tricuspid and pulmonic valve disease - dr. stultz 01 13 tricuspid and pulmonic... · tricuspid...

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Tricuspid and Tricuspid and Pulmonic Pulmonic Valve Disease Valve Disease The Forgotten Valves The Forgotten Valves David Stultz, MD, FACC David Stultz, MD, FACC Southwest Cardiology, Inc. Southwest Cardiology, Inc. January 13, 2009 January 13, 2009 (c) 2000-2008 David Stultz, MD

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Page 1: Tricuspid and Pulmonic Valve Disease - Dr. Stultz 01 13 Tricuspid and Pulmonic... · Tricuspid andTricuspid and PulmonicPulmonic Valve DiseaseValve Disease The Forgotten ValvesThe

Tricuspid andTricuspid and PulmonicPulmonic Valve DiseaseValve DiseaseThe Forgotten ValvesThe Forgotten Valves

David Stultz, MD, FACCDavid Stultz, MD, FACC

Southwest Cardiology, Inc.Southwest Cardiology, Inc.

January 13, 2009January 13, 2009

(c) 2000-2008 David Stultz, MD

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Goals of ConferenceGoals of Conference

Understand Tricuspid andUnderstand Tricuspid and PulmonicPulmonic valvevalvestenosisstenosis and regurgitationand regurgitation

DiagnosisDiagnosis

Treatment optionsTreatment options

Specific conditionsSpecific conditions

EbsteinEbstein anomalyanomaly

Carcinoid SyndromeCarcinoid Syndrome

(c) 2000-2008 David Stultz, MD

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A Few BasicsA Few Basics

Venous WaveformVenous Waveform

Understanding Echo terminology with respectUnderstanding Echo terminology with respectto pressure gradients andto pressure gradients and stenosisstenosis

(c) 2000-2008 David Stultz, MD

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Right Atrial WaveformRight Atrial Waveform

a wave - RA contractionelevated in RV failure

c wave - tricuspid closurev wave - passive filling of RA duringventricular systole = T wave on ECG

elevated in tricuspid regurgitationx descent - atrial diastoley descent - atrial emptying

http://www.staff.vu.edu.au/CriticalCare/Critical%20Care/lecture2_notes.htm

(c) 2000-2008 David Stultz, MD

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UnderstandingUnderstanding StenosisStenosis and Pressureand PressureGradientsGradients

No “Stenosis” at the end of the hoseNo SIGNIFICANT Pressure gradientNo impedence to water flow

Now there is a “Stenosis” at the end of the hoseSIGNIFICANT Pressure gradient across the thumbWater flow impeded, creating increased velocity

Put Thumb on hose

(c) 2000-2008 David Stultz, MD

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Tricuspid Valve AnatomyTricuspid Valve Anatomy

SL – Septal leaflet, AL – Anterior leaflet, PL – Posterior leaflet

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Feigenbaum, 6th ed

Right Ventricle InflowRight Ventricle Inflow(c) 2000-2008 David Stultz, MD

Page 8: Tricuspid and Pulmonic Valve Disease - Dr. Stultz 01 13 Tricuspid and Pulmonic... · Tricuspid andTricuspid and PulmonicPulmonic Valve DiseaseValve Disease The Forgotten ValvesThe

ParasternalParasternal Short AxisShort Axis

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

Page 9: Tricuspid and Pulmonic Valve Disease - Dr. Stultz 01 13 Tricuspid and Pulmonic... · Tricuspid andTricuspid and PulmonicPulmonic Valve DiseaseValve Disease The Forgotten ValvesThe

Apical 4 ChamberApical 4 Chamber

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

Page 10: Tricuspid and Pulmonic Valve Disease - Dr. Stultz 01 13 Tricuspid and Pulmonic... · Tricuspid andTricuspid and PulmonicPulmonic Valve DiseaseValve Disease The Forgotten ValvesThe

