aortic and pulmonary valve disease

8/11/2019 Aortic and Pulmonary Valve Disease

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AORTICVALVEDISEASEObjectives:Aftercompletingthischapterthestudentshouldbeabletostatethecausesofleft

ventricularoutflowtractobstruction,theclinicalpresentationandphysicalfindingsinaorticstenosis,

thedifferentetiologiesandthepathophysiologyofthedisease,thenaturalhistory,investigativetools

andtherapyforaorticstenosis.

Thestudentshouldbeableaswelltodifferentiatebetweentheacuteandchronicformsofaortic

regurgitationasregardsthecausesandclinicalpresentation.Theetiology,pathophysiology,naturalhistory,investigativetoolsandtherapyforaorticregurgitationshouldbememorized.

1AORTICSTENOSIS

Valvularaorticstenosisisthemostcommoncauseofleftventricularoutflowtractobstruction.

Othercausesinclude

Subvalvularaorticstenosisbyafixedmembraneorafibromusculartunnel

Dynamicsubvalvularobstructioncausedbyhypertrophiccardiomyopathy

Supravalvularaorticstenosis

Etiology:1.CongenitalAorticStenosis:

Themostcommonisthebicuspidaorticvalve,whichleadstoprematuredegenerationand

calcificationofthecusps(fig.1.). Abnormaltrileafletvalvewithleafletfusion,unicuspidandcommissural

valveareothercongenitalcausesforstenosis.

Figure1.Normaltricuspidandabnormalbicuspidaorticvalve

2.AcquiredAorticStenosis:

i. DegenerativeCalcificAorticStenosis:

Thisoccursduetoabnormalcalcificationinatrileafletaorticvalveusuallyinthe7thor

8thdecades.Thecalcificationmayalsoinvolvethemitralannulusorextendintotheconduction

system,resultinginatrioventricularorintraventricularconductiondefects.Seniledegenerative

ASisnowtheleadingindicationforaorticvalvereplacement(AVR).

ii.

RheumaticAortic

stenosis: Itisusuallypresentwithcoexistingmitralvalvedisease,with

fibrosis,calcificationandcommissuralfusion

iii.

OtherinfrequentcausesofASincludeobstructivevegetations, homozygoustypeII

hypercholesteroemia,Pagetdisease,Fabrydisease, ochronosis,andirradiation.

Pathophysiology:

1. Theaorticvalveorificecontinuestogetnarrowerwithtime,andastheaorticvalvearea

decreasestheleftventriclefacesahigherafterload.


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2. Tomaintainthecardiacoutputtheleftventriclemustdevelophighersystolicpressureswhich

increasesthewallstress.Thisleadstocompensatoryleftventricularhypertrophywhichallows

theleftventricularwallstresstonormalize.

3. LVsystolicfunctionisusuallywellpreserved,andcardiacoutput(CO)ismaintainedformany

yearsdespitealargepressuregradientacrosstheAV.Duringthistime,thepatientdoesnot

experienceareductioninrestingCO,LVdilatation,ordevelopmentofsymptoms.

4. Theleftventricularcompliancedecreasesandwithalesscompliantleftventriclelessfilling

occurspassivelyintheearlyphaseofdiastole.Leftatrialcontractionbecomesmoreimportantin

maintaininganadequatepreload.

5. ASintensifiestheseverityofexistingmitralregurgitation(MR)byincreasingtheventricular

pressuregradientresponsiblefordrivingbloodfromtheleftventricletotheleftatrium.

Additionally,functionalMRasaconsequenceofLVdilatationinlatestagesofASmay

superimposethehemodynamicchangesassociatedwiththislesiononthoseproducedbyAS.

6. Themyocardialoxygendemandincreasesduetoanincreaseoftheleftventricularmass,

increasedsystolicpressureandprolongationofsystole.

7. Theelevationoftheleftventricularenddiastolicpressuredecreasestheperfusionpressure

across

the

coronary

vascular

bed

with

diminution

of

the

endocardial

O2

supply.

