first degree relatives appr. 10% Huntington K et al J Am Coll Cardiol 1997;30: Cripe L et al J Am Coll Cardiol 2004;44: In families with 1 member with BAV appr. 25% Glick BN and Roberts WC Am J Cardiol 1994;73:400-4 Autosomal dominant with variable expressivity and reduced penetrance
8 Bicuspid Aortic Valve GENETICS Heritable component to BAV generally accepted Prevalence in first degree relatives appr. 10% Huntington K et al J Am Coll Cardiol 1997;30: Cripe L et al J Am Coll Cardiol 2004;44: In families with 1 member with BAV appr. 25% Glick BN and Roberts WC Am J Cardiol 1994;73:400-4 Autosomal dominant with variable expressivity and reduced penetrance Mutations in gene NOTCH1 other loci: chromosmes 15q, 18q, 5q, 13q
9 Bicuspid Aortic Valve GENETICS Heritable component to BAV generally accepted Prevalence in first degree relatives appr. 10% Huntington K et al J Am Coll Cardiol 1997;30: Cripe L et al J Am Coll Cardiol 2004;44: In families with 1 member with BAV appr. 25% Glick BN and Roberts WC Am J Cardiol 1994;73:400-4 Autosomal dominant with variable expressivity and reduced penetrance Mutations in gene NOTCH1 other loci: chromosmes 15q, 18q, 5q, 13q Screening? No evidence sufficient pick-up rate of BAV in 1st degree relatives Kerstjens-Frederikse WS et al Heart 2011;97:
10 Bicuspid Aortic Valve Congenital aortic stenosis aortic regurgitation Infective endocarditis - calcific aortic stenosis
11 Bicuspid Aortic Valve: Associated Pathololgies Dilation/aneurysm of the asc. aorta / arch Coarctation (50-75% BAV) Turner syndrome Coronary anomalies Supravalvular AS Ventricular septal defect Patent ductus arteriosus Shone complex Williams syndrome
12 Aortopathy in Bicuspid Aortic Valve TYPES of MORPHOLOGY
13 Aortopathy in Bicuspid Aortic Valve TYPES of Dilation and Valve Morph. right + left coronary right + acoronary
14 Aortopathy in bicuspid AV Morphology vs. Flow Pattern
15 xxxxx BAV Normal aorta Hope MD et al Radiology 2010; 255:53-61 Hope
16 Right-handed helical flow in BAV (right and left coronary cusp fusion) with aortic aneurysm Hope MD et al Radiology 2010;255:53-61
17 Left-handed helical flow in BAV (right and noncoronary cusp fusion) Hope Hope MD et al Radiology 2010; 255:53-61
18 Flow pattern in BAV Normal Flow Righthanded Lefthanded Aorta normal Aorta dilated Right-left coron. cusp fusion Right-noncoron. cusp fusion Hope MD et al Radiology 2010;255:53-61
19 Bicuspid Aortic Valve COMPLICATIONS Valvular dysfunction (stenosis / regurgitation) Infective endocarditis Degeneration (calcific AS) Aortic dilatation - dissection - rupture Sudden death
20 Bicuspid Aortic Valve New frontiers in intervention Aortopathy Aortic complications
21 Aortopathy in bicuspid AV The aorta is larger in pts with bicuspid aortic valves and dilates more rapidly than in tricuspid valves Beroukhim RS et al Am J Cardiol 2006;98: Gurvitz M et al Am J Cardiol 2004;94: Ciotti GR et al Am J Cardiol 2006;98:
22 Aortopathy in bicuspid AV The aorta is larger in pts with bicuspid aortic valves and dilates more rapidly than in tricuspid valves Beroukhim RS et al Am J Cardiol 2006;98: Gurvitz M et al Am J Cardiol 2004;94: Ciotti GR et al Am J Cardiol 2006;98: % of pts 35±16 years old present with aortic enlargement, progressing to 45% after 9 years FU (Progression rate 0.2mm[0-0.9]/yr) Tzemos N et al JAMA 2008;300:
23 Aortopathy in bicuspid AV The aorta is larger in pts with bicuspid aortic valves and dilates more rapidly than in tricuspid valves Beroukhim RS et al Am J Cardiol 2006;98: Gurvitz M et al Am J Cardiol 2004;94: Ciotti GR et al Am J Cardiol 2006;98: % of pts 35±16 years old present with aortic enlargement, progressing to 45% after 9 years FU (Progression rate 0.2mm[0-0.9]/yr) Tzemos N et al JAMA 2008;300: Olmstead County: Prevalence of asc. aorta >40mm: 15% with increase to 39% at study conclusion Michelena HI et al Circulation 2008;117:
