Diagnosis of cardiac murmurs in children - Vessel Plus-

The author prefers to utilize the eponyms, RUSB, LUSB, LLSB, apex, and LMSB, instead of aortic area, pulmonary area, tricuspid area, mitral area, etc. The ... vp Search OAEJournals SearchinallOAEJournals Advanced VesselPlus Advanced HotKeywords cardiacsurgery coronaryarterybypassgrafting myocardialinfarction valveimplantation revascularization atherosclerosis lipid diabetesmellitus inflammation angiogenesis NavigationBar Menu vp Home About AbouttheJournal AimsandScope EditorialPolicies EditorialBoard JuniorEditorialBoard News Partners ContactUs ForAuthors AuthorInstructions ArticleProcessingCharges EditorialProcess ManuscriptTemplates SubmitaManuscript ForReviewers PeerReviewGuidelines Articles AllArticles ArticleswithVideoAbstracts SpecialIssues AllSpecialIssues OngoingSpecialIssues SpecialIssueEbooks SpecialIssueGuidelines Volumes Webinars AcceptedManuscripts Videos AbouttheJournal EditorialPolicies APCs Articles EditorialProcess AllSpecialIssues News ContactUs SignIn Submit Top Home  Articles  AllArticles  ArticleVesselPlus 2022;6:22.10.20517/2574-1209.2021.105©TheAuthor(s)2022.OpenAccessReviewDiagnosisofcardiacmurmursinchildrenViews: 552 | Downloads: 55 | Cited:  0P.SyamasundarRao DepartmentofPediatrics,UniversityofTexasatHoustonMcGovernMedicalSchoolandChildren’sMemorialHermannHospital,Houston,Texas,TX77030,USA.Correspondenceto:P.SyamasundarRao,MD,DepartmentofPediatrics,UniversityofTexasatHoustonMcGovernMedicalSchoolandChildren’sMemorialHermannHospital,6410FanninStreet,Suite#425,Houston,TX77030,USA.E-mail:P.Syamasundar.Rao@uth.tmc.eduThisarticlebelongstotheSpecialIssueInsightsintoCongenitalHeartDisease:DiagnosisandManagementViews:552 | Downloads:55 | Cited:0 | Comments:0 | :0Received:20Jul2021|FirstDecision:15Sep2021|Revised:27Sep2021|Accept:2Nov2021|Published:8Apr2022AcademicEditors:ChristopherLau,AlexanderD.Verin|CopyEditor:Yue-YueZhang|ProductionEditor:Yue-YueZhang©TheAuthor(s)2022.OpenAccessThisarticleislicensedunderaCreativeCommonsAttribution4.0InternationalLicense(https://creativecommons.org/licenses/by/4.0/),whichpermitsunrestricteduse,sharing,adaptation,distributionandreproductioninanymediumorformat,foranypurpose,evencommercially,aslongasyougiveappropriatecredittotheoriginalauthor(s)andthesource,providealinktotheCreativeCommonslicense,andindicateifchangesweremade.AbstractHeartmurmursarefrequentlyheard,andthemurmursaretheusualcauseforuncoveringheartdefectsinpediatricpatients.Themurmursaregroupedintosystolicmurmurs,diastolicmurmurs,andcontinuousmurmurs.Cautiousassessmentofthemurmurandassociatedabnormalitiesinphysicalexaminationarelikelytoproducecorrectdiagnosisofthecauseofthemurmur.Sometimesitmaybenecessarytoutilizenoninvasiveandinvasive(rarely)investigationstoachieveanaccuratediagnosis.Nonetheless,suchdiagnosticstudiesmayfrequentlyberequiredforquantification,andbeforeinterventioneitherbytranscathetermethodsorbysurgery. KeywordsEjectionsystolicmurmur,holosystolicmurmur,functionalmurmur,diastolicmurmurs,Graham-Steelmurmur,venoushumINTRODUCTIONOnemightaskwhyapaperonauscultationisincludedinajournalissuedealingwithadvancesandinsightsintocongenitalheartdisease.Whilesuchaquestionisjustified,theauthor’sresponseisthatsuchinclusioniswarrantedbecauseofitsvalueinitsabilitytodiscernintricatediagnosticdilemmas[1,2]priortoinvokingtheassistanceofsophisticatedinvestigativestudies,namely,echocardiography,magneticresonanceimaging(MRI),computedtomography(CT),andcardiaccatheterizationwithselectivecineangiography.Whiletheyoungergenerationofcardiologistsreadilyinvokestheadvantagesofechocardiography,MRI,andCT,theauthorseekstoemphasizethevalueofcarefullyperformedphysicalexamination,includingauscultationpriortoafore-mentionedinvestigativestudies,particularlyinchildren.Suchanassertion,however,isnotapplicableinneonatesbecause(1)severecyanoticcongenitalheartdefects(CHDs)canexistwithoutacardiacmurmur;(2)aloudmurmurdoesnotautomaticallysuggestthatthereasonforthedistressinthebabyisrelatedtoaheartdefect;and(3)whenamurmurisdetected,itisnotnecessarilycharacteristicofaspecifiedCHD,asemphasizedelsewhere[3,4].Therefore,thediscussioninthispaperismainlyfocusedonmurmurevaluationinchildrenandnotinneonates.Inthispaper,thecardiacmurmurisdefined,theprevalenceofthemurmursiscited,anapproachtoauscultationispresented,murmursareclassified,andthedifferentialdiagnosisofthecardiacmurmursisdetailed.DefinitionofcardiacmurmursCardiacmurmursaredefinedasabnormalsoundsorvibrationsthatoriginateintheheartand/orlargebloodvesselsandareusuallyauscultatedintheprecordiumand/orgreatvesselsites.Thesoundfrequencyofthevibrationsvariesfrom50to1000/s.Theword“murmur”isusedforabnormalvibrationsofalongerperiod,whereastheshorterdurationvibrationsaretermedsnapsorclicks.Amurmurauscultatedattheperipheralvascularsitesisnamed“bruit”;forexample,carotidbruitforasoundappreciatedoverthecarotidvesselsandabdominalbruitforasoundperceivedacrosstheabdominalaortaregion.Conversely,abruitheardovertheprecordiumiscalledamurmur.Thepatternofnormalbloodflowinthecardiovascularstructuresisassumedtobelaminar.Amurmurisbelievedtobeproducedonceturbulenceisformedsecondarytoanabnormalflowpattern[5].ItiscommonlythoughtthatanaudiblemurmurisheardwhentheReynoldsnumberisinexcessof2000[5]:Reynoldsnumber=rQD/uDmeansblooddensity,Qindicatesbloodflowvelocity(mean),rmeansconduit(tube)radius,anduindicatesviscosityoftheblood.PrevalenceofmurmursCardiacmurmuristhemostfrequentabnormalitybywhichheartdiseaseisdetected,especiallyinchildren(notneonates).Furthermore,intheauthor’spersonalexperience,murmuristhemostfrequentreasonforcallingforanappointmentforachildtoseeapediatriccardiologistforevaluationoftheheart.Onroutineauscultation,cardiacmurmursintheneonatesandprematureinfantsaresomewhatinfrequent(<2%).However,duringcarefulauscultation,heartmurmursmaybeappreciatedinexcessofhalfofthesebabies,andtheprevalencemaybeintheorderof80%innormalpretermbabies[5].Inotherwisenormalchildrenandteenagers,theprevalenceofcardiacmurmurvariesfrom30%to50%.However,mostofthesearefunctional,normal,orinnocentmurmursandshouldnotbeofanyconcern[5].Recentstudiesalsoindicatedthatthemajorityofthemurmursarefunctionalmurmurswhethertheexaminationisperformedforroutinescreeningorasapartofpre-sportsparticipationscreening[6,7].AnapproachtoauscultationItisessentialthatthepatientisquiet,andtheauscultationshouldtakeplaceinnoiselesssurroundingssoastoensureaccurateinterpretationofauscultatoryfindings.Inyoungbabiesandtoddlers,itiscriticaltolistentothechestfirstbeforetheinfantisdisturbedwiththeexaminationoftheliver,palpationofthefemoralpulses,orevenprecordium.Ofcourse,earandthroatexaminationshouldnotbeperformedpriortoauscultation.Younginfantsarebetterexaminedinitiallyonthemother’slap.Atthestart,the1stand2ndheartsounds,clicks,andsnapsshouldbeassessedbeforeaddressingthemurmur[5,8,9].Theauscultationshouldbeperformedoverthefourclassicsitesofauscultationsuchas:(1)apexormitralarea;(2)rightuppersternalborder(RUSB)oraorticarea;(3)leftuppersternalborder(LUSB)orpulmonaryarea;and(4)leftlowersternalborder(LLSB)ortricuspidarea.Theleftmid-sternalborder(LMSB)andasitemidwaybetweentheapexandLLSBshouldalsobeutilizedforauscultation.Otherareasasdeemedappropriateforagivenclinicalscenarioshouldalsobeusedforauscultation.Theauthorpreferstoutilizetheeponyms,RUSB,LUSB,LLSB,apex,andLMSB,insteadofaorticarea,pulmonaryarea,tricuspidarea,mitralarea,etc.Theauscultationshouldbeperformedinbothuprightandsupinepositions.Boththebellanddiaphragmofthestethoscopeshouldbeusedforauscultation.Afterthemurmurisdiscovered,initiallydeterminethetimingofoccurrenceofthemurmur,namely,systolic,diastolic,orcontinuous.Additionalmurmurtimingofthediastolicmurmurstoearly,mid,andlateisuseful(seetheclassificationofthemurmursbelow).Next,thepointofgreatestloudnessofthemurmurisappraised,ifnecessary,bymovingtheheadofthestethoscopeinchbyinch.Anyparticularradiationcharacteristicsofthemurmurarethendetermined.Theexamplesare