Therefore,

the

subendocardiumissusceptibletolownutrientflow,andthisunderperfusionresultsin

myocardialischemia.

NaturalHistory

Patientswithpureaorticstenosisdonothavesymptomsuntilthe aorticvalveareaislessthan

1.0cm2.

Thereisalonglatentperiodorphaseduringwhichthepatienthasnosymptoms.

Therateofincreaseofthemeanaorticpressureisabout7mmHg/year.

Asymptomaticpatients,evenwith criticalAS,haveanexcellentprognosis regarding survival,

withanexpecteddeath rateof less than1%peryear;only4%of suddencardiacdeaths in

severeASoccurinasymptomaticpatients.

Withtheonsetofsymptomsthesurvivalratedecreasessignificantly:

Onsetofanginaisassociatedwithanaveragesurvivalof5years,

Onsetofsyncopeisassociatedwithanaveragesurvivalof3years,

Onsetofcongestiveheartfailureisassociatedwithanaveragesurvivalof2years.

More than 50% of deaths were sudden. Death in general, including sudden death, occurs

primarilyinsymptomaticpatients.

ClinicalPresentation:

Aortic

stenosis

should

by

suspected

when

any

patient

has

a

systolic

ejection

murmur

at

the

right

upper

sternalborderthatradiatestothecarotidarteries.Mostpatientsareasymptomaticatpresentation.

1.Symptoms:

o TheclassicsymptomtriadofASincludesanginapectoris,syncope,andheartfailure.

o Exertionaldyspneaisthemostcommoninitialcomplaint,evenwithnormalLVsystolicfunction,

anditrelatestoabnormalLVdiastolicfunction.Paroxysmalnocturnaldyspnea,orthopnea,and

pulmonaryedemausuallyarelateoccurringsymptomsofheartfailure.

o Becauseanginacommonlyisprecipitatedbyexertionandrelievedbyrest,itoftensimulates

symptomsofCAD.


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o Anginaresultsfromaconcomitantincreasedoxygenrequirementbythehypertrophic

myocardiumanddiminishedoxygendeliverysecondarytotheexcessivecompressionof

coronaryvesselsandrelativesubendocardialmyocardialischemia.Ofcourse,anginaalsocan

resultfromcoexistentCAD.

o Riskofinfectiveendocarditisishigherinyoungerpatientswithmildvalvulardeformitythanin

olderpatientswithdegeneratedcalcifiedAVs,butitcanoccurinboth.

o EmbolizationfromacalcifiedorinfectedAVthatresultsinunilateralvisionloss,focalneurologic

signs,andmyocardialinfarctioncanbethefirstsignsofAVpathology.

2.PhysicalFindings:

(a)Arterialpulse:

Pulsusparvusettardus(Diminishedamplitudeanddelayed),inelderlyindividuals,thismaynotbe

presentdespiteseverestenosisbecauseofmorerigidaortaandcarotidvessels.

PulsusalternanscanoccurwiththeonsetofLVfailure.

Asystolic"thrill"maybepresentatthesecondrightintercostalspaceoratthesuprasternalnotchand

usuallyindicatesameanAVgradienthigherthan50mmHg.Thethrillisbestfeltwhilethepatientis

leaningforward.Onoccasion,itcanbetransmittedtothecarotids.

(b)Palpation:Leftventricularhypertrophyisnotaccompaniedbyenlargementoftheleftventricle;thus

theapicalimpulseisnotdisplaced.Theimpulseissustainedduetotheprolongationofsystole.

