AORTICVALVEDISEASE

Objectives:Aftercompletingthischapterthestudentshouldbeabletostatethecausesofleft
ventricularoutflowtractobstruction,theclinicalpresentationandphysicalfindingsinaorticstenosis,
thedifferentetiologiesandthepathophysiologyofthedisease,thenaturalhistory,investigativetools
andtherapyforaorticstenosis.
Thestudentshouldbeableaswelltodifferentiatebetweentheacuteandchronicformsofaortic
regurgitationasregardsthecausesandclinicalpresentation.Theetiology,pathophysiology,natural
history,investigativetoolsandtherapyforaorticregurgitationshouldbememorized.
1AORTICSTENOSIS
Valvularaorticstenosisisthemostcommoncauseofleftventricularoutflowtractobstruction.
Othercausesinclude
Subvalvularaorticstenosisbyafixedmembraneorafibromusculartunnel
Dynamicsubvalvularobstructioncausedbyhypertrophiccardiomyopathy
Supravalvularaorticstenosis
Etiology:
1.CongenitalAorticStenosis:
Themostcommonisthebicuspidaorticvalve,whichleadstoprematuredegenerationand
calcificationofthecusps(fig.1.).Abnormaltrileafletvalvewithleafletfusion,unicuspidandcommissural
valveareothercongenitalcausesforstenosis.

Figure1.Normaltricuspidandabnormalbicuspidaorticvalve
2.AcquiredAorticStenosis:
i. DegenerativeCalcificAorticStenosis:
Thisoccursduetoabnormalcalcificationinatrileafletaorticvalveusuallyinthe7
th
or
8
th
decades.Thecalcificationmayalsoinvolvethemitralannulusorextendintotheconduction
system,resultinginatrioventricularorintraventricularconductiondefects.Seniledegenerative
ASisnowtheleadingindicationforaorticvalvereplacement(AVR).
ii. RheumaticAorticstenosis:Itisusuallypresentwithcoexistingmitralvalvedisease,with
fibrosis,calcificationandcommissuralfusion
iii. OtherinfrequentcausesofASincludeobstructivevegetations,homozygoustypeII
hypercholesteroemia,Pagetdisease,Fabrydisease,ochronosis,andirradiation.
Pathophysiology:
1. Theaorticvalveorificecontinuestogetnarrowerwithtime,andastheaorticvalvearea
decreasestheleftventriclefacesahigherafterload.
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
acrossthecoronaryvascularbedwithdiminutionoftheendocardialO2supply.Therefore,the
subendocardiumissusceptibletolownutrientflow,andthisunderperfusionresultsin
myocardialischemia.
NaturalHistory
Patientswithpureaorticstenosisdonothavesymptomsuntiltheaorticvalveareaislessthan
1.0cm
2
.
Thereisalonglatentperiodorphaseduringwhichthepatienthasnosymptoms.
Therateofincreaseofthemeanaorticpressureisabout7mmHg/year.
Asymptomatic patients, even with critical AS, have an excellent prognosis regarding survival,
with an expected death rate of less than 1% per year; only 4% of sudden cardiac deaths in
severeASoccurinasymptomaticpatients.
Withtheonsetofsymptomsthesurvivalratedecreasessignificantly:
Onsetofanginaisassociatedwithanaveragesurvivalof5years,
Onsetofsyncopeisassociatedwithanaveragesurvivalof3years,
Onsetofcongestiveheartfailureisassociatedwithanaveragesurvivalof2years.
More than 50% of deaths were sudden. Death in general, including sudden death, occurs
primarilyinsymptomaticpatients.
ClinicalPresentation:
Aorticstenosisshouldbysuspectedwhenanypatienthasasystolicejectionmurmurattherightupper
sternalborderthatradiatestothecarotidarteries.Mostpatientsareasymptomaticatpresentation.
1.Symptoms:
o TheclassicsymptomtriadofASincludesanginapectoris,syncope,andheartfailure.
o Exertionaldyspneaisthemostcommoninitialcomplaint,evenwithnormalLVsystolicfunction,
anditrelatestoabnormalLVdiastolicfunction.Paroxysmalnocturnaldyspnea,orthopnea,and
pulmonaryedemausuallyarelateoccurringsymptomsofheartfailure.
o Becauseanginacommonlyisprecipitatedbyexertionandrelievedbyrest,itoftensimulates
symptomsofCAD.
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 S
1
isusuallynormalorsoft.
o Theaorticcomponentofthesecondheartsound,A
2
,isusuallydiminishedorabsentbecausethe
AViscalcifiedandimmobileand/oraorticejectionisprolongedorburiedintheprolonged
systolicejectionmurmur.ThepresenceofanormalorA
2
speaksagainstthepresenceofsevere
AS.
o ParadoxicalsplittingoftheS
2
alsooccursbecauseoflateclosureofA
2
.
o P
2
mayalsobeaccentuatedwhenLVfailureleadstosecondarypulmonaryhypertension.
o Thepresenceofanejectionclickisdependentonthemobilityofthevalvecuspsanddisappears
whentheybecomeimmobileandseverelycalcified.Thus,itiscommoninchildrenandyoung
adultswithcongenitalASbutrareinelderlyindividualswithacquiredcalcificASwithrigidvalves.
o AprominentS
4
isusuallypresentduetoforcefulatrialcontractionintoahypertrophiedleft
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
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
4cm
2
.Avalveareaof1.5cm
2
ismild,1.01.5cm
2
ismoderatewhilelessthan1.0cm
2
is
severeaorticstenosis(table1).
o InvalvularAS,theetiology(bicuspid,rheumatic,orseniledegenerative)maybe
assessed.LVsize,mass,andfunctionshouldbeevaluatedineachpatient.
o DopplerechocardiographyisanexcellenttoolforassessingtheseverityofAS.Usingthe
modifiedBernoulliequation,amaximuminstantaneousandmeanAVgradientcanbe
derivedfromthecontinuouswaveDopplervelocityacrosstheAV.

