Mitral Valve Disease

MitralMitral valvevalve diseasedisease

IdentifyIdentify thethe principleprinciple causecause ofof mitralmitral stenosisstenosis NameName severalseveral mechanismsmechanisms ofof mitralmitral regurgitationregurgitation IdentifyIdentify howhow oftenoften mitralmitral regurgitationregurgitation shouldshould bebe followedfollowed byby echocardiogramechocardiogram ©2010 David Stultz, MD ThisThis ConferenceConference isis anan OverviewOverview

ManyMany aspectsaspects cannotcannot bebe coveredcovered inin aa 11 hourhour conferenceconference ThisThis isis meantmeant toto serveserve asas aa frameworkframework forfor furtherfurther knowledgeknowledge ©2010 David Stultz, MD OutlineOutline ofof conferenceconference

MitralMitral StenosisStenosis MitralMitral regurgitationregurgitation MitralMitral valvevalve prolapseprolapse

Fedak PW, McCarthy PM, Bonow RO. Evolving concepts and technologies in mitral valve repair. Circulation. 2008 Feb 19;117(7):963- 74. ©2010 David Stultz, MD MitralMitral StenosisStenosis NarrowingNarrowing ofof thethe mitralmitral valvevalve orificeorifice RestrictsRestricts flowflow fromfrom leftleft atriumatrium toto leftleft ventricleventricle duringduring diastolediastole RheumaticRheumatic feverfever almostalmost alwaysalways thethe causecause SenileSenile calcificcalcific (annular(annular calcification)calcification) AnorecticAnorectic drugs,drugs, carcinoidcarcinoid MitralMitral valvevalve areaarea normallynormally 44--6cm6cm2 2cm2cm2 isis mildmild stenosisstenosis <1cm<1cm2 isis criticalcritical stenosisstenosis

Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr., Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2008;52:e1–142. Carabello BA. Modern management of mitral stenosis. Circulation. 2005 Jul 19;112(3):432-7. ©2010 David Stultz, MD

ExertionalExertional dyspneadyspnea HemoptysisHemoptysis ChestChest painpain SystemicSystemic embolizationembolization MostlyMostly duedue toto atrialatrial fibrillationfibrillation

DiastolicDiastolic murmurmurmur LowLow pitchedpitched rumblerumble atat apexapex withwith bellbell BestBest heardheard inin leftleft laterallateral decubitusdecubitus positionposition ProminentProminent S1S1 (If(If leafletsleaflets areare pliable)pliable) OpeningOpening snapsnap CausedCaused byby aa tensiontension onon thethe valvevalve leafletsleaflets whenwhen thethe valvevalve opensopens AudibleAudible atat apex,apex, withwith bellbell ofof stethoscopestethoscope

FollowsFollows AA2 byby 4040--120ms120ms ShorterShorter AA2--OSOS intervalinterval == moremore severesevere MSMS

Severity MVA Gradient PAP Symptoms Signs Therapy cm2 mmHg

Mild >1.8 2-4 Normal Usually absent S2-OS > 120ms; normal P2

Moderate 1.2-1.6 4-9 Normal Class II S2-OS Diuretics 100- 120ms; normal P2

Moderate 1.0-1.2 10-15 Mild Class II-III S2-OS 80- BMV if to Severe pulmonary 100ms; P2 applicable or HTN increase surgery if more than mild Sx

Severe <1.0 15 Mild to Class II-IV S2-OS BMV or surgery severe <80ms; P2 pulmonary increase; HTN RV lift

MSMS AuscultationAuscultation

Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr., Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2008;52:e1–142. ©2010 David Stultz, MD MedicalMedical ManagementManagement

DiureticsDiuretics SaltSalt restrictionrestriction AnticoagulationAnticoagulation ifif indicatedindicated AtrialAtrial fibrillationfibrillation TreatmentTreatment ofof atrialatrial fibrillationfibrillation

Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, Saunders, 2007. ©2010 David Stultz, MD MitralMitral StenosisStenosis ““HockeyHockey StickStick”” ©2010 David Stultz, MD MeanMean PressurePressure GradientGradient MitralMitral StenosisStenosis ©2010 David Stultz, MD SymptomaticSymptomatic MitralMitral StenosisStenosis

Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr., Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2008;52:e1–142. ©2010 David Stultz, MD NaturalNatural HistoryHistory ofof MitralMitral ValveValve diseasedisease (1991)(1991)

IndicatedIndicated forfor ModerateModerate (NYHA(NYHA ClassClass 2)2) symptomssymptoms PAPA pressurepressure >60mmHg>60mmHg PCWPPCWP pressurepressure >25mmHg>25mmHg PercutaneousPercutaneous balloonballoon valvotomyvalvotomy FavoredFavored ifif echoecho showsshows High leaflet mobility Low calcification, thickening, and subvalvular thickening Open/ClosedOpen/Closed SurgicalSurgical valvotomyvalvotomy MitralMitral ValveValve replacementreplacement

Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr., Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2008;52:e1–142. ©2010 David Stultz, MD InoueInoue methodmethod ofof balloonballoon mitralmitral valvotomyvalvotomy ((transseptaltransseptal approach)approach)

Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, Saunders, 2007. ©2010 David Stultz, MD MeanMean pressurepressure gradientgradient acrossacross MitralMitral ValveValve PrePre-- andand PostPost-- BalloonBalloon ValvotomyValvotomy

Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, Saunders, 2007. ©2010 David Stultz, MD WhyWhy isis mitralmitral regurgitationregurgitation soso complicated?complicated? VariableVariable etiologiesetiologies VariableVariable symptomssymptoms GenerallyGenerally slowslow onsetonset SymptomsSymptoms oftenoften overlapoverlap withwith deconditioningdeconditioning andand agingaging VariableVariable comorbitiescomorbities CardiacCardiac Role of coronary & myocardial disease SystemicSystemic GuidelinesGuidelines oftenoften basedbased onon specificspecific numericalnumerical cutoffscutoffs forfor variousvarious measurementsmeasurements ©2010 David Stultz, MD SymptomsSymptoms ofof MRMR

TypicallyTypically developdevelop overover aa longerlonger timetime frameframe thanthan mitralmitral stenosisstenosis ShortnessShortness ofof breathbreath Weakness/fatigueWeakness/fatigue

SystolicSystolic murmurmurmur HolosystolicHolosystolic ConstantConstant intensityintensity Blowing,Blowing, highhigh pitchpitch LoudestLoudest atat apex,apex, radiatesradiates toto axillaaxilla

http://en.wikipedia.org/wiki/File:Phonocardiograms_from_normal_and_abnormal_heart_sounds.png http://depts.washington.edu/physdx/heart/tech.html ©2010 David Stultz, MD CausesCauses ofof ChronicChronic MRMR

Inflammatory Rheumatic heart disease Systemic lupus erythematosus Scleroderma Degenerative Myxomatous degeneration of mitral valve leaflets (Barlow click-murmur syndrome, prolapsing leaflet, mitral valve prolapse) Marfan syndrome Ehlers-Danlos syndrome Pseudoxanthoma elasticum Calcification of mitral valve annulus Infective Infective endocarditis affecting normal, abnormal, or prosthetic mitral valves Structural Ruptured chordae tendineae (spontaneous or secondary to myocardial infarction, trauma, mitral valve prolapse, endocarditis) Rupture or dysfunction of papillary muscle (ischemia or myocardial infarction) Dilation of mitral valve annulus and left ventricular cavity (congestive cardiomyopathies, aneurysmal dilation of the left ventricle) Hypertrophic cardiomyopathy Paravalvular prosthetic leak Congenital Mitral valve clefts or fenestrations Parachute mitral valve abnormality in association with: Endocardial cushion defects Endocardial fibroelastosis Transposition of the great arteries Anomalous origin of the left coronary artery

Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, Saunders, 2007. ©2010 David Stultz, MD HowHow oftenoften shouldshould II getget anan echo?echo? ModerateModerate toto SevereSevere MRMR (Asymptomatic)(Asymptomatic) EveryEvery 66--1212 monthsmonths ModerateModerate MRMR NotNot specifiedspecified inin GuidelinesGuidelines oror appropriatenessappropriateness criteriacriteria AsymptomaticAsymptomatic MildMild MRMR NotNot routinelyroutinely recommendedrecommended EchoEcho isis recommendedrecommended forfor changechange inin symptomssymptoms Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr., Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2008;52:e1–142.

Douglas PS, Khandheria B, Stainback RF, Weissman NJ. ACCF/ASE/ACEP/ASNC/SCAI/SCCT/SCMR, 2007 appropriateness criteria for transthoracic and transesophageal echocardiography. J Am Coll Cardiol 2007. ©2010 David Stultz, MD

ManagingManaging ChronicChronic SevereSevere MRMR

Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr., Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2008;52:e1–142. ©2010 David Stultz, MD EchocardiographyEchocardiography

PrimaryPrimary tooltool forfor assessingassessing severityseverity ofof MitralMitral regurgitationregurgitation ©2010 David Stultz, MD MildMild MitralMitral RegurgitationRegurgitation ©2010 David Stultz, MD ModerateModerate MitralMitral RegurgitationRegurgitation ©2010 David Stultz, MD ModerateModerate MRMR CWCW jetjet ©2010 David Stultz, MD PISAPISA –– ModerateModerate MRMR ©2010 David Stultz, MD SevereSevere MitralMitral RegurgitationRegurgitation ©2010 David Stultz, MD EtiologyEtiology ofof MitralMitral RegurgitationRegurgitation