(c)Auscultation:

o S1isusuallynormalorsoft.

o Theaorticcomponentofthesecondheartsound,A2,isusuallydiminishedorabsentbecausethe

AViscalcifiedandimmobileand/oraorticejectionisprolongedorburiedintheprolonged

systolicejectionmurmur.ThepresenceofanormalorA2speaksagainstthepresenceofsevere

AS.

o ParadoxicalsplittingoftheS2alsooccursbecauseoflateclosureofA2.

o

P2may

also

be

accentuated

when

LV

failure

leads

to

secondary

pulmonary

hypertension.

o Thepresenceofanejectionclickisdependentonthemobilityofthevalvecuspsanddisappears

whentheybecomeimmobileandseverelycalcified.Thus,itiscommoninchildrenandyoung

adultswithcongenitalASbutrareinelderlyindividualswithacquiredcalcificASwithrigidvalves.

o AprominentS4isusuallypresentduetoforcefulatrialcontractionintoahypertrophiedleft

ventricle.

o TheclassiccrescendodecrescendosystolicmurmurofASisbestheardatthesecondintercostal

spaceintherightuppersternalborder;itisharshatthebaseandradiatestobothcarotid

arteries.

o However,themurmurmaybemoreprominentattheapexinelderlypersonswithcalcificAS

duetoradiationofthehighfrequencycomponentsofthemurmurtotheapex(Gallavardin

phenomenon)leadingtoitsmisinterpretationasamurmurofMR.

o Themoreseverethestenosis,thelongerthedurationofthemurmurandthemorelikelyit

peaksatmidtolatesystole.Whentheleftventriclefailsandcardiacoutputfalls,theAS

murmurbecomessofterandmaybebarelyperceptible.

DifferentialDiagnosis:

o Supravalvularaorticstenosis

o Congenitalsubvalvularaorticstenosis

o Hypertrophicobstructivecardiomyopathy


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o Mitralregurgitationduetoposteriormitralleafletdysfunction

Complications

o Suddencardiacdeath

o Heartfailure

o Conductiondefects

o Infectiveendocarditis

o Calcificembolization

LaboratoryExamination:

1. ECG:

o AlthoughtheECGfindingsmaybeentirelynormal,theprincipalfindingisleftventricular

hypertrophy(LVH),whichisfoundin85%ofpatientswithsevereAS;however,its

absencedoesnotprecludecriticalAS.

o TwaveinversionandSTsegmentdepressioninleadswithpredominantlypositiveQRS

complexesarecommon.STdepressionexceeding0.3mVinpatientswithASindicatesLV

strainandsuggeststhatsevereLVHispresent(fig.2).

Fig.2.ECGinapatientwithsevereaorticstenosisshowingLVHwithstrain

2. ChestXray:Maybeentirelynormal(Notuseful).Ifcardiomegalyispresentitusuallydenotes

leftventriculardysfunctionorassociatedaorticregurgitation.Poststenoticdilatationofthe

ascendingaortamaybeevident.Onlateralview,AVcalcificationisfoundinalmostalladults

withhemodynamicallysignificantAS.

DiagnosticTesting:

1) Echocardiography:(fig.3&4)

o Thisisthemethodofchoiceforestablishingthediagnosisandassessingitsseverity.It

measuresthetransvalvulargradientandtheaorticvalvearea.Thenormalvalveareais2

4cm2.Avalveareaof1.5cm

2ismild,1.01.5cm

2ismoderatewhilelessthan1.0cm

2is

severeaorticstenosis(table1).

o InvalvularAS,theetiology(bicuspid,rheumatic,orseniledegenerative)maybe

assessed.LVsize,mass,andfunctionshouldbeevaluatedineachpatient.


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Cardiaccatheterization:Whenthereisdoubtaboutthediagnosisandinpatientswithangina

cardiaccatheterizationisindicated;inadditionmalesabovetheageof40yearsandfemales

abovetheageof50yearsarecandidatesforcoronaryangiography(fig.5).

Treatment:

1) PriorityofTherapy:Themainstayoftherapyforsevereaorticstenosisissurgicalreplacementof

theaorticvalve.

Thisisrestrictedtopatientswithsymptomsbecausesurvivalbenefitisapparentonly

aftersymptomsoccur.

Olderpatientswithcriticalstenosisandyoungpatientswithveryhighgradientsmay

benefitfromelectivesurgery.

2) MedicalTreatment:

Antibioticprophylaxisbeforeproceduresismandatory.