SubcostalSubcostal

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

Page 11: Tricuspid and Pulmonic Valve Disease - Dr. Stultz 01 13 Tricuspid and Pulmonic... · Tricuspid andTricuspid and PulmonicPulmonic Valve DiseaseValve Disease The Forgotten ValvesThe

TEE 4 chamberTEE 4 chamber

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

Page 12: Tricuspid and Pulmonic Valve Disease - Dr. Stultz 01 13 Tricuspid and Pulmonic... · Tricuspid andTricuspid and PulmonicPulmonic Valve DiseaseValve Disease The Forgotten ValvesThe

TEE RV inflowTEE RV inflow

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

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TricuspidTricuspid StenosisStenosis

EtiologyEtiology

Almost always rheumaticAlmost always rheumatic

Other causes are rareOther causes are rare

Congenital tricuspidCongenital tricuspid atresiaatresia

Right atrial tumorsRight atrial tumors

Carcinoid syndromeCarcinoid syndrome

More often tricuspid regurgitationMore often tricuspid regurgitation

EndomyocardialEndomyocardial fibrosisfibrosis

VegetationsVegetations

Pacemaker leadPacemaker lead

ExtracardiacExtracardiac tumorstumors

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Rheumatic TricuspidRheumatic Tricuspid StenosisStenosis

At autopsy, seen in 15% of patients withAt autopsy, seen in 15% of patients withrheumatic heart diseaserheumatic heart disease

But clinically significant in only 5%But clinically significant in only 5%

Isolated TS is rareIsolated TS is rare

Almost always mitral valve involvementAlmost always mitral valve involvement

Also aortic valve involvement commonAlso aortic valve involvement common

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Pathologic FindingsPathologic FindingsRheumatic TricuspidRheumatic Tricuspid StenosisStenosis

Similar to mitralSimilar to mitral stenosisstenosis

Fusion and shortening ofFusion and shortening of chordaechordae tendineaetendineae

Fusion of leaflet edgesFusion of leaflet edges

Calcification is rareCalcification is rare

Right atrial dilatation, wall thickeningRight atrial dilatation, wall thickening

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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PathophysiologyPathophysiology

Mean pressure gradient across tricuspid valve ofMean pressure gradient across tricuspid valve of5mmHg is enough to cause symptoms5mmHg is enough to cause symptoms

Jugular venous distensionJugular venous distension

AscitesAscites

EdemaEdema

Mean pressure gradient of 2mmHg is sufficientMean pressure gradient of 2mmHg is sufficientfor diagnosisfor diagnosis

Augmented by inspiration, fluid statusAugmented by inspiration, fluid status

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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SymptomsSymptoms

FatigueFatigue

Abdominal discomfortAbdominal discomfort HepatomegalyHepatomegaly

AscitesAscites

AnasarcaAnasarca

Neck discomfortNeck discomfort

DyspneaDyspnea,, orthopneaorthopnea rare with isolated TSrare with isolated TS More often these symptoms due to mitral valveMore often these symptoms due to mitral valve

diseasedisease

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Physical ExaminationPhysical Examination

Diastolic rumble at left lowerDiastolic rumble at left lower sternalsternal borderborder Increased with inspirationIncreased with inspiration

Often confused with mitralOften confused with mitral stenosisstenosis

Tricuspid opening snapTricuspid opening snap

Neck vein distentionNeck vein distention Prominent a waveProminent a wave

Slow y descentSlow y descent

Hepatic pulsationHepatic pulsation

AscitesAscites

Aortic and Mitral murmursAortic and Mitral murmurs

http://www.rjmatthewsmd.com/Definitions/venous_pulse.htmBraunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Maneuvers to Change intensity of Murmur inManeuvers to Change intensity of Murmur inTricuspidTricuspid StenosisStenosis

Increase MurmurIncrease Murmur InspirationInspiration Mueller maneuverMueller maneuver Right lateral decubitus positionRight lateral decubitus position Leg raisesLeg raises SquattingSquatting Isotonic exercise (hand grip)Isotonic exercise (hand grip) Amyl nitrite inhalationAmyl nitrite inhalation