PrimaryPrimary FlailFlail leafletleaflet MitralMitral valvevalve prolapseprolapse PerforationPerforation (endocarditis)(endocarditis) ChordalChordal rupturerupture SecondarySecondary AnnularAnnular dilatationdilatation IschemicIschemic mitralmitral regurgitationregurgitation RemodelingRemodeling ofof papillarypapillary musclemuscle ©2010 David Stultz, MD SimplifiedSimplified MechanismsMechanisms ofof MitralMitral RegurgitationRegurgitation Normal

Prolapse

Flail leaflet

Restricted leaflet motion

Perforated leaflet

Type I - normal leaflet length and motion but with either annular dilation or leaflet perforation

Type II MR is caused by leaflet prolapse or by papillary muscle rupture or elongation.

Type III MR is caused by restricted leaflet motion. Type IIIa - rheumatic disease with subvalvular involvement. Type IIIb – tethered and restricted leaflet motion due to ischemic or idiopathic cardiomyopathy with ventricular dilation.

Fedak PW, McCarthy PM, Bonow RO. Evolving concepts and technologies in mitral valve repair. Circulation. 2008 Feb 19;117(7):963- 74. ©2010 David Stultz, MD FlailFlail posteriorposterior leafletleaflet tiptip ©2010 David Stultz, MD IschemicIschemic MitralMitral RegurgitationRegurgitation ©2010 David Stultz, MD MitralMitral valvevalve leafletleaflet perforationperforation EndocarditisEndocarditis ©2010 David Stultz, MD DilatedDilated CardiomyopathyCardiomyopathy ((NonischemicNonischemic))

DefinedDefined asas billowingbillowing ofof thethe mitralmitral valvevalve leafletleaflet 2mm2mm beyondbeyond thethe annularannular planeplane inin thethe parasternalparasternal longlong axisaxis viewview MyxomatousMyxomatous degenerationdegeneration inin youngeryounger patientspatients FibroelasticFibroelastic tissuetissue deficiencydeficiency inin elderlyelderly 11--2.5%2.5% prevalenceprevalence HeterogenousHeterogenous naturalnatural historyhistory 55--10%10% progressprogress toto severesevere mitralmitral regurgitationregurgitation

SystolicSystolic ClickClick BestBest heardheard withwith diaphragmdiaphragm OccursOccurs atat leastleast 140ms140ms afterafter S1S1 CausedCaused byby suddensudden tensingtensing ofof chordaechordae duringduring systolesystole ManeuversManeuvers thatthat decreasedecrease LVLV volumevolume movemove clickclick closercloser toto S1S1 ManeuversManeuvers thatthat increaseincrease LVLV volumevolume movemove clickclick awayaway fromfrom S1S1 andand lowerlower intensityintensity

Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, Saunders, 2007. http://www.texasheart.org/education/cme/explore/events/eventdetail_5469.cfm ©2010 David Stultz, MD

Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, Saunders, 2007. ©2010 David Stultz, MD MitralMitral ValveValve ProlapseProlapse SyndromeSyndrome ClusterCluster ofof symptomssymptoms PalpitationsPalpitations ChestChest painpain TIATIA symptomssymptoms

GuidelinesGuidelines basebase treatmenttreatment onon CerebrovascularCerebrovascular eventevent AtrialAtrial fibrillationfibrillation SeveritySeverity ofof MitralMitral regurgitationregurgitation ©2010 David Stultz, MD MildMild MVPMVP

UsuallyUsually SignificantSignificant SymptomsSymptoms ShortnessShortness ofof BreathBreath HypotensionHypotension SuddenSudden changechange inin valvularvalvular functionfunction PerforationPerforation PapillaryPapillary musclemuscle dysfunctiondysfunction ChordalChordal rupturerupture ©2010 David Stultz, MD CausesCauses ofof AcuteAcute MRMR

Mitral Annulus Disorders Infective endocarditis (abscess formation) Trauma (valvular heart surgery) Paravalvular leak caused by suture interruption (surgical technical problems or infective endocarditis) Mitral Leaflet Disorders Infective endocarditis (perforation or interference with valve closure by vegetation) Trauma (tear during percutaneous balloon mitral valvotomy or penetrating chest injury) Tumors (atrial myxoma) Myxomatous degeneration Systemic lupus erythematosus (Libman-Sacks lesion) Rupture of Chordae Tendineae Idiopathic (e.g., spontaneous) Myxomatous degeneration (mitral valve prolapse, Marfan syndrome, Ehlers-Danlos syndrome) Infective endocarditis Acute rheumatic fever Trauma (percutaneous balloon valvotomy, blunt chest trauma) Papillary Muscle Disorders Coronary artery disease (causing dysfunction and rarely rupture) Acute global left ventricular dysfunction Infiltrative diseases (amyloidosis, sarcoidosis) Trauma Primary Mitral Valve Prosthetic Disorders Porcine cusp perforation (endocarditis) Porcine cusp degeneration Mechanical failure (strut fracture) Immobilized disc or ball of the mechanical prosthesis