Managementofpatientswithoutsymptomsisdirectedtowardsprimaryprevention

ofcoronaryarterydisease,maintenanceofsinusrhythmandproperbloodpressure

control.Patientsareinstructedtoreportsymptoms.

Therapyforheartfailureisdirectedatvolumecontroltoreliefpulmonarycongestion.

Diureticsshouldbeusedwithcaution.Vasodilatorsincludingnitratesshouldbe

avoidedinpatientswithsevereaorticstenosisandheartfailure.

Atrialfibrillationispoorlytolerated,sincetheatrialcomponentisimportantforLV

filling;thecadiacoutputdecreasesandpulmonarycongestionrapidlyensues.Thus

rhythmcontrolshouldbeattemptedifpossible.

3) PercutaneousTherapy: PercutaneousAorticBalloonValvuloplasty(PABV)hassomeroleonlyinpediatric

congenitalaorticstenosishoweverwithanearlyrestenosisrate.

Itisausefultechniqueforpatientswhoarenotcandidatesforsurgicaltreatment

becauseofcomorbiditiesoradvancedage,orperhapsasabridgetononelective

surgicaltreatment.

Transcatheteraorticvalveimplantation(TAVI):thisisanovelandexcitingtechnique

whereby anartificialaorticheartvalveattachedtoawireframeisguidedbycatheter

totheheart.Onceintheproperpositionintheheart,thewireframeexpands,

Fig.5.


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allowingthenewaorticvalvetoopenand functioninposition.TAVIisrecommended

intheveryelderlypatientswithseveresymptomaticASwhoareconsidered

unsuitableforconventionalsurgerybecauseofseverecomorbiditiesandhighriskof

preioperativemortality.

4) SurgicalTherapy:

Replacementoftheaorticvalveisthestandardmethodfortreatmentofvalvularaortic

stenosis

AORTICREGURGITATIONBackground:

Aorticregurgitationcanbeclassifiedasbeingtheresultofanacuteorchronicprocess.

Chronicaorticregurgitationistheresultoffailureofcoaptationoftheaorticvalveleaflets

causedbydiseasedvalvecusps,dilatationoftheaorticrootorboth.

Acuteaorticregurgitationisusuallyassociatedwithbluntchesttrauma,endocarditisoraortic

dissectionandisasurgicalemergencyinmostsituations.

A.Clininal

Presentation

1.Signsand

Symptoms:

a)

Chronic

Aortic

Regurgitation:

Patientsareusuallyasymptomaticforalongtimeandwhensymptomsdeveloptheyare

usuallyrelatedtopulmonarycongestion;firsteffortdyspnea,orthopneaandparoxysmal

nocturnaldyspneaandlatersignsofrightheartfailure.

Anginabaybepresentduetodiminishedpressuregradientacrossthecoronarybed.

b)

Acute

Aortic

Regurgitation:

TheLVdoesnothavesufficienttimetodilateinresponsetothesuddenincreasein

volume.

Asaresult,LVenddiastolicpressureincreasesrapidly,causinganincreaseinpulmonary

venouspressure.

Aspressureincreasesthroughoutthepulmonarycircuit,thepatientdevelopsdyspnea

andpulmonaryedema.

Earlysurgicalinterventionshouldbeconsidered(particularlyifARisduetoaortic

dissection,inwhichcasesurgeryshouldbeperformedimmediately).

2.PhysicalFindings:

GeneralExamination:

ExamineforMarfanoidcharacteristicsamongyoungpatientswithaorticregurge.(Ectopialentis;

higharchedpalate;pectusandarachnodactly)

Signsof

bacterial

endocarditis.

Prominentapicalimpulse.

Bloodpressure:Wideningofthepulsepressurecausinghyperdynamiccirculationwithan

elevatedsystolicandanabnormallylowdiastolicpressure.

ArterialExamination:Water Hammerpulse. Pulsepressureiswidened,associatedwithalow

diastolicpressure,oftenlessthan60mmHg.

Palpation:Forceful hyperdynamicapicalimpulse.


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Auscultation:

Blowingearlydiastolicdecrescendomurmur,bestheardintheleftuppersternal border,sitting

leaningforwardatexpiration.