Decrease MurmurDecrease Murmur ExpirationExpiration Strain phase of ValsalvaStrain phase of Valsalva

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Diagnostic EvaluationDiagnostic Evaluation

EchocardiographyEchocardiography Diastolic doming of leafletsDiastolic doming of leaflets

Thickening of leaflets, restricted motionThickening of leaflets, restricted motion

Doppler echo shows prolonged antegrade slopeDoppler echo shows prolonged antegrade slope

EKGEKG Right atrial enlargementRight atrial enlargement

ChestChest XrayXray Right atrial enlargementRight atrial enlargement

CatheterizationCatheterization Largely replaced by EchoLargely replaced by Echo

HemodynamicsHemodynamics

Angiography of RA shows a small diastolic jetAngiography of RA shows a small diastolic jet

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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ManagementManagement

Sodium restrictionSodium restriction

DiureticsDiuretics

Surgical managementSurgical management

Mean pressure gradient of 5mmHgMean pressure gradient of 5mmHg

Valve orifice area ofValve orifice area of ≤≤2.0cm22.0cm2

Often coexistent Mitral diseaseOften coexistent Mitral disease

TS + MSTS + MS –– fix both at same timefix both at same time

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Surgical TechniquesSurgical Techniques

OpenOpen valvotomyvalvotomy (Create a functional bicuspid(Create a functional bicuspidvalve)valve) OpenOpen commisurescommisures between anteriorbetween anterior--septalseptal andand

septalseptal--posterior leafletsposterior leaflets Not between anterior and posterior leafletsNot between anterior and posterior leaflets

Leads to severe regurgitationLeads to severe regurgitation

BioprostheticBioprosthetic valve replacementvalve replacement Preferred over mechanical due to risk ofPreferred over mechanical due to risk of

thromboembolismthromboembolism

BalloonBalloon valvuloplastyvalvuloplasty??

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Tricuspid RegurgitationTricuspid RegurgitationEtiologyEtiology

Secondary (functional) TRSecondary (functional) TR

Most CommonMost Common

Structurally Normal ValveStructurally Normal Valve

Due to dilatation of right ventricleDue to dilatation of right ventricle

Commonly due to mitral valve diseaseCommonly due to mitral valve disease

RV systolic pressure more than 55mmHg will cause TRRV systolic pressure more than 55mmHg will cause TR

Other etiologiesOther etiologies

Pulmonary hypertensionPulmonary hypertension

EisenmengerEisenmenger’’ss syndrome/Congenitalsyndrome/Congenital

RV infarctionRV infarction

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Tricuspid RegurgitationTricuspid RegurgitationEtiologyEtiology

Primary Causes (abnormal valve)Primary Causes (abnormal valve)

Less CommonLess Common

LupusLupusConnective Tissue DiseaseConnective Tissue Disease

FenfluramineFenfluramine--phenterminephentermineRadiation InjuryRadiation Injury

MethysergideMethysergide valvular diseasevalvular diseaseRheumatoid ArthritisRheumatoid Arthritis

EndomyocardialEndomyocardial fibrosisfibrosisCarcinoidCarcinoid

RepeatedRepeated EndomyocardialEndomyocardial biopsybiopsyProlapseProlapse (Floppy Valve)(Floppy Valve)

Pacemaker leadsPacemaker leadsEbsteinEbstein anomalyanomaly

Cardiac Tumors (RACardiac Tumors (RA MyxomaMyxoma))InfectiveInfective endocarditisendocarditis

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Carcinoid SyndromeCarcinoid Syndrome

Tricuspid regurgitation or combinedTricuspid regurgitation or combinedstenosisstenosis/regurgitation/regurgitation

Deposits of fibrous tissue onDeposits of fibrous tissue on endocardialendocardial surfacesurfaceof valvular cuspsof valvular cusps