24 Aortopathy in bicuspid AV The aorta is larger in pts with bicuspid aortic valves and dilates more rapidly than in tricuspid valves Beroukhim RS et al Am J Cardiol 2006;98: Gurvitz M et al Am J Cardiol 2004;94: Ciotti GR et al Am J Cardiol 2006;98: % of pts 35±16 years old present with aortic enlargement, progressing to 45% after 9 years FU (Progression rate 0.2mm[0-0.9]/yr) Tzemos N et al JAMA 2008;300: Olmstead County: Prevalence of asc. aorta >40mm: 15% with increase to 39% at study conclusion Michelena HI et al Circulation 2008;117: Risk factors: age, hypertension, male gender, significant valvular disease (increased stroke volume in aortic regurgitation)
25 Aortopathy in bicuspid AV Zystistic media degeneration Inbalance between proteolytic enzymes and their inhibitators (increased apoptosis, higher grade of elastic fragmentation, increased MMP-2 expression) Decreased content of fibrillin-1 (primary or sec.?) Disturbed TGF-ß signalling Normal Aneurysma GENETICS: Prevalence in 1st degree relatives 9% Autosomal dominant with variable Expressivity and reduced penetrance (15q, 18q, 5q, 13q, NOTCH1) Abnormal neural crest migration (AV, Ao, PA)
26 Aortopathy in bicuspid AV Cause? CONGENITAL Independent of valve dysfunction Pathology of pulmonary artery Progression after valve replacement Systemic vasculopathy Aortopathy in relatives with tricuspid valve HEMODYNAMIC
27 Aortopathy in bicuspid AV Cause? CONGENITAL Independent of valve dysfunction Pathology of pulmonary artery Progression after valve replacement Systemic vasculopathy Aortopathy in relatives with tricuspid valve HEMODYNAMIC Experimental data Increase with severity of aortic regurgitation Regional pathology (histo, humoral...) Low progression rate after AVR No pulmonary artery involvement CMR flow analysis
28 Aortopathy in bicuspid AV CONGENITAL Independent of valve dysfunction Pathology of pulmonary artery Progression after valve replacement Systemic vasculopathy Aortopathy in relatives with tricuspid valve Cause? + HEMODYNAMIC Experimental data Increase with severity of aortic regurgitation Regional pathology (histo, humoral...) Low progression rate after AVR No pulmonary artery involvement CMR flow analysis
29 Aortic Dissection and BAV Actual incidence of aortic dissection in pts. with BAV is controversially debated Guntherroth WG. Am J Cardiol 2008;102:107-10
30 Aortic Dissection and BAV Actual incidence of aortic dissection in pts. with BAV is controversially debated Guntherroth WG. Am J Cardiol 2008;102: Pooled estimate of cases of dissection associated with BAV was 4% Larson EW, Edwards WD. Am J Cardiol 1984;53: Roberts CS, Roberts WC. J AM Coll Cardiol 1991;17:712-6 Guntherroth WG. Am J Cardiol 2008;102:107-10
31 Aortic Dissection and BAV Actual incidence of aortic dissection in pts. with BAV is controversially debated Guntherroth WG. Am J Cardiol 2008;102: Pooled estimate of cases of dissection associated with BAV was 4% Larson EW, Edwards WD. Am J Cardiol 1984;53: Roberts CS, Roberts WC. J AM Coll Cardiol 1991;17:712-6 Guntherroth WG. Am J Cardiol 2008;102: Toronto series: 0.1%/patient-year Tzemos N et al JAMA 2008;300:
32 Aortic Dissection and BAV Actual incidence of aortic dissection in pts. with BAV is controversially debated Guntherroth WG. Am J Cardiol 2008;102: Pooled estimate of cases of dissection associated with BAV was 4% Larson EW, Edwards WD. Am J Cardiol 1984;53: Roberts CS, Roberts WC. J AM Coll Cardiol 1991;17:712-6 Guntherroth WG. Am J Cardiol 2008;102: Toronto series: 0.1%/patient-year Tzemos N et al JAMA 2008;300: Olmstead County study: no cases of dissection Michelena HI et al Circulation 2008;117:
33 Aortic Dissection and BAV Actual incidence of aortic dissection in pts. with BAV is controversially debated Guntherroth WG. Am J Cardiol 2008;102: Pooled estimate of cases of dissection associated with BAV was 4% Larson EW, Edwards WD. Am J Cardiol 1984;53: Roberts CS, Roberts WC. J AM Coll Cardiol 1991;17:712-6 Guntherroth WG. Am J Cardiol 2008;102: Toronto series: 0.1%/patient-year Tzemos N et al JAMA 2008;300: Olmstead County study: no cases of dissection Michelena HI et al Circulation 2008;117: Dissection is more common in dilated aortas but has also been reported in normal-sized aorta Russo CF et al Ann Thorac Surg 2002;74:S1773-6
34 Aortic Dissection and BAV Actual incidence of aortic dissection in pts. with BAV is controversially debated Guntherroth WG. Am J Cardiol 2008;102: Pooled estimate of cases of dissection associated with BAV was 4% Larson EW, Edwards WD. Am J Cardiol 1984;53: Roberts CS, Roberts WC. J AM Coll Cardiol 1991;17:712-6 Guntherroth WG. Am J Cardiol 2008;102: Toronto series: 0.1%/patient-year Tzemos N et al JAMA 2008;300: Olmstead County study: no cases of dissection Michelena HI et al Circulation 2008;117: Dissection is more common in dilated aortas but has also been reported in normal-sized aorta Russo CF et al Ann Thorac Surg 2002;74:S Risk factors for dissection: aortic size, aortic stiffness, male sex, family history, presence of other lesions such as coarction and Turner syndrome
35 BAV vs Marfan Syndrome Cystic medial necrosis is present in both Nataatmadja M et al Circulation 2003;108 Medial degeneration in only 18% of 161 pts. with aoritc dissection Larson EW, Edwards WD AJC 1984;53:849 Pulmonary artery involvement: controversial: dilatation less common in BAV, histopath. abnormalities in both Presence of BAV and Marfan similar in dissection series / BAV 1-2/100 vs Marfan 1/10000 Control Marfan BAV
36 Natural history of asym. pts. with normally functioning or minimally dysfunctional BAV in the community Michelena HI et al Circulation 2008;117:
37 Natural history of asym. pts. with normally functioning or minimally dysfunctional BAV in the community Michelena HI et al Circulation 2008;117:
38 Natural history of asym. pts. with normally functioning or minimally dysfunctional BAV in the community Michelena HI et al Circulation 2008;117:
39 Natural history of asym. pts. with normally functioning or minimally dysfunctional BAV in the community Michelena HI et al Circulation 2008;117:
40 Natural history of asym. pts. with normally functioning or minimally dysfunctional BAV in the community Michelena HI et al Circulation 2008;117:
41 Outcomes in Adults with BAV Tzemos N et al JAMA 2008;300:
42 Outcomes in Adults with BAV Riks factors: Age>30 - AS mod/sev AR mod/sev Tzemos N et al JAMA 2008;300:
43 Incidence of Aortic Complications in BAV Olmsted county, retrospective analysis of 416 consecutive BAV pts 2/ / pt. years Age adjusted RR 8.4 Michelena HI N et al JAMA 2011;306:
44 Incidence of Aortic Complications in BAV Olmsted county, retrospective analysis of 416 consecutive BAV pts Michelena HI N et al JAMA 2011;306:
45 Incidence of Aortic Complications in BAV Olmsted county, retrospective analysis of 416 consecutive BAV pts Michelena HI N et al JAMA 2011;306:
46 Dilation of the Ascending Aorta: AORTIC SURGERY Elective aortic surgery is indicated: Marfan syndrome 50mm IC Marfan syndrome 45mm with RF IIaC Bicuspid aortic valve 50mm with RF* IIaC Other Pathologies 55mm IIaC * Coarctation, hypertension, family history of dissection, increase in DM >2mm on repeat measurements ESC-Guidelines VHD Update 2012
47 Bicuspid Aortic Valve (BAV) BAV is frequent (appr. 1% of population) BAV is associated with aortic stenosis, aortic regurgitation, endocarditis, aortopathy and sudden cardiac death BAV is heritable (appr. 10% of first degree relatives) and family screening can therefore recommended Longterm outcome is mainly related to the degree of valular pathology and dysfunction Aortopathy requires particular attention but intervention should be much less aggressive than in Marfan s syndrome
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