AconcomitantsystolicejectionmurmuriscommoninmoderatetosevereARduetothe

increasedvolumeofbloodflowingacrosstheaorticvalve.

AntegradeflowacrossapartiallyclosedmitralvalveisthoughttocauseanAustinFlintmurmur,

whichisamid andlatediastolicapicallowfrequencymurmurorrumble.

S4:OftenpresentduetoLVHandpoorLVcompliance.S1:ItsintensitydecreasesasLVfunction

worsens.TheappearanceofS3impliesleftventricularfailure.

Physical

findings

related

to

widening

of

the

pulse

pressure

(table

2)

SIGN PHYSICALFINDING

Musset'sSign Headbobbingwitheachbeat

Muller'sSign SystolicpulsationoftheUvula

Hill'sSign Poplitealcuffpressure>40mmHgabovebrachial

WaterHammerPulse

(Corriganspulse)

Rapiddistensionandcollapseofarterial

pulse

Quincke'sPulse Capillarypulsationsvisibleinthe

fingernailbedsandlips

Duroziez'sSign Toandfromurmuroverthefemoral

arterywiththearterycompressed

Pistolshotsounds Prominentsystolicanddiastolicsounds

overthefemorals

B.Etiology

of

Aortic

Regurgitation

(table

3)

TypeofAbnormalityAcuteAortic

Regurgitation

ChronicAortic

Regurgitation

Abnormalities

in

the

aortic

root

resulting

in

distortedcuspsuspension

1.Aorticdissection

2.Traumaticdissection

1.Marfan'sSyndrome

2.Aorticaneurysm

3.Annuloaorticectasia

4.SyphiliticAortitis

5.SystemicLupus

erythematosis

6.Ankylosing

Spoddylitis


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C.Pathophysiology

1.ChronicAorticRegurgitation:

ChronicARproducesleftventricular(LV)volumeoverloadthatleadstoaseriesofcompensatory

changes,includingleftventricularenlargementandeccentricleftventricularhypertrophy,as

opposedtoconcentrichypertrophyobservedinapressureoverloadstate(ie,aorticstenosis).

Withincreasedleftventricularenddiastolicvolumethestrokevolumewillincrease.

Theleftventricleaccommodatesfortheregurgitantvolumewithoutincreaseoftheenddiastolic

pressure.

Leftventricularsystolicdysfunctioneventuallydevelops,leadingtoprogressivedilatationand

impairedemptyingoftheleftventricle.ThustheEjectionfractionwilldecreasewithanincrease

oftheenddiastolicvolumeandenddiastolicpressure.

2.AcuteAorticRegurgitation:

Thereisarapidincreaseoftheleftventricularenddiastolicvolume,withnotimefor

hypertrophyorabilityoftheLVtoaccommodate.

Theleftventricularenddiastolicpressure(LVEDP)increasesrapidly,prematurelyclosingthe

mitralvalveandcausingdiastolicmitralregurgitation.

TherapidincreaseoftheLVEDPleadstopulmonaryedema.

NaturalHistory:

TheprognosisofsevereARinasymptomaticpatientswithnormalLVfunctionremainsexcellent,

butextravigilanceisrequiredinmonitoringthesepatientstoensurethattheoptimaltimefor

surgicalinterventionisnotoverlooked.

Oncesymptomsdevelopinchronicaorticregurgitationthereisrapidprogressionanddeclinein

thepatient'sfunctionalstatus.

Theleftventricularejectionfraction(LVEF)isthemostimportantdeterminantofsurvival.

Inmedicallytreatedpatientswithmildtomoderateaorticregurgitationthe10yearsurvivalis

8595%;whileinthosewithmoderatetosevereaorticregurgitationthe10yearsurvivalis50%

only.

Inchronicaorticregurgitationsurvivalratesimprovedwithaggressiveuseofvasodilator

therapy(Nifedipine/AngiotensinConvertingEnzymeInhibitors).However,medicaltherapydoes

notreplacesurgerywhenindicated.