Usually ventricular surface of tricuspid valveUsually ventricular surface of tricuspid valve

Causes adhesion to right ventricular wall,Causes adhesion to right ventricular wall,creating regurgitationcreating regurgitation

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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SymptomsSymptoms

Generally well toleratedGenerally well tolerated

Pulmonary hypertension + TR = decreasedPulmonary hypertension + TR = decreasedcardiac outputcardiac output

FatigueFatigue

Abdominal discomfortAbdominal discomfort HepatomegalyHepatomegaly

AscitesAscites

AnasarcaAnasarca

Neck discomfortNeck discomfort

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Physical ExaminationPhysical Examination

Jugular venous distensionJugular venous distension ProminentProminent cc--vv wavewave

Sharp y descentSharp y descent

MurmurMurmur Associated pulmonary HTNAssociated pulmonary HTN

PansystolicPansystolic murmurmurmur

Left lowerLeft lower sternalsternal borderborder

Loud P2Loud P2

Without pulmonary HTNWithout pulmonary HTN Low intensityLow intensity

Murmur in 1Murmur in 1stst half of systolehalf of systole

http://www.rjmatthewsmd.com/Definitions/venous_pulse.htmBraunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Diagnostic EvaluationDiagnostic Evaluation

EchocardiographyEchocardiography Tricuspid regurgitation severityTricuspid regurgitation severity

Right ventricular functionRight ventricular function

Pulmonary artery pressurePulmonary artery pressure

EKGEKG -- nonspecificnonspecific Incomplete RBBBIncomplete RBBB

Atrial fibrillationAtrial fibrillation

ChestChest XrayXray CardiomegalyCardiomegaly, right atrial enlargement, right atrial enlargement

Catheterization/HemodynamicsCatheterization/Hemodynamics RV systolic pressure <40 mmHg favors Primary causeRV systolic pressure <40 mmHg favors Primary cause

RV systolic pressure >55 mmHg favors Secondary causeRV systolic pressure >55 mmHg favors Secondary cause

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Surgical ManagementSurgical Management

Well tolerated without pulmonary HTNWell tolerated without pulmonary HTN ValvectomyValvectomy tolerated well, with right heart dilatation developing monthstolerated well, with right heart dilatation developing months

to years after surgeryto years after surgery

Primary causePrimary cause Usually requireUsually require bioprosthesisbioprosthesis

Secondary causeSecondary cause UsuallyUsually annuloplastyannuloplasty ring (in conjunction with mitral valve surgery)ring (in conjunction with mitral valve surgery)

May not require TV surgery if mild TR and normalMay not require TV surgery if mild TR and normal TriscupidTriscupid annulus sizeannulus size

EndocarditisEndocarditis in IV drug usein IV drug use ControversialControversial

ValvectomyValvectomy followed later byfollowed later by bioprosthesisbioprosthesis??

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Pulmonary Valve (TTE Short Axis)Pulmonary Valve (TTE Short Axis)

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

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Pulmonary Valve (TTE Long Axis)Pulmonary Valve (TTE Long Axis)

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

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PulmonicPulmonic ValveValve StenosisStenosis

EtiologyEtiology

CongenitalCongenital pulmonicpulmonic valvevalve stenosisstenosis

VariableVariable atresiaatresia,, dysplasticdysplastic valvevalve

Associated with NoonanAssociated with Noonan SydromeSydrome

May beMay be supravalvularsupravalvular

RhuematicRhuematic disease (uncommon)disease (uncommon)

CarcinoidCarcinoid

Cardiac TumorsCardiac Tumors

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Physical ExamPhysical Exam

Decreased intensity of P2Decreased intensity of P2

Possible thrill in 2Possible thrill in 2ndnd leftleft intercostalintercostal spacespace

(c) 2000-2008 David Stultz, MD

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Treatment ofTreatment of PulmonicPulmonic ValveValveStenosisStenosis

