Mitral valve apparatus in Hypertrophic Cardiomyopathy: a specific assessment?
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1 Nancy, September 17th, 2015 Mitral valve apparatus in Hypertrophic Cardiomyopathy: a specific assessment? Inserm UMR1087 Institut du Thorax, Nantes Thierry le Tourneau
2 Déclaration de Relations Professionnelles - Disclosure Statement of Financial Interest J'ai actuellement, ou j'ai eu au cours des quatre dernières années, une affiliation ou des intérêts financiers ou intérêts de tout ordre avec une société commerciale ou je reçois une rémunération ou des redevances ou des octrois de recherche d'une société commerciale : I currently have, or have had over the last five years, an affiliation or financial interests or interests of any order with a company or I receive compensation or fees or research grants with a commercial company : Affiliation/Financial Relationship Company Consulting Fees/Honoraria Congress Invitation-Meeting lunch GE healthcare, Philips BMS Daiichi-Sankyo Edwards Daiichi-Sankyo Servier Edwards, Abbott
3 Mitral Valve Apparatus in HCM - Echocardiography (leaflets +++) - Cardiac Magnetic Resonance Imaging (PM +++) - 3D Echo - Exercise Echocardiography +++
4 Pathophysiology: Mitral remodeling - Anomalies of Mitral valve apparatus Congenital: development alteration Acquired: remodeling of mitral valve apparatus associated with myocardial remodeling - Alterations : Mitral leaflets Chords Papillary muscles
5 Mitral Valve Anomalies of HCM
6 Mitral Valve: Lengthening Anterior Leaflet Length: 26 5 mm vs 19 5 mm Posterior Leaflet Length: 14 4 vs 10 3 mm Primary phenotypic trait Involved in LVOT obstruction 42mm Kim DH, Circulation 2010; 122: Maron MS, Circulation 2011; 124: 40-47
7 Mitral Valve: Lengthening Kim DH, Circulation 2010; 122: Maron MS, Circulation 2011; 124: 40-47
8 Mitral Valve: Lengthening Kim DH, Circulation 2010; 122: Maron MS, Circulation 2011; 124: 40-47
9 Chords: abnormal chordal insertion Patel P, Circ CV Img 2015; 8: epub
10 Papillary Muscles: Hypertrophy - Hypertrophy (CMR Img: 6±2 vs 3±2 g/m²) associated with increased (88%) or normal LV mass and localized wall thickness (12%) Harrigan CJ, Am J Cardiol 2008; 101:
11 Papillary Muscles: Hypertrophy Kwon DH, Heart 2008; 94:
12 Papillary Muscles: Hypertrophy Harrigan CJ, Am J Cardiol 2008; 101:
13 Papillary Muscles: Number - Multiples PM: 2.5 in 200 HCM vs 2.1 in 43 controls (P<0.001) - Accessory PMs (6%) Harrigan CJ, Am J Cardiol 2008; 101:
14 Papillary Muscles: Bifid - Double bifid PM (70% vs 17% in controls) Double bifid Kwon DH, Heart 2008; 94:
15 Papillary Muscles: Antero position - Antero-apical displacement of anterolateral PM (77% vs 17%, P<0.001) Normal PMs Abnormal PMs Kwon DH, Heart 2008; 94:
16 Papillary Muscles Anterior displacement Odds ratio of significant obstruction: -Double bifid: OR Anterior displaced: 0R 7.1 Kwon DH, Heart 2008; 94:
17 Papillary Muscles: Mobility Predictors of LVOT gradient: -Basal septal thickness -Bifid PM mobility -AML length -Abnormal chordal attachement to the base of AML Patel P, Circ CV Img 2015; 8: epub
18 Degenerative Mitral Valve Prolapse
19 Mitral Valve Prolapse - 3% des patients avec CMH, association fortuite - Isolated postero-latero basal LV free wall hypertrophy and Mitral valve prolapse Petrone RK, JACC 1992; 20: Maron BJ, AJC 2010; 106: 750-2
20 Flail Leaflet: Chord rupture - Chordal rupture without known MVP - Likely favoured by obstruction - Mayo Clinic: 32 cases operated : 4% myomectomy, 3% mitral valve repair Wan CK, Ann Thorac Surg, 2009, 88: Zia MI, Am J Cardiol, 2012, 109:
21 Flail Leaflet: Chord rupture Boissier F, ACVD 2015; 108: HCM patients -6 (1%) chordal rupture -5 men, 1 women, 70±5 yrs -Delay from HCM to diagnosis: 16±3 yrs -No MVP before CRupture -Obstruction in all patients before rupture: 56±17 mmhg -Regression of obstruction after chordal rupture
22 Conclusion: Mitral Valve in HCM Assessment by echocardiography and CMR Imaging Mitral leaflet: length and dynamics Chordal alteration: attachment, tension Papillary muscles: number, position, mobility Obstruction (rest or latent)
23 Anterior displacement of MV apparatus
24 Mitral valve: SAM
25 Mitral valve: SAM Mechanism of obstruction Anomalies of mitral valve apparatus -antero-displacement of the mitral valve apparatus, mitral coaptation point -Pushing of the anterior leaflet by the posterior one -Pushing of leaflets by ejection flow deviated by septal hypertrophy Venturi effect Septal hypertrophy and hyperkinesy Flow acceleration, hydrostatic depression, and attraction of the mitral leaflet toward the septum
26 Pathophysiology: LV remodeling Wall hypertrophy Myocardial fibrosis LVOT obstruction Genetic factors Environments factors Wall stress C LV remodeling activation Fœtal program reexpression Hypertrophy, Apoptosis, Fibrosis
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