Suddendeathmayoccuramongpatientswithseveresymptomaticaorticregurgitation.

Abnormalities

of

the

aorticvalvecusps

1.Infectious

Endocarditis

2.Traumaticleaflet

Inversionorprolapse

1.Bicuspidvalve

2.Rheumaticheart

disease

3.Calcificdegeneration

4.SinusofValsalva

aneurysm.

5.Myxomatous

degeneration.


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LaboratoryTesting

ECG:TypicallyshowsLVHwithupright"t"wavesandleftatrialabnormality.Atrialfibrillationif

associatedmitralvalvediseaseispresent.

ChestRadiography:

DramaticCardiomegaly(CorBovinum)

Ascendingaortadilatation.

Leftatrialdilatation.

Echocardiography:Estimatesseverityofaorticregurgitation,leftventricularsizeandsystolic

function(fig.6).

Fig.6.Themosaiccolorrepresentssevereaorticregurgitation

onthis2dimensionalechopicture

CardiacCatheterization:Malesabove40yearsandfemalesabove50yearsgoingforaorticvalve

replacementneedtheircoronariesvisualizedbyangiography.Itisalsoofvaluetoconfirmthe

hemodymamicfindingsanddegreeofregurgitationbyaortographywhencontradictorydata

fromotherinvestigationsarepresent.

Treatment

PrioritiesofTherapyaretoestablishthecause,ensurethatthepatient'sconditionis

hemodynamicallystableanddeterminetheneedforandtimingofsurgicalintervension.

MedicalTherapy:

Considerantibioticprophylaxisforpatientswithendocarditiswhenperformingprocedures

likelytoresultinbacteremia

InseverechronicAR,vasodilatortherapymaybeusedinselectconditionstoreduce

afterloadinpatientswithsystolichypertension,inordertominimizewallstressandoptimize

LVfunction.

SurgicalTherapy:SurgicaltreatmentofARusuallyrequiresreplacementofthediseasedvalvewitha

prostheticvalve.Itisindicatedin

AllsymptomaticpatientswithsevereARregardlessoftheLVfunction.

AsymptomaticpatientswithsevereARwithLVdysfunctionasevidencedbyan EF70

mmor LVESDis>50mm(byechocardiography)


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PulmonicStenosisPulmonicstenosis(PS)referstoadynamicorfixedanatomicobstructiontoflowfromthe right

ventricle(RV)tothepulmonaryarterialvasculature.

Etiology

Congenitaleitherinisolationorinassociationwithothercongenitalcardiacdefects

Rarelyrheumaticheartdisease

Carcinoidsyndrome

RVoutflowtractmassesandtumours

Pathophysiology

PScanbeduetoisolatedvalvular(90%),subvalvular,orperipheral(supravalvular)obstruction,oritmay

befoundinassociationwithmorecomplicatedcongenitalheartdisorders.

Valvularpulmonicstenosis

IsolatedvalvularPScomprisesapproximately10%ofallcongenitalheartdisease.Typically,the

valvecommisuresarepartiallyfusedandthe3leafletsarethinandpliant,resultinginaconical

ordomeshapedstructurewithanarrowedcentralorifice.Poststenoticpulmonaryartery

dilatationmayoccurowingto"jeteffect"hemodynamics.

WithseverevalvularPS,subvalvularrightventricularhypertrophycancauseinfundibular

narrowingandcontributetotherightventricularoutflowobstruction.Thisoftenregressesafter

correctionofvalvularstenosis.

SubvalvularPSoccursasanarrowingoftheinfundibularorsubinfundibularregion,oftenwitha

normalpulmonicvalve.ThisconditionispresentinindividualswithtetralogyofFallotandcanalso

beassociatedwithaventricularseptaldefect.

ClinicalPicture

History:

Mostchildrenandadultswithmildtomoderatelyseverepulmonicstenosis(PS)are

asymptomatic.

ThosewithseverePSmayexperienceexertionaldyspneaandfatigue.