PercutaneousPercutaneous BalloonBalloon ValvotomyValvotomy

Usually when mean pressure gradient across valve isUsually when mean pressure gradient across valve is50mmHg50mmHg

May require additionalMay require additional valvotomiesvalvotomies in futurein future

(c) 2000-2008 David Stultz, MD

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PulmonicPulmonic Valve RegurgitationValve Regurgitation

EtiologyEtiology Dilatation ofDilatation of pulmonicpulmonic valve ring (Most Common)valve ring (Most Common)

Pulmonary hypertension (Any cause)Pulmonary hypertension (Any cause)

Pulmonary arteryPulmonary artery dilitationdilitation Connective tissue diseaseConnective tissue disease

InfectiveInfective endocarditisendocarditis

Less Common CausesLess Common Causes Surgical treatment forSurgical treatment for tetralogytetralogy ofof FallotFallot

Other Congenital causesOther Congenital causes

Carcinoid syndromeCarcinoid syndrome

SyphillisSyphillis

Chest traumaChest trauma

Pulmonary artery catheterizationPulmonary artery catheterization

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Carcinoid ofCarcinoid of PulmonicPulmonic ValveValve

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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SymptomsSymptoms

Similar to tricuspid regurgitationSimilar to tricuspid regurgitation

Tolerated well as long as pulmonary arteryTolerated well as long as pulmonary arterypressure is normalpressure is normal

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Physical ExaminationPhysical Examination

Low pitch diastolic murmur at left 3Low pitch diastolic murmur at left 3rdrd or 4or 4thth intercostalintercostalspacespace

S3 or S4 at left lowerS3 or S4 at left lower sternalsternal border, augmented byborder, augmented byinspirationinspiration

GrahamGraham--SteellSteell MurmurMurmur Pulmonary pressure >55mmHgPulmonary pressure >55mmHg High pitched, blowing decrescendo murmurHigh pitched, blowing decrescendo murmur LeftLeft parasternalparasternal area, starts after prominent P2area, starts after prominent P2 Increases with inspirationIncreases with inspiration Confused with aortic regurgitation murmurConfused with aortic regurgitation murmur

However AR is more commonHowever AR is more common

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Diagnostic EvaluationDiagnostic Evaluation

EchocardiographyEchocardiography Severity of regurgitationSeverity of regurgitation PulmonicPulmonic valve annulus sizevalve annulus size Pulmonary arterial pressurePulmonary arterial pressure

EKGEKG Without pulmonary hypertensionWithout pulmonary hypertension

Incomplete RBBBIncomplete RBBB

With pulmonary hypertensionWith pulmonary hypertension RV hypertrophyRV hypertrophy

XRayXRay and Angiographyand Angiography –– nonspecificnonspecific Cardiac MRICardiac MRI –– Assess PA dilatation, PR severityAssess PA dilatation, PR severity

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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ManagementManagement

Usually not severe enough alone to requireUsually not severe enough alone to requirespecific treatmentspecific treatment

Treatment of pulmonary hypertension usuallyTreatment of pulmonary hypertension usuallysufficientsufficient

Mitral valve surgeryMitral valve surgery

EndocarditisEndocarditis may require valve replacementmay require valve replacement

BioprosthesisBioprosthesis or allograft preferred foror allograft preferred forreplacementreplacement

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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Specific DiseasesSpecific Diseases

EbsteinEbstein’’ss AnomalyAnomaly

Carcinoid SyndromeCarcinoid Syndrome

(c) 2000-2008 David Stultz, MD

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Crawford & DiMarco, 2nd ed

(c) 2000-2008 David Stultz, MD

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EbsteinEbstein’’ss AnomalyAnomaly

11--2 in 10,000 approximate incidence in live births2 in 10,000 approximate incidence in live births

Apical displacement of tricuspid valve leafletsApical displacement of tricuspid valve leaflets

Anterior leaflet never displacedAnterior leaflet never displaced

Variable leaflet deformityVariable leaflet deformity

““AtrializationAtrialization”” of right ventricular tissueof right ventricular tissue