AuscultationAuscultation maymay notnot bebe remarkableremarkable EchocardiographyEchocardiography isis primaryprimary diagnosticdiagnostic modalitymodality

MedicalMedical managementmanagement limitedlimited NitroprussideNitroprusside forfor afterloadafterload reductionreduction SurgicalSurgical managementmanagement usuallyusually indicatedindicated

RepairRepair favoredfavored overover valvevalve replacementreplacement

NewNew endovascularendovascular techniquestechniques promisingpromising EVERESTEVEREST 22 trialtrial forfor MitraClipMitraClip OtherOther percutaneouspercutaneous methodsmethods ©2010 David Stultz, MD RepairRepair ofof PosteriorPosterior MVPMVP

Fedak PW, McCarthy PM, Bonow RO. Evolving concepts and technologies in mitral valve repair. Circulation. 2008 Feb 19;117(7):963- 74. ©2010 David Stultz, MDCatheter-Based Mitral Valve Repair MitraClip® System

4 Investigational Device only in the US; Not available for sale in the US Endovascular Valve Edge-to-Edge Repair Study (EVEREST 2) Randomized Clinical Trial: Primary Safety and Efficacy Endpoints. Ted Feldman, Laura Mauri, Elyse Foster, Don Glower on behalf of the EVEREST 2 Investigators. ACC 2010 Annual Scientific Sessions, March 14, 2010, Atlanta, GA ©2010 David Stultz, MD EVEREST II RCT: Summary

Safety & effectiveness endpoints met • Safety: MAE rate at 30 days – MitraClip device patients: 9.6% – MV surgery patients: 57%

• Effectiveness: Clinical Success Rate at 12 months – MitraClip device patients: 72% – MV Surgery patients: 88%

Clinical benefit demonstrated for MitraClip System nda MV surgery patients through 12 months – Improved LV function – Improved NYHA Functional Class – Improved Quality of Life

Surgery remains an option after the MitraClip procedure

27 Investigational Device only in the US; Not available for sale in the US

Endovascular Valve Edge-to-Edge Repair Study (EVEREST 2) Randomized Clinical Trial: Primary Safety and Efficacy Endpoints. Ted Feldman, Laura Mauri, Elyse Foster, Don Glower on behalf of the EVEREST 2 Investigators. ACC 2010 Annual Scientific Sessions, March 14, 2010, Atlanta, GA ©2010 David Stultz,OtherOther MD investigationalinvestigational percutaneouspercutaneous methodsmethods

Carillon (Cardiac Dimensions, Inc., Kirkland, Washington)

Edwards MONARC (Edwards Lifesciences, Irvine, California)

Percutaneous Transvenous Mitral Annuloplasty (PTMA) (Viacor, Inc., Wilmington, Massachusetts)

Mitralign – retrograde catheter in LV anchors to AV junction and cinches together Quantumcor – thermal energy at AV junction to shrink orifice Ample PS3 – Left atrial T bar anchored by septal occluder/coronary sinus iCoapsys – Transventricular bridge to change LV geometry

Van Mieghem NM, Piazza N, Anderson RH, Tzikas A, Nieman K, De Laat LE, McGhie JS, Geleijnse ML, Feldman T, Serruys PW, de Jaegere PP. Anatomy of the mitral valvular complex and its implications for transcatheter interventions for mitral regurgitation. J Am Coll Cardiol. 2010 Aug 17;56(8):617-26. ©2010 David Stultz, MD ReferencesReferences Carabello BA. Modern management of mitral stenosis. Circulation. 2005 Jul 19;112(3):432-7. Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr., Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease). J Am Coll Cardiol 2008;52:e1–142. Douglas PS, Khandheria B, Stainback RF, Weissman NJ. ACCF/ASE/ACEP/ASNC/SCAI/SCCT/SCMR, 2007 appropriateness criteria for transthoracic and transesophageal echocardiography. J Am Coll Cardiol 2007. Fedak PW, McCarthy PM, Bonow RO. Evolving concepts and technologies in mitral valve repair. Circulation. 2008 Feb 19;117(7):963-74. Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, Saunders, 2007. Van Mieghem NM, Piazza N, Anderson RH, Tzikas A, Nieman K, De Laat LE, McGhie JS, Geleijnse ML, Feldman T, Serruys PW, de Jaegere PP. Anatomy of the mitral valvular complex and its implications for transcatheter interventions for mitral regurgitation. J Am Coll Cardiol. 2010 Aug 17;56(8):617-26.