Inextremelyrarecases,patientspresentwithexertionalangina,syncope,orsuddendeath.

Peripheraledemaandothertypicalsymptomsoccurwithrightheartfailure.

Cyanosisispresentinthosewithsignificantrighttoleftshuntviaapatentforamenovale,atrial

septaldefect,orventricularseptaldefect

Physicalexam

precordialheaveorapalpableimpulsefromtheRValongtheleftparasternalbordermaysuggestseverePS.Intheleftuppersternalborder,asystolicthrillmaybepalpableatthelevelof

thesecondintercostalspace.

InvalvularPS,auscultationrevealsanormalS1andawidelysplitS2,withasoftanddelayedP2.

ValvularPStypicallycausesasystoliccrescendodecrescendoejectionmurmurintheleftupper

sternalborderthatincreaseswithinspirationandradiatesdiffusely.Asystolicejectionclickmay

precedethemurmur.


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Diagnosis

EchocardiographyEchocardiographyprovidesadefinitiveconfirmationofthediagnosisofPS.Both

2dimension.alandDopplertechniquesshouldbeusedtocomprehensivelyevaluatethepulmonic

valve.Thepulmonaryvalveareaofahealthyadultis2.0cm2/m

2ofbodysurfacearea.

MildvalvularPSisdefinedbyavalvearealargerthan1cm2andatransvalvularpressuregradient

oflessthan50mmHg.

ModeratelyseverePSoccursifthevalveareais0.51.0cm2,withatransvalvularpressure

gradientbetween50and75mmHg.

SeverePSisdefinedbyavalveareasmallerthan0.5cm2andatransvalvularpressuregradient

greaterthan75mmHg.

Treatment

Percutaneousballoonvalvuloplastyhasbecometheinitialinterventioninchildren,adolescents,and

adultswithcongenitalvalvarPS.Balloonvalvuloplastyshouldbeconsideredinanypatientwitha

transvalvularpressuregradientgreaterthan50mmHg.

PulmonaryRegurgitation

Etiology

Primarypulmonaryhypertension(idiopathic)

Secondarypulmonaryhypertension.Thisisthemostcommoncauseinadults

Rheumaticheartdisease(rare)

Infectiveendocarditis

Carcinoiddisease

Medicationsthatactviaserotoninergicpathways(eg,methysergide,pergolide,fenfluramine)

Pathphsiology

Incompetenceofthepulmonicvalveoccursinanyoneof3basicpathologicprocesses:

Dilatationofthepulmonicvalvering

Acquiredalterationofpulmonicvalveleafletmorphology Congenitalabsenceormalformationofthevalve

History

Symptomsofrightsidedheartfailurecanoccurwhentheseverityanddurationoftheregurgitation

resultinrightventricularenlargementanddecompensation.Dyspneaonexertionisthemostcommon

complaint.Easyfatigability,lightheadedness,peripheraledema,chestpain,palpitations,andfrank

syncopemayoccur.

Physicalexam

Jugularvenouspressure(JVP)isusuallyincreased.Whenrightventricularenlargementispresent,a

palpableimpulse(liftorheave)isusuallypresentattheleftlowersternalborder.Palpablepulmonary

arterypulsationattheleftuppersternalbordermaybepresentinthesettingofsignificantpulmonary

arterydilatation.

TheGrahamSteellmurmurofpulmonaryhypertensionisahighpitched,earlydiastolicdecrescendo

murmurnotedovertheleftuppertoleftmidsternalareaandisaresultofhighvelocityregurgitantflow

acrossanincompetentpulmonicvalve.


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Diagnosis

Echocardiography.ColorflowDopplerechocardiographyisthemainstayforrecognizing

pulmonicregurgitation.

Treatment

1.

Inprimarypulmonaryvalveregurgitationtheprognosisisverygood,rarelyiscorrectionofthe

defectnecessary.

2.

Insecondarypulmonaryvalveregurgitationtheprognosisdependsonthecauseandisdirected

towardstheprimarydisease.