Small right ventricle, large right atriumSmall right ventricle, large right atrium

Associated lesionsAssociated lesions

50% have atrial50% have atrial septalseptal defect or patent foramendefect or patent foramen ovaleovale

25% have accessory bypass tract25% have accessory bypass tract

Braunwald, 8th ed

(c) 2000-2008 David Stultz, MD

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EbsteinEbstein’’ss Anomaly (TTE Apical 4 chamber)Anomaly (TTE Apical 4 chamber)

Feigenbaum, 6th ed

(c) 2000-2008 David Stultz, MD

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EKGEKG

http://pediatriccardiology.uchicago.edu/MP/CHD/Ebstein/Ebstein.htm

(c) 2000-2008 David Stultz, MD

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Invasive catheterization of RVInvasive catheterization of RV

(c) 2000-2008 David Stultz, MD

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ChestChest XrayXray(Infant)(Infant)

ACCSAP 6

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Natural HistoryNatural History

Variable!Variable!

If extreme, inIf extreme, in uteroutero deathdeath

If mild, may live into 9If mild, may live into 9thth decadedecade

Severe valve deformitySevere valve deformity –– symptoms develop insymptoms develop ininfancyinfancy

Moderate valve deformityModerate valve deformity –– symptoms insymptoms inadolescenceadolescence

Braunwald, 8th ed

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Indications for interventionIndications for intervention

Significant cyanosisSignificant cyanosis

RightRight--sided heart failuresided heart failure

Declining functional capacity (Declining functional capacity (≥≥Class IIIClass IIINYHA)NYHA)

Relative indicationsRelative indications

Paradoxical emboliParadoxical emboli

RecurrentRecurrent supraventricularsupraventricular arrhythmiasarrhythmias

Asymptomatic substantialAsymptomatic substantial cardiomegalycardiomegaly

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Surgical TechniquesSurgical Techniques

Tricuspid Valve RepairTricuspid Valve Repair Anterior leaflet cannot be tethered downAnterior leaflet cannot be tethered down Repair results inRepair results in ““monocuspidmonocuspid”” valvevalve

Valve replacementValve replacement –– bioprostheticbioprosthetic ConcomitantConcomitant

Accessory path ablationAccessory path ablation MAZE procedure if atrial fibrillation presentMAZE procedure if atrial fibrillation present Closure of PFO/ASDClosure of PFO/ASD

Unusual proceduresUnusual procedures BidirectionalBidirectional cavopulonarycavopulonary shuntshunt FontanFontan

1010--20% will develop20% will develop supraventricularsupraventricular arrhythmias afterarrhythmias after EbsteinEbsteinrepairrepair

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Carcinoid SyndromeCarcinoid Syndrome

Caused by carcinoid producing tumorCaused by carcinoid producing tumor

Symptoms of diarrhea and flushing, usually occurringSymptoms of diarrhea and flushing, usually occurringtogethertogether

Asthma less commonAsthma less common

1010--40% incidence of cardiac involvement40% incidence of cardiac involvement

About 10% of carcinoid tumors cause the carcinoidAbout 10% of carcinoid tumors cause the carcinoidsyndromesyndrome MostlyMostly midgutmidgut tumors cause syndrometumors cause syndrome

Caused by overproduction of 5HTCaused by overproduction of 5HT (5(5--Hydroxytryptophan)Hydroxytryptophan)

Harrsion’s, 17th ed

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Carcinoid Tumor Location, Frequency of Metastases, andCarcinoid Tumor Location, Frequency of Metastases, andAssociation with the Carcinoid SyndromeAssociation with the Carcinoid Syndrome