Fig.3. Calcific aortic stenosis by 2-dimensional echocardiography in parasternal long axis and short axis
views

Fig.4.Continous wave Doppler imaging in aortic stenosis. Aortic stenosis produces a high
velocity jet of blood across the aortic valve.
Table1.Differentgradesofseverityofvalvularaorticstenosis
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
wherebyanartificialaorticheartvalveattachedtoawireframeisguidedbycatheter
totheheart.Onceintheproperpositionintheheart,thewireframeexpands,
Fig.5.
allowingthenewaorticvalvetoopenandfunctioninposition.TAVIisrecommended
intheveryelderlypatientswithseveresymptomaticASwhoareconsidered
unsuitableforconventionalsurgerybecauseofseverecomorbiditiesandhighriskof
preioperativemortality.
4) SurgicalTherapy:
Replacementoftheaorticvalveisthestandardmethodfortreatmentofvalvularaortic
stenosis
AORTICREGURGITATION
Background:
Aorticregurgitationcanbeclassifiedasbeingtheresultofanacuteorchronicprocess.
Chronicaorticregurgitationistheresultoffailureofcoaptationoftheaorticvalveleaflets
causedbydiseasedvalvecusps,dilatationoftheaorticrootorboth.
Acuteaorticregurgitationisusuallyassociatedwithbluntchesttrauma,endocarditisoraortic
dissectionandisasurgicalemergencyinmostsituations.
A.ClininalPresentation
1.SignsandSymptoms:
a) ChronicAorticRegurgitation:
Patientsareusuallyasymptomaticforalongtimeandwhensymptomsdeveloptheyare
usuallyrelatedtopulmonarycongestion;firsteffortdyspnea,orthopneaandparoxysmal
nocturnaldyspneaandlatersignsofrightheartfailure.
Anginabaybepresentduetodiminishedpressuregradientacrossthecoronarybed.
b) AcuteAorticRegurgitation:
TheLVdoesnothavesufficienttimetodilateinresponsetothesuddenincreasein
volume.
Asaresult,LVenddiastolicpressureincreasesrapidly,causinganincreaseinpulmonary
venouspressure.
Aspressureincreasesthroughoutthepulmonarycircuit,thepatientdevelopsdyspnea
andpulmonaryedema.
Earlysurgicalinterventionshouldbeconsidered(particularlyifARisduetoaortic
dissection,inwhichcasesurgeryshouldbeperformedimmediately).
2.PhysicalFindings:
GeneralExamination:
ExamineforMarfanoidcharacteristicsamongyoungpatientswithaorticregurge.(Ectopialentis;
higharchedpalate;pectusandarachnodactly)
Signsofbacterialendocarditis.
Prominentapicalimpulse.
Bloodpressure:Wideningofthepulsepressurecausinghyperdynamiccirculationwithan
elevatedsystolicandanabnormallylowdiastolicpressure.
ArterialExamination:WaterHammerpulse.Pulsepressureiswidened,associatedwithalow
diastolicpressure,oftenlessthan60mmHg.
Palpation:Forcefulhyperdynamicapicalimpulse.