MCQ

1.Inaorticvalvedisease,markastrue orfalseX

a) Severeaorticstenosiswithleftventricularfailureisassociatedwithamediansurvivalofapprox.

2 years

b) Mildaorticstenosisinyoungpeopleisassociatedwithanegligibleriskofendocarditis

c) Longtermtreatmentwithnifedipineinaorticregurgitationisassociatedwithimprovedoutcome

d) Systolicbloodpressuremaybenormalorelevatedinpatientswithsevereaorticstenosis

1. A72yearoldgentlemanisreferredtoyoubecauseofaprecordialsystolicmurmur.On

examination,thereisaharshmidsystoliccrescendodecescendomurmurattherightparasternal

ICS,radiatingtothecarotids.Thepatientisasymptomatic.Echocardiographyrevealsanaortic

valveareaof 1.2cm2andameansystolicgradientof 30mmHgandanormalLVsystolic

function.

Whatisthemostappropriateinterventionforthispatientatthepresenttime?

a.Percutaneous balloonaorticvalvuloplasty

b.Aorticvalvereplacement

c.Conserveandfollowup

3.Matchthefollowingvalvelesionswiththeir associatedmurmur

a.Aorticstenosis 1.decrescendodiastolicblowingmurmurheardbestalongleftsternal border

b.Mitralregurgitation 2.holosystolic murmurattheapexradiatingtoleftaxilla

c Aorticregurgitation 3.crescendodecrescendosystolicmurmuralongtheleftsternalborder

d.Mitralvalveprolapse 4.midorlatesystolicmurmurattheapexthatmaybeprecededbyaclick

4.Hillssignmeansthat

a. Brachialandpoplitealarterialbloodpressuresareequal

b. Brachialarterialbloodpressureishigherthanpoplitealpressure

c. Poplitealarterialbloodpressureishigherthanbrachialby30mmHg

d. Popliteal arterialpulsationsarenotpalpable


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5Adiamondshapedmurmurrefersto

a. Crescendo

b. Decrescendo

c. Crescendodecrescendo

d. Noneoftheabove

6Murmurofaorticstenosisradiatesto

a. Axilla

b. Neck

c. Back

d. Rightshoulder

7 Aorticstenosisisalwaysofrheumaticoriginwhenassociatedwith

a. aorticregurgitation

b. mitralregurgitation

c. mitralstenosis

d. carotidbruit

8Themostcommonformofcongenitalvalvularaorticstenosisis

a. Unicommmisural

b. Bicuspid

c. Homozygoushypercholesterolemia

d. Ochronsis

9Themostcommonformofcompensatoryhypertrophyinaorticstenosisis

a. Eccentric

b. Concentric

c. Asymmetric

d. Alloftheabove

10 Onsetofsyncopeisassociatedwithanaveragesurvivalof

a. 1year

b. 3years

c. 5yearsd. 8years

11TheclassicsymptomtriadofASincludesallofthefollowingexcept

a. Angina

b. Syncope

c. Cyanosis

d. Heartfailure

12 Anejectionclickheardinapatientwithvalvularaorticstenosisimpliesanimmobileandcalcificvalve

a. True

b. False

13 Thesimplestandmostfeasibletoolforassessmentofseverityofaorticstenosisis

a. Cardiaccatheterization

b. Magneticresonanceimaging

c. Electrocardiography

d. Dopplerechocardiography


15/15

14 Apatientwithnewlydiscoveredaorticregurgitationandseverechestpainshouldbeconsideredtohave

a. Myocardialinfarction

b. Acutepericarditis

c. Aorticdissection

d. Syphyliticaortitis

15 Acuteaorticregurgitationmaybecausedby

a. Bluntchesttrauma

b. Infective

endocarditis

c. Aortic dissection

d. Alloftheabove

16 Preoperativepredictorsofpoorpostoperativesurvival inpatientswithsevereaorticregurgitationainclude

allofthefollowingexcept:

a. LVESDgreaterthan55mm

b. LVEFlessthan50%

c. NYHACHFclassIII,IV

d. Waterhammerpulse

aortic and pulmonary valve disease

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