—3.913.6Rectum

Hindgut

50—<0.1Testis

50321.0Ovary

—32.20.4Liver

5518.6Colon

<138.84.8Appendix

13—0.5Meckel's diverticulum

914.9Ileum

958.41.8Jejunum

Midgut

135.727.9Bronchus, lung, trachea

517.80.3Gallbladder

2071.90.7Pancreas

3.4—2.0Duodenum

9.5104.6Stomach

——<0.1Esophagus

Foregut

Incidence of CarcinoidSyndrome

Incidence ofMetastases

Location(% of Total)

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Diagnosis of CarcinoidDiagnosis of Carcinoid

Urinary 5HIAA (Urinary 5HIAA (HydroxyindolaceticHydroxyindolacetic acid)acid)

Normal excretion is 2Normal excretion is 2--8mg/day8mg/day

78% sensitivity, 100% specificity78% sensitivity, 100% specificity

Platelet serotoninPlatelet serotonin –– more sensitivemore sensitive

Plasma serotoninPlasma serotonin

SerumSerum chromograninchromogranin AA

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Anatomic LocalizationAnatomic Localization

CT scan combined with nuclear imagingCT scan combined with nuclear imaging

Carcinoid tumors often express highCarcinoid tumors often express high--affinityaffinitysomatostatin receptorssomatostatin receptors

IndiumIndium--111111--pentetreotide is apentetreotide is a radiolabeledradiolabeledsomatostatin analoguesomatostatin analogue

False positive uptake can occur withFalse positive uptake can occur withgranulomasgranulomas, wounds, thyroid disease, wounds, thyroid disease

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CT LocalizationCT Localizationcombined withcombined with

SomatastatinSomatastatin ReceptorReceptorScintigraphyScintigraphy

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TreatmentTreatment

Carcinoid Syndrome:Carcinoid Syndrome:

OctreotideOctreotide

Somatostatin analogueSomatostatin analogue

100mcg SQ q8h; up to 3 mg/day100mcg SQ q8h; up to 3 mg/day

Controls symptoms of diarrhea and flushingControls symptoms of diarrhea and flushing

InterferonInterferon

Hepatic arteryHepatic artery embolizationembolization

Surgical resectionSurgical resection

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Carcinoid effects on the HeartCarcinoid effects on the Heart

Seen with Carcinoid SyndromeSeen with Carcinoid Syndrome

Tricuspid andTricuspid and PulmonicPulmonic ValveValve

StenosisStenosis and Regurgitationand Regurgitation

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SummarySummary

TricuspidTricuspid StenosisStenosis

RheumaticRheumatic

Tricuspid RegurgitationTricuspid Regurgitation

Secondary CausesSecondary Causes

PulmonicPulmonic ValveValve StenosisStenosis

CongenitalCongenital

PulmonicPulmonic Valve RegurgitationValve Regurgitation

Secondary CausesSecondary Causes

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ReferencesReferences

Crawford MH,Crawford MH, DiMarcoDiMarco JP,JP, PaulusPaulus WJ. Cardiology 2WJ. Cardiology 2ndnd ed. New York:ed. New York: MosbyMosby,,20042004

FauciFauci AS,AS, BraunwaldBraunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL,E, Kasper DL, Hauser SL, Longo DL, Jameson JL,LoscalzoLoscalzo J: Harrison's Principles of Internal Medicine, 17J: Harrison's Principles of Internal Medicine, 17thth ed. McGrawed. McGraw--HillHillProfessional, 2008.Professional, 2008.

FeigenbaumFeigenbaum H, Armstrong WF, Ryan T: Echocardiography 6H, Armstrong WF, Ryan T: Echocardiography 6thth ed.ed. LippincottLippincottWilliams & Wilkins, 2004.Williams & Wilkins, 2004.

Libby P,Libby P, BonowBonow RO, Mann DL,RO, Mann DL, ZipesZipes DP:DP: Braunwald'sBraunwald's Heart Disease: AHeart Disease: ATextbook of Cardiovascular Medicine 8Textbook of Cardiovascular Medicine 8thth ed. Philadelphia, WB Saunders,ed. Philadelphia, WB Saunders,20072007

(c) 2000-2008 David Stultz, MD