Auscultation:
Blowingearlydiastolicdecrescendomurmur,bestheardintheleftuppersternalborder,sitting
leaningforwardatexpiration.
AconcomitantsystolicejectionmurmuriscommoninmoderatetosevereARduetothe
increasedvolumeofbloodflowingacrosstheaorticvalve.
AntegradeflowacrossapartiallyclosedmitralvalveisthoughttocauseanAustinFlintmurmur,
whichisamidandlatediastolicapicallowfrequencymurmurorrumble.
S4:OftenpresentduetoLVHandpoorLVcompliance. S1:ItsintensitydecreasesasLVfunction
worsens.TheappearanceofS3impliesleftventricularfailure.
Physicalfindingsrelatedtowideningofthepulsepressure(table2)

SIGN PHYSICALFINDING
Musset'sSign

Headbobbingwitheachbeat
Muller'sSign

SystolicpulsationoftheUvula
Hill'sSign Poplitealcuffpressure>40mmHgabove
brachial
WaterHammerPulse
(Corriganspulse)
Rapiddistensionandcollapseofarterial
pulse
Quincke'sPulse Capillarypulsationsvisibleinthe
fingernailbedsandlips
Duroziez'sSign Toandfromurmuroverthefemoral
arterywiththearterycompressed
Pistolshotsounds Prominentsystolicanddiastolicsounds
overthefemorals
B.EtiologyofAorticRegurgitation(table3)
TypeofAbnormality
AcuteAortic
Regurgitation
ChronicAortic
Regurgitation
Abnormalitiesinthe
aorticrootresultingin
distortedcuspsuspension
1.Aorticdissection
2.Traumaticdissection
1.Marfan'sSyndrome
2.Aorticaneurysm
3.Annuloaorticectasia
4.SyphiliticAortitis
5.SystemicLupus
erythematosis
6.Ankylosing
Spoddylitis

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.
Abnormalitiesofthe
aorticvalvecusps

1.Infectious
Endocarditis
2.Traumaticleaflet
Inversionorprolapse
1.Bicuspidvalve
2.Rheumaticheart
disease
3.Calcificdegeneration
4.SinusofValsalva
aneurysm.
5.Myxomatous
degeneration.
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.
AsymptomaticpatientswithsevereARwithLVdysfunctionasevidencedbyanEF<50%
AsymptomaticpatientswithsevereARandLVenddiastolicdimension(LVEDD)is>70
mmorLVESDis>50mm(byechocardiography)

PulmonicStenosis
Pulmonicstenosis(PS)referstoadynamicorfixedanatomicobstructiontoflowfromtheright
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
precordialheaveorapalpableimpulsefromtheRValongtheleftparasternalbordermay
suggestseverePS.Intheleftuppersternalborder,asystolicthrillmaybepalpableatthelevelof
thesecondintercostalspace.
InvalvularPS,auscultationrevealsanormalS1andawidelysplitS2,withasoftanddelayedP2.
ValvularPStypicallycausesasystoliccrescendodecrescendoejectionmurmurintheleftupper
sternalborderthatincreaseswithinspirationandradiatesdiffusely.Asystolicejectionclickmay
precedethemurmur.

Diagnosis
EchocardiographyEchocardiographyprovidesadefinitiveconfirmationofthediagnosisofPS.Both
2dimension.alandDopplertechniquesshouldbeusedtocomprehensivelyevaluatethepulmonic
valve.Thepulmonaryvalveareaofahealthyadultis2.0cm
2
/m
2
ofbodysurfacearea.
MildvalvularPSisdefinedbyavalvearealargerthan1cm
2
andatransvalvularpressuregradient
oflessthan50mmHg.
ModeratelyseverePSoccursifthevalveareais0.51.0cm
2
,withatransvalvularpressure
gradientbetween50and75mmHg.
SeverePSisdefinedbyavalveareasmallerthan0.5cm
2
andatransvalvularpressuregradient
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.

Diagnosis
Echocardiography.ColorflowDopplerechocardiographyisthemainstayforrecognizing
pulmonicregurgitation.
Treatment
1. Inprimarypulmonaryvalveregurgitationtheprognosisisverygood,rarelyiscorrectionofthe
defectnecessary.
2. Insecondarypulmonaryvalveregurgitationtheprognosisdependsonthecauseandisdirected
towardstheprimarydisease.
MCQ

1.Inaorticvalvedisease,markastrueorfalseX
a) Severeaorticstenosiswithleftventricularfailureisassociatedwithamediansurvivalofapprox.
2years
b) Mildaorticstenosisinyoungpeopleisassociatedwithanegligibleriskofendocarditis
c) Longtermtreatmentwithnifedipineinaorticregurgitationisassociatedwithimprovedoutcome
d) Systolicbloodpressuremaybenormalorelevatedinpatientswithsevereaorticstenosis

1. A72yearoldgentlemanisreferredtoyoubecauseofaprecordialsystolicmurmur.On
examination,thereisaharshmidsystoliccrescendodecescendomurmurattherightparasternal
ICS,radiatingtothecarotids.Thepatientisasymptomatic.Echocardiographyrevealsanaortic
valveareaof1.2cm
2
andameansystolicgradientof30mmHgandanormalLVsystolic
function.
Whatisthemostappropriateinterventionforthispatientatthepresenttime?
a.Percutaneousballoonaorticvalvuloplasty
b.Aorticvalvereplacement
c.Conserveandfollowup
3.Matchthefollowingvalvelesionswiththeirassociatedmurmur

a.Aorticstenosis1.decrescendodiastolicblowingmurmurheardbestalongleftsternalborder
b.Mitralregurgitation2.holosystolicmurmurattheapexradiatingtoleftaxilla
cAorticregurgitation3.crescendodecrescendosystolicmurmuralongtheleftsternalborder
d.Mitralvalveprolapse4.midorlatesystolicmurmurattheapexthatmaybeprecededbyaclick

4.Hillssignmeansthat
a. Brachialandpoplitealarterialbloodpressuresareequal
b. Brachialarterialbloodpressureishigherthanpoplitealpressure
c. Poplitealarterialbloodpressureishigherthanbrachialby30mmHg
d. Poplitealarterialpulsationsarenotpalpable

5Adiamondshapedmurmurrefersto
a. Crescendo
b. Decrescendo
c. Crescendodecrescendo
d. Noneoftheabove
6Murmurofaorticstenosisradiatesto
a. Axilla
b. Neck
c. Back
d. Rightshoulder
7Aorticstenosisisalwaysofrheumaticoriginwhenassociatedwith
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
10Onsetofsyncopeisassociatedwithanaveragesurvivalof
a. 1year
b. 3years
c. 5years
d. 8years
11TheclassicsymptomtriadofASincludesallofthefollowingexcept
a. Angina
b. Syncope
c. Cyanosis
d. Heartfailure
12Anejectionclickheardinapatientwithvalvularaorticstenosisimpliesanimmobileandcalcificvalve
a. True
b. False
13Thesimplestandmostfeasibletoolforassessmentofseverityofaorticstenosisis
a. Cardiaccatheterization
b. Magneticresonanceimaging
c. Electrocardiography
d. Dopplerechocardiography

14Apatientwithnewlydiscoveredaorticregurgitationandseverechestpainshouldbeconsideredtohave
a. Myocardialinfarction
b. Acutepericarditis
c. Aorticdissection
d. Syphyliticaortitis
15Acuteaorticregurgitationmaybecausedby
a. Bluntchesttrauma
b. Infectiveendocarditis
c. Aorticdissection
d. Alloftheabove
16Preoperativepredictorsofpoorpostoperativesurvivalinpatientswithsevereaorticregurgitationainclude
allofthefollowingexcept:
a. LVESDgreaterthan55mm
b. LVEFlessthan50%
c. NYHACHFclassIII,IV
d. Waterhammerpulse