:radiationintotheaxillaofanapicalholosystolicmurmurofmitralregurgitation(MR)andradiationfromRUSBintotheneckvesselsofanejectionsystolicmurmurofaorticstenosis(AS).Bydefinition,radiationofmurmurmeansthatthemurmurisofequalintensitybothatitsoriginandwhereitradiatesto.TheloudnessofthemurmurintogradesIthroughVI(Levine[8])shouldbeappraisedsothatacomparisonbetweendifferentobserversduringthesamevisitorbetweenexaminationsbythesameobserveratdifferentvisits[Table1]canbemade.Whilegradingofthemurmurisroutinelyperformedbymostcardiologists,thegradeofthemurmurdoesnotindicateagivendiagnosisorseverityofthecardiacproblem.Table1GradingofthemurmursGradeI-NotimmediatelyheardGradeII-Soft,butimmediatelyheardGradeIII-Loud,butnothrillGradeIV-AssociatedwithathrillGradeV-HeardwiththeedgeofthetiltedstethoscopeGradeVI-HeardwiththestethoscopeliftedawayfromthechestwallDescriptionssuchas“blowing”andothersarenothelpfulinmakingadiagnosisbecausesuchdescriptivequalitieshavebeenusedtocharacterizeseveralheartdefects.However,thedepictionofthemurmur’sshapeisofsomevalue.Descriptions,namely,crescendo,decrescendo,flat,maybeusedtocharacterizethemurmur.Assessmentofthepitchofthemurmurisalsohelpful,andmurmur’spitchmaybelow,medium,orhigh.Theintensityofthemurmurmayvarywiththerespiration,andifsuchispresent,itshouldbedocumented.Asarule,themurmursarisingfromtherightsideoftheheart,forinstance,tricuspidregurgitation(TR),doalterwiththerespiratorycycle,whereasthemurmursfromdisturbancesoftheleftheartstructureswillnotexhibitachangewithrespiration.Otheruniquemurmurcharacteristics,forexample,multipleclickswithinthepatentductusarteriosus(PDA)murmurandmusicalorvibratorynatureoftheinnocentorfunctionalmurmur,arealsorecorded.Recentreviews[7,10]onauscultationsuggestedthatmasteryofskillsofauscultationacquiredbytrainingandexperienceisnecessarytodifferentiatefunctionalfrompathologicmurmurs,andIagreewiththisassessment.Simulation-guidedcardiacauscultationviaHarvey(©)mannequinorothercardiacpatientsimulatorsandmurmuronlinelearningexperiencehavebeenusedtoteachmedicalstudentsandresidents[11-14],andsuchtrainingmethodshavedemonstratedimprovementoftheoverallperformanceofthesetraineesinidentifyingthemurmurs.Whilethesemethodsareincreasinglyusedandmaybepracticalinthecurrentera,theauthorbelievesbedsideacquisitionofauscultatoryskillsunderthesupervisionofanexperiencedauscultatorisimportant,andsimulationsandonlinematerialsuchastheseandothersshouldserveassupplementarytools.CLASSIFICATIONOFTHEMURMURSTheheartmurmursareclassifiedinto:(1)systolic;(2)diastolic;and(3)continuousmurmurs.Typicalfeaturesofeachofthesemurmurs,causesofthemurmurs,anddifferentialdiagnosisofeachmurmurwillbereviewedonebyone.SystolicmurmursMurmursthatarelocatedinbetweenthe1standthe2ndheartsoundsarecharacterizedassystolicmurmurs.Discussionofthemurmursinsystoleisconfinedtosubjectswhoarenotcyanotic.Theapproachusedinthediagnosisofcyanoticinfantsandchildrenisbyexaminingthemagnitudeofpulmonarybloodflowonachestroentgenogram[3,4]andisnotdiscussedinthisscript.Systolicmurmursaresub-classifiedinto:(1)Ejectionsystolicmurmurs;and(2)holosystolicmurmurs.Ejectionsystolicmurmursbeginjustfollowingthe1stheartsoundandendjustpriortotheonsetofthe2ndsound.Thesemurmursaretypicallycrescendo-decrescendoinnatureandhaveadiamondshape(topsectionofFigure1).Thepeakingofthemurmurmayoccurintheearly,mid,orlateportionsofthesystole.Bydefinition,theholosystolicmurmurbeginswithandobscuresthe1stsoundandlaststhroughtheentiresystole(bottomofFigure1).Forpracticalpurposes,theauthorattachesahighervaluetotheinitialpartofthedefinitionbutdoesnotneedthesubsequentparttofulfillthecriteriatomakeadiagnosisofaholosystolicmurmur.Indeed,ifanauscultatorcandistinctlyappreciatethefirstsoundseparatelyfromthemurmuratthesiteofmaximalintensityofthemurmur,itmaybedesignatedasanejectionmurmur.Ontheotherhand,ifthefirstsoundandthemurmurcannotbeauscultatedasseparateentitiesatthesiteofmaximalloudnessofthemurmur,itisdesignatedasaholosystolicmurmur.Itisexceedinglyimportantthatsuchadistinctionisundertakensincethedifferentialdiagnosisofthesetwomurmursisdiverseandwithnosignificantoverlap,asillustratedin[Table2]and[Table3].Ejectionandholosystolicmurmurswillbeseparatelydiscussed.Figure1.Artist’srenditionofauscultatoryfindingsofsystolicmurmurs.Ejectionmurmur(upperpart)beginsjustfollowingthefirstsound(S1)andstopsjustpriortothesecondsound(A2indicatesaorticcomponent;P2indicatespulmonarycomponent)whileaholosystolicmurmur(lowerpart)startswithandconcealstheS1andmaylastthroughtheentiresystole(similartothatshowninthelowerpartofthediagram)ormaystoppriortoA2.ModifiedfromRao[5].Table2Etiologyofejectionsystolicmurmurs.ReproducedfromRao[5]CommoncausesAorticstenosisCoarctationoftheaortaPulmonarystenosisAtrialseptaldefectFunctionalorinnocentmurmurLesscommoncausesMitralprolapsesyndromeAcyanotictetralogyofFallotPatentductusarteriosusTable3Etiologyofholosystolicmurmurs.ModifiedfromRao[5]VentricularseptaldefectMitralregurgitationTricuspidregurgitationEjectionsystolicmurmursTheetiologyofejectionsystolicmurmursisshownin[Table2].Thesemurmursareclassifiedintofrequentandlessfrequentetiologies.Thecauseoftheejectionsystolicmurmurmaybeascertainedbyexaminingwherethemurmurisheardbestandhowitradiatesintoothersites;characteristicsofthemurmur,ifany;character(split,notsplit,widelysplitandfixed)plusintensityofthe2ndheartsound;presenceofsystolicclicks;intensityofimpulsesintheprecordium;andabnormalfemoralarterialpulses[Table4].Thefindingsoftheelectrocardiogram(ECG)andchestroentgenogramareusefulinarrivingatthediagnosis,andechocardiographicstudieshelptoconfirmthediagnosis.Table4DifferentialdiagnosisofejectionsystolicmurmursPointofmaximalintensityofthemurmurRadiationofthemurmurPrecordialimpulsesThrillFemoralpulses2ndheartsoundEjectionsystolicclickChestX-rayECGEcho-DopplerOtherfeaturesAorticstenosisRUSBLMSBCarotidarteriesNormalorincreasedLVimpulseRUSB&suprasternalnotchNormalNormalConstantclickatapex,LMSB&RUSBDilatedascendingaortaNormalorLVHThickenedbicuspidaorticvalveleaflets,increasedDopplerflowvelocityacrosstheaorticvalveSeverityofaorticstenosisisdifficulttojudgebyclinicalexaminationCoarctationoftheaortaRUSBCarotidarteriesNormalorincreasedLVimpulseSuprasternalnotchDecreased&delayedorabsentNormalConstantclickatapex,LMSB&RUSBInverted3signonbarium-filledesophagus,ribnotchingNormalorleftventricularhypertrophy(LVH)Suprasternalnotch2Dechoshowscoarctation,increasedflowvelocityindescendingaortaMeasurementofbloodpressureinarmsandlegsishelpfulPulmonarystenosisLUSBInfra-clavicularregions&backNormalorincreasedRVimpulseLSUB&suprasternalnotchNormalNormal,diminished,orabsentLUSBLMSBLLSB,varieswithrespirationDilatedmainpulmonaryarteryNormalorRVHRVenlargement,increasedDopplerflowvelocityacrossthepulmonaryvalveDuration&timingofpeakingofthemurmur,degreeofsplitting&intensityof2ndsoundmaysuggestseverityofstenosisAtrialseptaldefectLUSBNoneHyper-dynamicRVimpulseNoneNormalWidelysplitandfixedNoneProminentmainpulmonaryartery,increasedpulmonarybloodflowMildRVHEnlargedRV,paradoxicalseptalmotion,atrialdefectonsubcostalecho-DopplerMid-diastolicmurmuratLLSBFunctionalorinnocentmurmurBetweenapex&LLSBoratLUSBNoneNormalNoneNormalNormalNoneNormalNormalNormalVibratoryormusicalqualitytothemurmurECG:Electrocardiogram;RUSB:rightuppersternalborder;LMSB:leftmid-sternalborder;LLSB:leftlowersternalborder;LUSB:leftuppersternalborder;LV:leftventricular;RV:rightventricle;RVH:rightventricularhypertrophy.AorticstenosisTheejectionsystolicmurmurofASisappreciatedbestattheupperrightsternalborder(maybebetterheardatmidleftsternalborderinneonatesandyoungchildren).Themurmurtypicallytransmitswellintoboththecarotidvessels.Theleftventricular(LV)impulseisincreasedinmoderatetosevereAS.Athrillispalpatedattheupperrightsternalborderand/orinthesuprasternalnotch.SincemostAScasesareattheaorticvalvelevel,asystolicejectionclickisusuallyheardimmediatelybeforethemurmur.Thisejectionsystolicclickisauscultatedattheupperrightandmidleftsternalbordersandapex.Theclickisconstantanddoesnotvarywiththerespiratorycycle.Anormalsecondsoundisusuallyheard.Thearterialpulsesaretypicallynormal.TheexceptionsaresubjectswithverysevereAS.TheECGmaybenormalormaydemonstrateLVhypertrophy.Unfortunately,neitheranormalECGorabnormalECGfindingsareindicativeoftheseverityofAS.Nevertheless,invertedTwavesinleadsV5andV6suggestsevereAS.Theheartsizeisusuallynormalonachestroentgenogram,butmaycommonlydiscloseanenlargedascendingaorta,secondarytopost-stenoticdilatation.Two-dimensional(2D)echocardiogrammaydemonstratethickenedleafletsoftheaorticvalvewithdoming.TheLVfractionalshorteningmaybeincreased,largelyproportionaltotheseverityofAS.ThemagnitudeofDopplerflowvelocityacrosstheaorticvalveindicatestheseverityofobstruction.ThepeakinstantaneouspressuregradientacrosstheaorticvalveiscalculatedbyusingamodifiedBernoulliequation:ΔP=4V2Where,VisthepeakDopplervelocityacrosstheaorticvalveinmeters/sec,andΔPispeakinstantaneouspressuregradientinmmHg.PatientswithbicuspidaorticvalveswithoutsignificantASmayalsohaveanejectionsystolicclickandanejectionsystolicmurmur.Itshouldbenotedthatbicuspidaorticvalvemaynotexhibitanaudibleclickininfancy.Attimes,bicuspidaorticvalveisindistinguishablefrommildASbyphysicalexamination.OtherLVoutflowtractobstructionsEjectionsystolicmurmursarealsoheardinsubvalvarmembranousAS,hypertrophicobstructivecardiomyopathy(HCM),andsupravalvarAS.However,ejectionsystolicclicksarenotpresentinthesediseaseentitiesand,therefore,canbedifferentiatedfromvalvarAS.Inaddition,thesubjectswithsupravalvaraorticstenosismayhavedistinctivefeaturesofWilliamssyndromesuchastrigonalfaces,developmentaldelay,andinfantilehypercalcemia.Furthermore,someofthesepatientsmayhaveapulseorbloodpressuredifferencebetweenbotharms.ThemurmurofHCMmaybebetterauscultatedatleftmidsternalborderandmayincreaseinintensitywithValsalva.Inpatientswithsubaorticmembrane,noclicksarepresent,andthemurmurmaybeappreciatedbetteratLLSBorLMSBs.However,2DechostudiesareusefulindefiningthesiteofLVoutflowtractobstruction.TheseverityofobstructioncanbeestimatedbyDopplerinterrogationoftheLVoutflowtractandsupravalvaraorticregion.CoarctationoftheaortaSimultaneouspalpationoffemoralandbrachialpulseswillleadtothediagnosisofcoarctationoftheaorta,althoughthemurmuristhepresentingcomplaint.Theejectionsystolicmurmurrelatedtoaorticcoarctationmaybeauscultatedattherightuppersternalborderandisprobablycausedbyflowdisturbanceacrosstheaorticvalve.Themurmurofflowacrossaorticcoarctationmaybeauscultatedbestintheleftinter-scapularregionovertheback.TheLVimpulsemaybeprominent.Athrillisfrequentlyfeltinthesuprasternalnotch.Becausebicuspidaorticvalveisseeninahighpercentage,upto60%,ofcoarctationoftheaortapatients,asystolicejectionclickmaybeappreciatedattheapexandupperrightandmidleftsternalborders.Theseaorticclicksdonotvarywiththerespiratorycycle.The2ndsoundisnormalinmostpatientswithaorticcoarctation.Asmentionedabove,thebrachialandfemoralarterypulsesshouldbepalpatedsimultaneously,andsuchexaminationdemonstratesdelayedand/ordecreasedfemoralarterialpulses.Thefemoralarterypulsesmayevenbeabsent.Measurementofbloodpressures(BPs)inboththearmsandonelegisrecommended.Asystolicbloodpressuredifference≥20mmHgbetweenthearmsandlegsissuggestiveofcoarctationoftheaorta.Inpatientswhohavetheleftsubclavianarteryveryclosetothecoarctationmayhavediminishedleftbrachialpulse.Insubjectswhoserightsubclavianarteryhasanomalousorigin,belowthelevelofaorticcoarctation,willresultindecreasedorabsentrightbrachialpulse.Consequently,werecommendmeasurementofBPsinbotharmsaswellaspalpationofbothbrachialpulsestodiscernanydifference.TheECGmaydemonstrateLVhypertrophy,oritmaybenormal.ChestX-rayabnormalitiesare:(1)a“3”signinahighlypenetratedchestfilm;(2)inverted“3”signoftheesophagealbariumstudy;and(3)rib-notching(notininfants).2Dechocardiogramsecuredfromthetransducerplacedinthesuprasternalnotchusuallyshowsaorticcoarctation.DopplerinterrogationwilldemonstrateincreasedDopplerflowvelocity;thepressuregradientacrosscoarctationmaybecalculatedfromtheflowvelocitymagnitudeinthedescendingaorta(ΔP=4V2).Associationofventricularseptaldefect(VSD),PDA,AS,andmitralvalveabnormalitiesinpatientswithcoarctationoftheaortaiswellknown,particularlyintheneonateandyounginfant,andtherefore,theecho-Dopplerstudiesshouldscrutinizeforsuchdefects.PulmonarystenosisTheejectionsystolicmurmurofpulmonarystenosis(PS)isbestauscultatedatupperleftsternalborder.Themurmurtransmits(radiates)intotheinfraclavicularareas,axillaryregions,andback.Theimpulsesoftherightventricle(RV)andRVoutflowtractareprominent.Athrillisappreciatedattheupperleftsternalborderand/orinthesuprasternalnotch.SincemostPSpatientshavestenosisatthevalve,anejectionclickusuallyisheardandprecedestheejectionmurmur[Figure2].Theejectionclickisauscultatedatlowerleft,midleft,andupperleftsternalborders.Theclickchangeswiththerespiratorycycle(decreasesorbecomesabsentwithinspiration).ThelocationoftheclickcanhelpdifferentiateASfromPS.Thefeaturesofthe2ndsoundvarywiththemagnitudeofobstruction[Figure2].TheBPandpulsesareusuallynormal.Figure2.Invalvarpulmonarystenosis,thedegreeofpulmonaryvalveobstructionmaybepredictedbyauscultatoryfindings.Inmildobstruction(top),theejectionclick(EC)isevidentlyseparatedfromthefirstheartsound(S1),themurmurbeginswiththeclick,peaksinearlysystole,andstopswaybeforetheaorticcomponentofthesecondheartsound(A2),andthepulmonarycomponentofthesecondheartsound(P2)isnormaltoincreasedinintensity.Inmoderatepulmonarystenosis(middle),theclickisclosertothefirstheartsound,theejectionmurmurpeakslaterinthesystoleandthemurmurreachestheA2,andthesecondheartsoundiswidelysplitwithasoftpulmonarycomponent.Inseverevalvarstenosis(bottom),theclickiseithernotpresentoroccurssoclosetoS1thatitcannotbeauscultatedseparately.ThemurmurpeakslateinsystoleandextendsbeyondtheA2.ThesecondheartsoundiswidelysplitwithanextremelysoftorinaudibleP2.ModifiedfromRao[5].Therelationshipofclickwiththe1stheartsound,thedegreeofsplittingofthe2ndsound,theloudnessofthepulmonarycomponentofthe2ndheartsound,andthelengthofandtimingofpeakingofthesystolicmurmuraregenerallyindicativeofthedegreeofPS[Figure2].InmildPScases,theejectionclickisevidentlydistinctfromthe1stsound,the2ndheartsoundisnormallysplitwithnormaltoaslightlyincreasedpulmonarycomponentofthe2ndsound,andanejectionsystolicmurmurwhichpeaksearlyinsystoleandterminatesclearlypriortotheaorticclosureofthe2ndheartsound(Figure2;top).ThefindingsinmoderatePSareanejectionsystolicclickthatiscloserto1stheartsoundthaninmildPS,widesplittingofthe2ndsoundwithareduced(softer)pulmonarycomponentofthe2ndsound,andanejectionsystolicmurmurwhichpeaksinmidtolatesystoleandterminatesjustpriortotheaorticcomponentofthe2ndheartsound(Figure2;middle).ThecharacteristicsofseverevalvarPS(Figure2;bottom)areanejectionsystolicclickwhichiseitherabsentoroccurssoclosetothe1stsoundthatitisnotseparablefromit,noticeablyincreasedsplitofthe2ndheartsoundwitheitheraninaudibleorasoftpulmonarycomponent,andaprolongedejectionsystolicmurmurwhichpeakslateinsystoleandextendswaybeyondtheaorticclosureofthe2ndsoundsothatthelattercannotbeauscultated[15].TheloudnessofthesystolicmurmurdoesnotpointtotheseverityofPS,butinstead,thelengthofandtimingofpeakingofthemurmuraredeterminantsofseverityofPS.Thelongerthemurmurandthelateritpeaks,themoresevereisthePS.Likewise,theshortertheintervalbetweenthe1stheartsoundandtheejectionclick,thewiderthesplitofsecondsound,andsofterthepulmonarycomponent,themoresevereisthedegreeofPS[15].ModerateandseverePScasesareassociatedwithaprecordialthrillinpatientsolderthanthreetofourmonths.Usually,inPS,theECGdemonstratesrightventricularhypertrophy(RVH).TheseverityofRVHparallelsthemagnitudeofPS.Inmostcases,thechestroentgenogramshowsnocardiacenlargement,althoughadilatedmainpulmonaryartery(PA),post-stenoticdilatation,isfrequentlyseen.ThedegreeofPAdilatationhasnorelationshipwiththeseverityofPS.Echo-DopplerstudiesdemonstrateenlargementoftheRV,thickenedanddomedpulmonaryvalveleaflets,andincreasedDopplerflowvelocityacrossthepulmonaryvalve.DopplerflowvelocitymagnitudeacrossthepulmonaryvalveindicatestheseverityofPS.ThepeakinstantaneouspressuregradientacrossthepulmonaryvalvemaybecalculatedutilizingamodifiedBernoulliequation:ΔP=4V2Where,Visthepeakvelocityacrossthepulmonaryvalveinmeters/sec,andΔPispeakinstantaneouspressuregradientinmmHg.OtherRVoutflowtractobstructionsEjectionsystolicmurmursauscultatedbestatLUSBarealsopresentinpatientswhohaveinfundibularPS,supravalvarPAstenosis,branchPAstenosis,andidiopathicenlargementofthemainPA.Similarly,functionalsystolicmurmursofpulmonaryejectiontypearealsoheardbestatLUSB.TheseentitiesshouldbedifferentiatedfromvalvarPS.ThecharacteristicejectionsystolicclickofvalvarPSisnotpresentinalltheabove,withtheexceptionofidiopathicdilatationofthemainPA.Furthermore,themurmurofinfundibularPSisusuallybestauscultatedatthelowerleftandmidleftsternalborders.InperipheralPAstenosis,themurmuristransmittedtobothaxillaeandback.Onsomeoccasions,themurmurisofgreaterintensityintheaxillaryregionandbackthanattheleftuppersternalborder.AuscultatoryfindingsandchestX-rayfeaturesofidiopathicdilatationofthemainPAaredifficulttodistinguishfromthoseofmildvalvarPS.However,echo-Dopplerexaminationisveryhelpfulindistinguishingalltheaboveentities.AtrialseptaldefectInpatientswithatrialseptaldefect(ASD),thesystolicmurmurissoftandisusuallyofgradeIItoIII/VIintensity,andisheardbestatLUSB.Theejectionmurmurisduetoincreasedbloodflowacrossthepulmonaryvalve.TheRVandRVoutflowtractimpulsesarehyper-dynamicandincreasedsecondarytomarkedlyincreasedflowacrosstherightheartstructures.ThrillsareunusualinASDs.Onoccasion,pulmonaryejectionclickisauscultated.The2ndsoundissplitwidelywithoutanyvariation(fixed),andthisfindingisconsideredtobeadistinctivefeatureofASD.Amid-diastolicflowmurmurofgradeItoII/VIisauscultatedbestattheLLSBwiththestethoscope’sbell.Thismid-diastolicmurmurissecondarytoalargeamountofbloodflowacrossthetricuspidvalve.Patientslessthansixmonthsofagemaynotexhibitallthefeaturesdescribedabove.TheBPandpulsesarewithinthenormalrange.TheECGdemonstratesmildRVHwithrSR’patternintheleadsV4RandV1.Thishasbeendescribedasdiastolicvolumeoverloadpattern.Mildtomoderateenlargementoftheheartmaybeseenonthechestroentgenogram.Increaseinpulmonaryvascularmarkingsanddilatedmainpulmonaryarteryarealsoseen.Echo-DopplerstudiesrevealRVenlargement;somepatientsexhibitparadoxicalinter-ventricularseptalmotion.TheASDcanbeclearlydemonstratedin2Decho,andtheshuntacrosstheASDmaybeseenwithpulsedandcolorDopplerimaging.SubcostalviewsarebesttodemonstratethesizeoftheASDandtheseptalrims.OtheratriallevelshuntsSinusvenosusandcoronarysinustypesofASDshavefindingssimilartothoseofostiumsecundumASDinregardtohistory,physicalexamination,ECG,andchestroentgenogram.However,inalargeproportionofpatientswithsinusvenosusASD,thevectorofthePwaveisorientedsuperiorlysothatthePwaveaxisisbetween0°and-90°(leftaxisdeviationofthePwave).2DechodemonstratesanASDinthesuperiorportionoftheinter-atrialseptumorlowintheatrialseptum,suggestingsinusvenousASD;thisincontradistinctiontosecundumASDinwhichthedefectissituatedinthemidinter-atrialseptum.IncoronarysinusASDs,thedefectislocatedclosetothecoronarysinus.OtherdefectswithfeaturessimilartothoseofsecundumASDareostiumprimumASD,commonatrium,andpartialanomalouspulmonaryvenousconnection(PAPVC).PatientswithostiumprimumASDandcommonatriumexhibitanapicalholosystolicmurmurofmitralinsufficiencysecondarytocleftmitralvalve.Theyalsohaveadistinctiveleftaxisdeviationwithfrontalplaneaxisof-30°to-90°(abnormallysuperiorORSvector).2DechowilldemonstrateanASDintheinferiorportionoftheatrialseptumintheprimumASD,whereasthewholeatrialseptumisdeficientinsubjectswithcommonatrium.Themitralcleftandmitralinsufficiencycanalsobeshownintheecho.PAPVCisdifficulttodifferentiatefromsecundumASD.PAPVCwithintactatrialseptumwillexhibitwidebutvariablesplittingofS2.However,carefulecho-Dopplerstudiesmayidentifythepartialveins.Cardiaccatheterizationandselectivecine-angiographymaybeneededinrarecases.FunctionalorinnocentmurmursFunctional,normal,orinnocentmurmursarefrequentlyheardonroutineauscultationofchildren.Twosuchejectionsystolicmurmursareprecordialvibratorymurmurandpulmonaryejectionmurmur.TheprecordialvibratorymurmurisalsocalledStill’smurmur.Thismurmurisbestauscultatedwiththestethoscope’sbell.Itistypicallybestauscultatedatasiteinbetweentheapexandlowerleftsternalborderanddoesnotusuallytransmittoothersites.Rarely,themurmurmaybeappreciatedwidelyovertheentireprecordialarea.TheloudnessofthemurmurisbetweengradesIandIII/VI.Characteristically,thisStill’smurmurhasmusicalqualityandvibratorycharacter,andisunifrequent.Whensuchaqualityofthemurmurisappreciated,onecanbecertainthatthemurmurisfunctional.ThevibratoryinnocentmurmurwilldiminishwithaValsalvamaneuver.Otherfindingsincludenormalcardiacimpulses,normalcardiacsounds,andnormalBPandpulses.Likewise,thechestX-rayandECGarenormal.Echocardiogramisnotnecessary,butifperformed,itisnormal.Hence,thediagnosisofStill’smurmurisbasedonauscultatoryqualitiesofthemurmurdescribedaboveaswellasotherwisenormalcardiovascularevaluation.ThepulmonaryejectionmurmurisbestauscultatedattheLUSBandheardbetterwiththestethoscope’sdiaphragm.TheloudnessofthemurmurvariesbetweengradesIandIII/VI.Thepulmonaryejectionmurmurhasahigherpitchthantheprecordialvibratorymurmur.Thismurmurismorenotablyappreciatedinpersonswhoareofthinbuildandinpatientsthathave“straightbacksyndrome”.Otherfindingsincardiovascularexamination,namely,precordialimpulses,heartsounds,BP,andbrachialandfemoralpulses,arewithinnormallimits.ChestX-ray,ECG,andechocardiographicstudiesarealsonormal.Someinvestigatorshaveutilizedcomputer-assistedauscultation[16],piezoelectricsensors[17],andartificialintelligence[18]todistinguishfunctionalmurmursfrompathologicmurmurs.Mostexperiencedcardiologists,includingtheauthor,donotroutinelyperformechocardiogramsinpatientswiththedefinitiveclinicaldiagnosisoffunctionalorinnocentmurmurs.However,arecentstudy[19]foundcardiacabnormalitiesinthreeoutof62(4.8%)patients.Inaddition,thisstudyindicatedthatthereferringphysician’sexpectationisthatanechocardiogramisperformedaspartofcardiacevaluation.Whiletheauthorcontinuestobelievethatechocardiogramisnotnecessaryforpatientswithaclear-cutdiagnosisoffunctionalorinnocentmurmurs,anechomaybeperformedifrequestedbytheparentsorreferringphysician.Otheretiologiesofejectionsystolicmurmurs[Table2]“Ejection”typeofmurmursmayalsobeheardinpatientswithmitralvalveprolapse.Thisdiamond-shaped,crescendo-decrescendomurmurisauscultatedattheapex,usuallylateinsystole.Amid-systolicclickprecedesthemurmur.Echocardiogramishelpfulindocumentingmitralprolapse.PatientswithadiagnosisofacyanotictetralogyofFallotmayhaveanejectionsystolicmurmurduetoflowacrossthestenosedinfundibulum.ThismurmurisusuallyheardatLMSBandLUSB,peaksearlyinsystolewhencomparedwithPSwiththeintactventricularseptum.Pinktetralogyshouldbeconsideredwhentheexaminerhearsamurmurallalongtheleftsternalborder.TheRVimpulseisincreased,andthe2ndheartsoundissingle.ECGdemonstratesRVH.Echo-DopplerstudiesdemonstratealargeVSD,alargeascendingaortathatoverridestheinter-ventricularseptum,RVH,andDopplerevidenceofRVoutflowtractobstruction.SomesubjectswithPDAmayexhibitejectionsystolicmurmur.TheseexamplesareeitherneonatesorolderpatientswithelevatedPApressuresandresistance.ThediastolicpartofthePDAmurmurisnotpresentbecauseofincreasedpulmonaryvascularresistance.Multipleejectionclicksburiedwithinthemurmur,andboundingpulsesareusefulinthediagnosisofPDA.Echo-Dopplerstudiesconfirmthediagnosis.HolosystolicmurmursTheetiologyofholosystolicmurmursisshownin[Table3].Locationwherethemurmurisheardbest,whereitradiatesto,andchangeinintensityofthemurmurwithrespiratorycycleareusefulinthedifferentialdiagnosisofthesedefects[Table5].Ofcourse,thefindingsoftheECGandchestroentgenogramareusefulinmakingadiagnosis.Echo-Dopplerstudiesareconfirmatory.Table5DifferentialdiagnosisofholosytolicmurmursPointofmaximalintensityofthemurmurRadiationofthemurmurRespiratoryvariationofthemurmurVentricularimpulsesOtherclinicalfindingsChestX-rayECGEchoDopplerVentricularseptaldefectLLSBNoradiationDoesnotchangeIncreasedLVand/orRVimpulseMurmurmaybewidelyheardovertheprecordium.Mid-diastolicmurmuratapexsuggestsalargeshuntacrosstheVSDCardiomegaly,increasedpulmonaryvascularmarkingsLVH,biventricularhypertrophy(BVH)orRVHVSDcanbeimagedby2Decho.DopplerflowvelocityacrosstheVSDishelpfulinassessingthesizeofVSDMitralregurgitationApexRadiationtoanteriorormid-axillarylineDoesnotchangeIncreasedLVimpulseMid-diastolicmurmuratapexsuggestsmoderatetoseveremitralinsufficiencyCardiomegaly,leftatrialenlargement(LAE),normalpulmonaryvascularmarkingsLAE,LVHColorDopplerevidenceformitralinsufficiencyTricuspidregurgitationLLSBNoradiationIncreaseswithinspirationIncreasedRVimpulseMurmursounds“superficial”.ProminentV-wavesinjugularveins,prominentsystolichepaticpulsationsCardiomegaly,largerightatriumRVH,rightatrialenlargement(RAE)ColorDopplerevidencefortricuspidinsufficiencyECG:Electrocardiogram;LLSB:leftlowersternalborder;LV:leftventricular;RV:rightventricle;RVH:rightventricularhypertrophy;VSD:ventricularseptaldefect.VentricularseptaldefectHolosystolicmurmursofVSDareauscultatedbestatthelowerleftsternalborder;thesemurmursdonotradiate.However,themurmurmaybeauscultatedwidelyacrosstheentireprecordialareainsomepatients.Thismurmurdoesnotexhibitrespiratoryvariation.ThismurmurisproducedbybloodflowacrosstheVSDduringsystole.Insubjectswithextremelysmallventriculardefects,themurmurmaybeauscultatedbestatthemidleftandrarelyatupperleft[2]sternalborders.ThemurmurusuallyvariesbetweengradesIIandV/VIinintensity.TheloudnessofmurmurhasnorelationshipwiththediameteroftheVSD.InpatientswhohavesmallVSDs,themurmurbeginswiththe1stheartsound,butdoesnotlastthroughthewholesystole.Thebrieferthemurmur,thesmallertheVSD.SmallmuscularVSDmayhavea“squirty”or“bicyclepump”qualityofmurmur.IncreasedLVimpulsemaybepresentinmoderateandlargeVSDs.IncreaseinbothrightandleftventricularimpulsesmaybefeltinpatientswithlargeVSD.OnlyincreaseinRVimpulsesisseeninpatientswithhighPApressuresandthosewhohavedevelopedpulmonaryvascularobstructivedisease(PVOD).Athrillmaybeappreciatedatthelowerleftand/ormidleftsternalborders.Splittingofthe2ndsoundisheard,althoughitmaybesingleifthereisPVOD.Thesecond(pulmonary)componentof2ndheartsoundmaybenormalinsmallandmoderate-sizedVSDs,butisloudinsubjectswithlargedefectswithelevatedpulmonaryarterypressure.ClicksareuncommoninVSDpatients;however,theyhavebeendescribedinsubjectswithspontaneousclosureofVSDsbyaneurysmalformationofthemembranousventricularseptum.Amid-diastolicflowrumbleofgradeItoII/VIintensityisauscultatedslightlyinternaltotheapexinsubjectswithincreasedleft-to-rightshuntswithpulmonarytosystemic(Qp:Qs)flowratio≥2:1(mediumandlargeVSDs).Thismurmurisauscultatedbestwiththestethoscope’sbell.Thismid-diastolicflowrumbleissecondarytoaugmentedbloodflowviathemitralvalve.TheBPandperipheralpulsesareusuallynormal.TheECGshowsnoabnormalitiesinsmallVSDs.EvidenceformildLVhypertrophyisseeninmoderate-sizedVSDs.BiventricularhypertrophyhasseenlargeVSDs.RVHispresentinlargedefectswithhighpulmonaryarterypressuresandthosewhohavedevelopedPVOD.Chestroentgenogramusuallyshowsanenlargementoftheheartandincreasedpulmonarybloodflow;themagnitudeofsuchabnormalitiesisproportionatetothediameteroftheVSD.M-modeechocardiogramdemonstratestheincreasedsizeoftheleftatrium(LA)andLV;thesechangesareagainareproportionaltothesizeoftheVSD[20].ThepositionoftheVSDintheventricularseptumandthesizeoftheVSDcanbeimagedby2Dechocardiography.Left-to-rightshuntingacrosstheVSDcanbeshownoncolorDopplerimaging.DopplerflowvelocitymagnitudesacrosstheVSDareusefulinestimatingthesizeoftheVSDandthePApressure.MitralregurgitationTheholosystolicmurmurofMRisbestauscultatedattheapicalregion.Themurmurtransmitswellintotheanteriorormid-axillarysites.Theloudnessofthemurmurdoesnotvarywithrespiration.TheloudnessofthemurmurvariesbetweengradesIIandIV/VI.TheLVimpulseisprominentandhyper-dynamic.ThedegreeofprominenceoftheLVimpulseisrelatedtothemagnitudeoftheMR.Asystolicthrillmaybefeltattheapicalregion.The2ndsoundissplitinanormalfashion.Thepulmonarycomponentofthe2ndsoundisusuallynormal,althoughitmaybeloudinpatientswithelevatedPApressuresorincreasedpulmonaryvascularresistance.Amoderatelyloud3rdsoundisauscultatedifMRismoderatetosevereindegree.AgradeI-II/VImid-diastolicflowrumbleisauscultatedattheapexinpatientswhohavemoderatetosevereindegreesofMR.Thismid-diastolicmurmurisheardbetterwiththestethoscope’sbell.Mid-diastolicmurmurisindicativeoflargeflowacrossthemitralvalveanddoesnotimplyadditionalmitralstenosis.TheBPandperipheralpulsesaregenerallywithinnormallimits.ECGdoesnotshowabnormalitiesinsubjectswithmildMR.LAenlargementandLVhypertrophymaybeseeninpatientswithmoderatetosevereMR.ChestX-rayisnormalinpatientswithmildMR.EnlargementoftheheartduetodilatedLAandLVmaybeseeninpatientswithmoderatetosevereMR.ThedegreeofcardiomegalyisinproportiontothemagnitudeofMR.M-modeand2DechostudiesareofvalueinquantifyingthesizesoftheLAandLV.Asarule,theLAandLVsizesareproportionaltotheamountofMR.LVshorteningfraction(SF)isusuallynormal.Insomepatients,thenormalSFvalueisduetotheLVemptyingintothelowresistanceLAinsteadoftheaorta,whichhashighperipheralvascularresistance.IftheLVsystolicfunctionworsens,theSFwilldecrease.ColorDopplerstudyishelpfulinconfirmingMR.TricuspidregurgitationTheholosystolicmurmurofTRisauscultatedatthelowerleftsternalborder.Typically,theintensityofthemurmurchangeswiththerespiratorycycle(increasesduringinspiration)andhenceisdissimilartothemurmurofventricularseptaldefect.Inaddition,themurmurofTRsoundsmore“superficial”and“scratchy”.However,theTRmurmurislessfrequentlyseenthanaVSDmurmur.TheTRmurmurisheardinassociationwithstructuraltricuspidvalvediseasessuchasEbstein’sanomalyofthetricuspidvalveanddysplastictricuspidvalve.Or,itisduetofunctionalabnormalityofthetricuspidvalve,relatedtoRVdysfunctioninbabieswithmyocardialischemiasyndromeormarkedelevationofPAorRVsystolicpressures.Accordingly,itappearsthatother,moresevereheartdiseasecoexistsinthesubjectswithTR.Increased“v”wavesinthejugularvenouspulsemaybeseen,andsystolicpulsationsinthelivermaybefelt.TheRVimpulseisincreased.Nothrillsareusuallyfelt.Thecharacterofthe2ndsoundisrelatedtothemaincardiacabnormalityproducingTR.Mid-diastolicflowmurmurofgradeI-II/VIintensityisheardbestatthelowerleftsternalborderifthedegreeofTRismorethanmoderate.Mid-diastolicmurmurisduetoaugmentedbloodflowviathetricuspidorifice.ECGdemonstratesRVHandfrequentlyreflectstheprimarydiseaseprocess.Chestroentgenogramgenerallyshowsanenlargedcardiacsilhouette,andtherightatrialshadowisprominent.Echo-DopplerstudiesshowRVvolumeoverloading.Theechofindingsunraveltheprimarydiseaseentity.ColorDopplerimagingclearlyshowstheTR.DiastolicmurmursIfthemurmurisplacedinbetweenthe2ndand1stheartsounds,itisdescribedasadiastolicmurmur[Figure3].Thesemurmursareclassifiedasfollows:(1)early;(2)mid;and(3)late[Figure3].Latediastolicmurmursaregenerallycalledpresystolicmurmurs.Whilethisclassificationisarbitrary,suchsubdivisionisconsideredclinicallyuseful.Figure3.Artist’srenditionofdiastolicmurmursclassifyinginto:(1)early(top);(2)mid(middle);and(3)lateorpre-systolic(bottom)diastolicmurmursareillustrated.ModifiedfromRao[5].EarlydiastolicmurmursEarlydiastolicmurmursusuallyhaveadecrescendocharacter(Figure3;top)andareproducedbyinsufficiencyofaorticorpulmonaryvalves.Theircausesareenumeratedin[Table6].Theprecordialsitewherethemurmurisheardbest,murmur’spitch,andattimes,carefulhistoryandfindingsonphysicalexaminationarehelpfulincomingupwithadiagnosis.Table6Causesofearlydiastolicmurmurs.ModifiedfromRao[5]AorticregurgitationPulmonaryregurgitationPulmonaryhypertension(Graham-Steelmurmur)AorticregurgitationEarlydiastolicmurmursofaorticregurgitation(AR)haveadecrescendocharacterandareauscultatedattheRUSBandLMSBs.Themurmurhasahighpitchandisauscultatedbetterwiththestethoscope’sdiaphragm.Themurmurbeginswiththeaorticcomponentofthe2ndsound(Figure3;top)andisbetterauscultatedwhenthepatientsitsup,leansforward,andholdsthebreathatend-expiration.ItmaytransmitinferiorlytotheLLSB.TheLVimpulseistypicallyprominent.Diastolicthrillsarerarelyfelt.Generally,therearenoabnormalcardiacsounds.IftheARisduetoabicuspidaorticvalve,anaorticsystolicclickisauscultated.Asystolicejectionmurmurisheardatupperrightoratmidleftsternalborders;thismayberelatedtotheincreasedamountofbloodthathastobepumpedbackviatheaorticvalveandnotduetoadditionalAS[Figure4].Alternatively,thesystoliccomponentmaybeduetoassociatedaorticvalvestenosis.Figure4.Artist’srenditionofejectionsystolicmurmur(secondarytostenoticaorticorpulmonaryvalveorduetoincreasedflowacrossthesemilunarvalvebecauseofvalvarregurgitation)andearlydiastolicdecrescendomurmur(duetoaorticorpulmonaryregurgitation).AnAustin-Flinttypeofmid-diastolicmurmurmaybeappreciatedattheapex(pleasereviewthediscussioninthesectiononmid-diastolicmurmurs).InmildARcases,theperipheralpulsesarenormallyfelt.Buttheyareamplifiedand“bounding”inpatientswithmoderateandsevereAR.Thepulsepressureisincreasedbecauseofincreasedsystolicbloodpressurewithconcurrentlydecreaseddiastolicpressure.PeripheralsignsofARsuchaswater-hammerpulse(quickincreaseanddecreaseofpulsewhenpalpatingtheforearm),Corrigan’spulse(strikinglyaugmentedcarotidpulses),Duroziez’smurmur[bruits(bothsystolicanddiastolic)auscultatedinthefemoralarteryregionwhileitispartlyblocked],pistolshot(Traube’s)sounds,andQuinke’spulse(flushingandblanchingalternativelyofthecapillarybedsofthetipsoffinger)areseeninsubjectswithmoderatetosevereAR;however,thesesignsdonotautomaticallyidentifythattheARissevere.TheECGisnormalinmildARcases.InmoderatetosevereARpatients,signsofLVenlargementareseen.Chestroentgenogramrevealscardiacenlargement(mostlyduetoLVdilatation).Classically,thereisnoevidenceforLAenlargementunlessmitralvalvediseaseisalsopresent.Echo-DopplerstudiesdemonstrateLVvolumeoverloading.TheanteriormitralleafletmaybeseentoflutteronechocardiographyandisindicativeofAR,andcorrespondstotheAustin-Flintmurmurdescribedabove.TheLVfractionalshorteningisnormalinitially,butincreaseswithtimeinresponsetoincreasedvolumetobepumpedbytheLV.Withtheonsetofmyocardialfailureeitherduetoverysevereand/orprolongedAR,theLVfractionalshorteningdecreases.DopplerstudyshowsflowreversalintheLVoutflowregion.ColorflowimagingunmistakablydemonstratesthejetofAR,whichisofuseinassessingthemagnitudeofAR.InmoderatetosevereARcases,flowreversalinthedescendingaortamaybeobserved;suchfindingsmayindicatethattheARissignificant.Pressure½timeofARjetisusefulinsemi-quantificationofAR.TheearlydiastolicmurmurofARmustbedistinguishedfromtheearlydiastolicmurmurassociatedwithpulmonaryregurgitation(PR)andpulmonaryhypertension.Thesewillbereviewedinthenextsection.ThecausesofARaremultiple,andtheseare:rheumaticheartdisease;bicuspidaorticvalve;prolapseoftheaorticvalveleafletinanattempttospontaneouslycloseaVSD;perforationofaorticvalveleaflet,whichmaybecongenitalinoriginorisduetopriorbacterialendocarditis;aorticrootdilatationsecondarytoMarfan’ssyndrome;aorta-to-LVtunnel,andperhapsothers.ItmayalsobeduetosurgicalvalvotomyorballoonaorticvalvuloplastyforAS.Informationfromhistory,otherphysicalfindings,andecho-DopplerexaminationarevaluableindeterminingthecorrectcauseofAR.PulmonaryregurgitationThemurmurofPRisalsoanearlydiastolicdecrescendomurmurandisbestauscultatedattheLUSBandLMSBs.Sometimes,itradiatesinferiorlytotheLLSB.ItisdifferentfromthemurmurofARbecauseitisoflowpitchqualityincontradistinctiontothehighpitchofARmurmur.Inaddition,therearenoperipheralmanifestationsofAR.Furthermore,themurmurofPRisbestauscultatedwiththestethoscope’sbell,withthepatientlyingflat.TheRVimpulseisusuallyprominent.Heartsoundsusuallyexhibitwidesplittingofthe2ndheartsound.Single2ndheartsoundmaybeheardinsomediseaseentitiesasreviewedinthenextsection.Asystolicejectionmurmur[Figure4]maybeappreciatedatLUSBandislikelytoberelatedtoaugmentedvolumeofbloodflowviathepulmonaryvalve,anddoesnotautomaticallysuggestassociatedPS.Nevertheless,pulmonaryvalvestenosisorpulmonaryvalveannularhypoplasiacanalsoproducesystolicmurmur.TheBPandpulsesareunremarkable.Asalludedtoabove,therearenoperipheralsignsofAR.ECGtypicallydemonstratesrightbundlebranchblock,especiallyifthePRisduetopreviouslyoperatedtetralogyofFallot.RVHmayalsobeseen.Chestroentgenogrammayeitherdemonstrateevidenceforpriorsurgery(forexample,sternalwires)oraneurismaldilatationofpulmonaryarteries(inpatientswiththesyndromeofabsentpulmonaryvalve).EchocardiogramwillshowRVvolumeoverloadinthepresenceofmoderatetoseverePRanddemonstratesotherheartdefects.DopplerexaminationshowsreversedDopplerflowvelocityintheRVoutflowtract.ColorflowmappingdocumentsthePRandhelpsquantifythedegreeofPR.ThemostfrequentcauseofPRispriorsurgicalrepairoftetralogyofFallot.SurgicalpulmonaryvalvotomyandballoonpulmonaryvalvuloplastyforvalvarPSaretheothercauses.Historicalinformationofthepreviousoperationorcatheterinterventionandthescarofprevioussurgeryareusefulhintsinthepatientassessment.Syndromeoftheabsenceofpulmonaryvalve,usuallyassociatedwithotherdefectssuchastetralogyofFallotorVSDshouldalsobeconsideredinthedifferential.ChestX-raymaysuggestaneurismaldilatationofthePAs.Butechocardiogramdemonstratesallthefeaturesofthesyndrome,namely,VSD,pulmonaryvalveringnarrowing,absentorrudimentarypulmonaryvalveleaflets,systolicpressuregradientacrossthepulmonaryvalve,PR,andmassivedilatationofthemain,right,andleftPAs.Cardiaccatheterizationandangiographyarenotnecessaryforthediagnosis,butifperformed,confirmtheabovefindings.PulmonaryregurgitationmurmurofpulmonaryhypertensionTheearlydiastolicdecrescendomurmurofpulmonaryhypertension,referredtoasGrahamSteel’smurmur,isauscultatedbestatLUSBandLMSBs.However,itexhibitsahighpitchsimilartoARmurmur.IncreasedRVimpulse,single2ndheartsound,andejectionsystolicclickalongtheleftsternalborderindicativeofpulmonaryhypertensionarealsofoundonexamination.Infrequently,amurmurofTRmaybeheard.Whilethehigh-pitchedqualityofthismurmurissimilartothatofAR,thelocationofthemurmurisattheLUSB,andtherearenoperipheralsignsofAR.RVHispresentintheECG.Echo-DopplerstudiesdemonstrateadilatedandhypertrophiedRV.ElevatedRV/PApressuresmaybedocumentedonthebasisofTRandPRjetvelocities.Inaddition,theheartdefectcausingpulmonaryhypertensionisalsoidentified.Mid-diastolicmurmursMid-diastolicmurmurs(Figure3;middle)aremoredifficulttoappreciatebecausetheyarelow-pitchedmurmurs,theyareoflowintensity(GradeI-II/VI),andaretypicallyconfinedtoasmallpartoftheprecordium.Thesemurmursaresecondarytoincreasedbloodflowthroughanormalatrioventricular(AV)valve(duringtherapidfillingphageoftheventricles)orduetoanormalamountofbloodflowthroughanarrowedAVvalve.Themid-diastolicmurmursrelatedtoflowacrossthemitralvalveareauscultatedbetteratanareasomewhatinternaltotheapex,whilethoserelatedtoflowacrossthetricuspidvalveareauscultatedattheLLSB.Bothmurmursareheardbestwhenusingthestethoscope’sbellforauscultation.TheetiologyofbothAVvalvemid-diastolicflowmurmursisshownin[Table7].Therearenodistinctivefeaturesofthesemurmursthatcandifferentiatethemid-diastolicmurmursfromoneanother;however,findingsinhistoryandphysicalexaminationareusefulinthedifferentialdiagnosis.Table7Etiologyofmid-diastolicmurmurs.ModifiedfromRao[5]1.Largeflowacrossthemitralvalve(a)Ventricularseptaldefect(b)Patentductusarteriosus(c)Mitralregurgitation2.Rheumaticmitralvalvulitis(CareyCoombsmurmur)3.Aorticregurgitation(AustinFlintmurmur)4.Mitralstenosis5.Largeflowacrossthetricuspidvalve(a)Atrialseptaldefect(b)Anomalouspulmonaryvenousconnection(partialortotal)(c)Tricuspidregurgitation6.TricuspidstenosisLargeflowacrossthemitralvalveIncreasedflowacrossthemitralvalveisproducedeitherbyaugmentedpulmonarybloodflowsecondarytoheartdefectssuchasVSDorPDAorduetomoderatetosevereMR(Table7;Item1).Aholosystolic,non-radiatingmurmurofVSDauscultatedatthelowerleftsternalborder,acontinuousmurmurofPDAheardattheLUSB,orholosystolicmurmurofMRauscultatedattheapicalregionwithradiationtoanterior,andmidaxillarylineswillhelpdifferentiatethemfromoneanother.RheumaticmitralvalvulitisInpatientswithacuterheumaticfever,amid-diastolicmurmurattheapex,namedCareyCoombsmurmurmaybeauscultated.Whiletheetiologyofthismurmurisnotclearlyestablished,itisgenerallythoughtthatthismurmurisrelatedtothethickeningofthemitralvalveleafletsalongwithedema.AnotherhypothesisisthatthereisrelativestenosisofthemitralvalveproducedbyadilatedLV.Inpatientswithclinicallysuspectedrheumaticfeverdiagnosis,appreciationofCareyCoombsmurmurisindicativeofinvolvementofthemitralvalveintherheumaticdiseaseprocess.AustinflintmurmurofaorticregurgitationInsubjectswithAR,amid-diastolicrumbleattheapex,namedAustinFlintmurmurmaybeheardattheapex.ThismurmurisbelievedtoresultfromthejetoftheARimpingingontheanteriorleafletofthemitralvalve,makingitshudder.Thepresenceofahigh-pitchedearlydiastolicmurmurofARatRUSBimpliesthatthisapicalmurmurisanAustinFlinttypeofmurmur.MitralstenosisRheumaticandcongenitalmitralstenosesdoresultinmid-diastolicmurmurs;however,typically,themid-diastolicmurmurspillsintothelaterpartofthediastoleandbecomesmoreprominentandistermedpresystolicaccentuation.IncreasedflowacrossthetricuspidvalveIncreasedflowacrossthetricuspidvalveisproducedby(1)largeshuntscausedbyASD,PAPVC,ortotalanomalouspulmonaryvenousconnection(TAPVC);and(2)moderatetosevereTR(Table7;Item5).AlltypesofASDs(secundum,primum,sinusvenosus,andcoronarysinus),iftheyarelargeenoughtoresultinQp:Qsratio≥2:1,mayhaveamid-diastolicmurmurauscultatedbestattheLLSB.Unfortunately,therearenocharacteristicfindingsofthemid-diastolicmurmurthatdifferentiatesonefromtheother.ClinicalsignsofASD,PAPVC,orTAPVCorofthemurmurofTRheardbestattheLLSBareusefulindifferentiatingtheetiologyofmid-diastolicflowmurmursheardbestattheLLSB.TricuspidstenosisCongenitalorrheumatictricuspidvalvenarrowing,althoughuncommon,mayresultinmid-diastolicmurmurheardbestatlowerleftsternalborder.Thereisusuallyapre-systolicaccentuationofthemurmur.PresystolicmurmursPresystolicmurmur(Figure3;bottom)isinfrequentinchildrenindevelopedcountriesandisproducedbystenoticAVvalves.Theetiologyofpresystolicmurmursistabulated[Table8].Themurmurlocationonauscultationandthefindingsinhistoryandphysicalexaminationareusefulindifferentiatingthecausesofthesemurmurs.Table8Etiologyofpresystolicmurmurs.ReproducedfromRao[5]Mitralstenosis,rheumaticandcongenitalTricuspidstenosis,congenitalandrheumaticLeft(orright)atrialmyxomaMitralstenosisMostcommonly,mitralstenosisisrheumaticinorigin.Mitralstenosisresultsinapresystolicmurmuraswellasaprecedinglow-pitchedmid-diastolicmurmur.Themid-diastolicmurmurbecomesmoreintenseinlatediastoleandisgenerallydescribedaspresystolicaccentuation.ThemurmurendsinS1.Themurmurisproducedbyrapidbloodflowviathestenoticmitralvalveallthroughtheatrialsystole.Consequently,themurmurcannotbeauscultatedinsubjectswhoareinatrialfibrillation.Themurmurisbetterauscultatedattheapicalregionandisbestheardwiththestethoscope’sbell.Themurmurisbetterheardwhenthepatientisinlateraldecubitus.TheRVimpulseisusuallyincreased,butwithanormalLVimpulse.Frequently,athrillindiastoleisfeltattheapex.Unlesscarefullytimed,thediastolicthrillofrheumaticmitralstenosismaybeerroneouslyinterpretedasasystolicthrill.Onauscultation,the1stheartsoundisloud.Thepulmonarycomponentofthe2ndheartsoundisaccentuatedinpatientswhohavehighPApressures.Anopeningsnapofhigh-frequencyqualitymaybeauscultatedattheapex;thesnapisthoughttobecausedbythesuddenopeningofthestiffenedmitralvalveleaflets.Itisimportanttodifferentiatetheopeningsnapfromaloud3rdsoundandthepulmonarycomponentofthe2ndsound.AsystolicmurmurcanbeheardattheapexinpatientswhohaveadditionalMR.TheBPandarterialpulsesareusuallynormal;however,inpatientswithveryseveremitralstenosis,thepulsevolume(pulsepressure)isdecreased.TheECGdemonstratesLAenlargementandRVH.ChestroentgenogramillustratesLAdilatation,dilatedmainPA,andcongestionofthepulmonaryvenousstructures.CephalizationofpulmonaryvascularmarkingsandKerleyBlines,indicativeofdilatedlymphaticsmaybevisualized.M-modeechorevealsdecreasedanteriormitralleaflet’sEtoFslopeandreducedmovementoftheposteriorleafletofthemitralvalve.2Dechohelpsestimationofthemitralvalvearea.DopplerinterrogationoftheLVinflowregion,measuringmitralinflowgradientisusefulindetermininggradientthroughthestenoticmitralvalve.Patientswhohavemitralstenosisofcongenitaloriginarelikelytoexhibitsimilarmurmurs;however,thepatientsareclearlyyounger.Inaddition,aloudfirstsoundisnotpresent,andanopeningsnapisnotheardbecausethemitralvalveleafletsarenotmobilewithshortandthickchordaeinbabieswithcongenitalmitralstenosis.OtherCHDswithcomparablepathophysiologyarecortriatriatumandparachutemitralvalve.Suchconditionsdonotexhibitdistinctivepresystolicmurmur.TricuspidstenosisTricuspidvalvestenoses,bothcongenitalandrheumatic,areuncommon.Frequently,tricuspidstenosisisseeninassociationwithotherCHDs.Insuchsituations,theclinicalfeaturesarelargelydeterminedbytheassociateddefects.Occasionallytricuspidvalvemaybeinvolvedinasevererheumaticprocesscausingtricuspidstenosis.ThepresystolicmurmuroftricuspidstenosisisauscultatedbestatLLSB;theintensityofmurmurincreaseswithinspiration.Intricuspidstenosisopeningsnapisnotcommonlyheard.Increased“a”wavesinthejugularvenouspulseandpresystolichepaticpulsationsmaybeseen/felt.However,ifthereisanatrialdefectdecompressingtherightatrium,thementionedabnormalitiesarenotpresent.ECGshowsrightatrialenlargement.M-modeechodemonstratesreducedamplitudeofthetricuspidvalveleaflets.2Dechomayshowasmalltricuspidvalveopening.DopplerinterrogationoftheRVinflowregion,measuringtricuspidinflowgradient,isusefulinquantifyingthegradientacrossthetricuspidvalve.AtrialmyxomaAtrialmyxomasareinfrequentinthepediatricpopulation.Ifpresent,atrialmyxomasaremorecommonintheleftthanintherightatrium.Embolicepisodesorposturalinducedsyncopalepisodesarelikelytobethepresentingsymptoms.Presystolicmurmursindicativeofmitralvalvestenosis,whichvarieswithpatientposition,aresuggestiveofmyxoma.2Dechoisanexcellentmethodindetectingmyxoma.ContinuousmurmursBydefinition,murmursthatstartinsystoleandspillintodiastolearenamedcontinuousmurmurs.Thesystoliccomponentofthemurmurcrescendosuptothe2ndheartsoundwhilstthediastolicpartdecrescendostoavariabletimeintothediastole(Figure5;top).Figure5.Murmurswhichstartinsystoleandspillintodiastole,arenamedcontinuousmurmurs(top).Themurmurstartsduringthesystole,hasacrescendocharacteruntilitreachesthe2ndheartsound(S2).Then,themurmurdecrescendostoavariabletimethroughthediastole.Incontradistinction,ato-and-fromurmur(bottom)iscomposedoftwoseparatemurmurs:(1)anejectionsystolicmurmur;and(2)earlydiastolicdecrescendomurmur.AdistinctspacebetweentheejectionmurmurandS2ispresent.ModifiedfromRao[5].Thecontinuousmurmursaretypicallycausedbybloodflowfromcardiacchambersorbloodvesselswithhighpressure/resistanceintovenousstructureswithlowerpressure/resistance.Thebloodflowoccursbothduringsystoleanddiastolesincethereisapressure/resistancedifferenceduringtheentirecardiaccycle.Thecontinuousmurmurmustbedifferentiatedfromtheto-and-fromurmur(Figure5;bottom).Thelatterisamixtureofsystolicejectionmurmur(duetoaorticorpulmonarystenosis)andanearlydiastolicdecrescendomurmur(duetoaorticorpulmonaryregurgitation).Adistinctgapbetweentheejectionsystolicmurmurandthe2ndheartsoundisseeninto-and-fromurmurs(Figure5;bottom),whereassuchagapisnotseeninthecontinuousmurmur(Figure5;top).Theetiologyofcontinuousmurmursisshownin[Table9].Themurmurlocation,changesinthemurmurcharacterwithchangesinbodyposture,andfindingsinhistoryandphysicalexaminationoftheprimarycardiacdefectareusefulinthedifferentialdiagnosis.Table9Causesofcontinuousmurmurs.ModifiedfromRao[5]CommoncausesPatentductusarteriosusVenoushumSurgicalaorto-pulmonaryshuntsLesscommoncausesAortopulmonarywindowPersistenttruncusarteriosusHemitruncusEmbryoniccollateralvesselsinpulmonaryatresiawithventricularseptaldefectCoronaryarteriovenousfistulaRupturedsinusofValsalvaaneurysmPulmonaryarteriovenousfistulaPeripheralpulmonaryarterystenosisCoarctationoftheaortaObstructedvenousreturnCervicalarteriovenousmalformationPatentductusarteriosusThecontinuousmurmurassociatedwithPDAisbetterauscultatedattheLUSB.ThemurmurvariesfromgradesItoV/VIinloudness.ThePDAisafrequentcauseofpathologiccontinuousmurmur.Characteristically,numerousejectionclickswithinthemurmurareauscultatedandareconsideredtypicalforPDA.Themurmurisalsoportrayedasamachinerymurmur.Mostofthetime,thereisnochangeinmurmurcharacteristicswithachangeinthebodyposition,thoughthediastolicpartofthemurmurisauscultatedbetterwhenthepatientissupinethanwhenhe/sheisinanuprightposition.Nevertheless,inpatientswithextremelysmallPDAs,thecontinuousmurmurmayeitherdisappearorbecomeonlysystolicintiminginsitting-upposition,butresumestocontinuoustypefollowingresumptionofsupineposture[1].Thesuggestedreasonforthisphenomenonis“kinking”oftheductuswhenthepatientisintheuprightposition[1].TheLVimpulseiswithinnormallimitsinsmallPDAs,butitisprominentinpatientswhohavemoderatetolargePDAs.Athrillisusuallyappreciatedattheupperleftsternalborderand/orinthesuprasternalnotch.The2ndsoundisfrequentlywithinnormalrange;however,itishardtobeheardsinceitisburiedintheloudcontinuousmurmur.InpatientswithmoderatetolargePDAs,amid-diastolicflowmurmurisauscultatedattheapexsecondarytoincreasedbloodflowacrossthemitralvalve.Mid-diastolicmurmurofthistypeimpliesaQp:Qsratio≥2:1.ArterialpulsesareboundinginpatientswithmoderatetolargePDAs.TheECGisnormalinsmallPDAs.EvidenceforLAandLVenlargementisseeninpatientswithmoderatetolargePDAs.ChestX-rayshowsanormal-sizedheartandnormalpulmonaryvascularmarkingsinpatientswithsmallPDAs,whileenlargementoftheheartwithincreasedpulmonaryvascularmarkingsmaybepresentinsubjectswithmoderatetolargePDAs.LAenlargementmayalsobedetectedonthechestfilm.LungcollapsewithsecondaryinflammatorychangesmaybeseeninsmallbabieswithlargePDAs.Echo-Dopplerstudiesdemonstratenear-normal-sizedLAandLVinsmallPDAs.EnlargementoftheLAandLVcanbeseeninpatientswithmoderatetolargePDAs;thesechangesarelargelyproportionaltothesize(minimalductaldiameter)ofthePDA.TheLVcontractilefunction,asevaluatedbyLVfractionalshorteningandejectionfraction,isnormalinitiallyandmaybecomehyper-contractilewithtime.Whenseveremyocardialdysfunctionduetoprolongedand/orlargeshuntoccurs,LVcontractilefunctionindicesdeteriorate.Dopplerinterrogationdemonstratesadistinctivediastolicflowpatterninthepulmonaryartery,suggestiveofPDA.ColorflowimagingclearlydemonstratesthePDA.VenoushumThemurmurofvenoushumisalsoacontinuousmurmur;butisnotduetobloodflowfromhighpressuretolowpressurecardiovascularstructures(describedabove).Itisbestauscultatedintheinfraclaviculararea,supraclavicularfossa,andateitherLUSBorRUSBs.ThemurmurisverysoftandisusuallynolouderthangradeII/VI.Thediastolicpartofthemurmurisalittlebitlouderthanthesystolicportion.Themurmurisheardbetterwhenthepatientissittingup.Themurmurfullygoesawayorturnsouttobeonlysystolicbycompressingtheveinsintheneckorbyrotatingthepatient’sheadtotheoppositeside.Themostimportantfeatureisthatthemurmurgoesawaycompletelyinthesupineposition.Thismurmuristhemostfrequentofallfunctionalheartmurmurs;however,itisnotfrequentlyidentifiedbyanuninitiatedauscultator.ThemurmurofvenoushumisdifferentfromthatofPDAinthatitismuchsofteranddisappearsinthesupineposition.Theotherfindingsincardiacevaluationarecompletelynormal.Also,theresultsoftheECG,chestX-ray,ifperformed,arenormal,asaretheresultsoftheechocardiogram.Surgicallycreatedaorto-pulmonaryshuntsAnumberofaorta-to-pulmonaryarteryshuntsarecreatedbysurgeryinordertoaugmentpulmonarybloodflow.Alltheseshuntproceduresexhibitcontinuousmurmursonauscultation.Inthe1940s,anastomosisofthesubclavianarterytothepulmonaryarteryonthesameside,nowcalledclassicalBlalock-Taussig(BT)operation[21]wasusedtoincreasethepulmonarybloodflow.Subsequently,severalothertypesofsurgeryhavebeenusedtoaccomplishthesamepurposeofaugmentingthepulmonarybloodflow,andtheseincludePottsanastomosis,Waterstonoperation,centralaorto-pulmonaryshunt(directlyorwithaGore-Texgraft),andmodifiedBTshunt.InthemodifiedBTshunt,aninter-positionGore-TexgraftisinsertedbetweenthesubclavianarteryandtheipsilateralPA[22].Morerecently,Sanoshunts[23,24],connectingtherightventricularoutflowtracttothepulmonaryarterywithaGore-Texgraft,havebeenemployedtoperfusethepulmonarycirculation.Alltheaboveshuntsgeneratecontinuousmurmurs.Inpractice,murmursresultingfromthesurgicallycreatedshuntsaredifficulttodifferentiatefromthemurmurofPDA,withtheexceptionthatthemultipleclicksofPDAarenotauscultatedinsubjectswhohadsurgicallycreatedshunts.Informationregardingthesurgicalhistoryandthesiteofthesurgeryscarmaybeusefulindiagnosingthetypeofoperationproducingthecontinuousmurmur.ChildrenwithpatentBTshuntswillhavecontinuousmurmursattheuppersternalborderonthesamesideasthescaronthechest.Onpalpation,thebrachialandradialpulsesarereducedornotpalpableinpatientswithclassicBTshunts,whilethosewithmodifiedBTshuntswillnothavesuchpulsedeficiency.However,itshouldbenotedthatclassicBTshuntsarerarely,ifever,performedatthistime.PatientswhohadPottsandWaterstonshuntswillhavemorecentrallylocatedcontinuousmurmurs,haveeithermid-sternal,left(Potts),orright(Waterston)thoracotomyscars,butitshouldbeknownthattheseshuntsarenolongerperformed.Thecentralaorto-pulmonaryGore-Texgraftshuntsaretypicallyperformedviaamid-sternotomyapproach,andthemurmurismoremedialinlocation,andtheydonotexhibitanypulsedeficit.Finally,theSanoshuntsusuallyhavenoorlessprominentdiastoliccomponent.Themajorityofthepatientswhohadbeenpalliatedwithanyoftheabove-describedshuntswillcontinuetoexhibitcyanosisbecausetheprimarycyanoticCHDhasnotbeencorrected.Iftheshuntislargewiththeincreasedpulmonaryflow,thecyanosisismildornotclinicallydetectable,andsuchpatientswillhavemid-diastolicflowrumbleattheapex.Thephysicalexamination,chestX-ray,ECG,andecho-Dopplerstudieswilldemonstratetheprimarycardiacdefect.OthercausesofcontinuousmurmurFrequentetiologiesofcontinuousmurmurwerereviewedintheprecedingsection.Lessfrequentcausesofcontinuousmurmur[5,25]arelistedin(Table9;bottom).Theseinclude:aorto-pulmonarywindow;truncusarteriosus;hemitruncus;multipleaorto-pulmonarycollateralarteriesinchildrenwithtetralogyofFallot;ruptureofsinusofValsalvaaneurysm;coronaryarterio-venousfistula;pulmonaryarterio-venousfistula;peripheralPAstenosis;aorticcoarctation,obstructedvenousreturn,andcervicalarteriovenousmalformation.Reviewoftheclinicalfeaturesandfindingsinchestroentgenogramsandecho-Dopplerstudiesarehelpfulinidentifyingtheserarecausesofcontinuousmurmur;theseweredetailedelsewhere[5]fortheinterestedreader.SUMMARYANDCONCLUSIONSCardiacmurmuriscommonlyheardonauscultation.Murmuristhefrequentreasonfortherecognitionofheartdiseaseinchildren(withtheexceptionofneonates).Masteryofskillsofauscultationacquiredbytrainingandexperienceisimportantindiagnosingthecausesofcardiacmurmurs.Cardiacpatientsimulatorsandcomputer-assistedtrainingmethodshavebeenusedtoeducatestudentsandresidents;thesemethodsshouldsupplementbedsideacquisitionofauscultatoryskillsunderthesupervisionofexperiencedcliniciansandnotbecomeprimarymodesoftrainingofouremergingphysicianpool.Murmursareclassifiedintosystolic,diastolic,andcontinuoustypes.Thesystolicmurmursarefurtherdividedintoejectionsystolicandholosystolicmurmurs.ThemorecommonetiologiesofejectionsystolicmurmursareAS,PS,ASD,coarctationoftheaorta,andfunctionalheartmurmurs.ThecausesofholosystolicmurmursareVSD,MR,andTR.Thediastolicmurmursareclassifiedintoearly,midandlate(orpresystolic)diastolicmurmurs.TheearlydiastolicmurmursarecausedbyAR,PR,andpulmonaryhypertension.Mid-diastolicmurmursareproducedbyincreasedflowacrossthemitralvalve(secondarytolargeshuntsacrossaVSDorPDAormoderatetosevereMR)orhighflowviathetricuspidvalve(secondarytoASD,partialortotalanomalouspulmonaryvenousconnectionormoderatetosevereTR).OthercausesareCarey-Coombsmurmurofrheumaticfever,Austin-FlintmurmurofAR,andstenosisoftheAVvalves.Thepresystolicmurmursareproducedbystenosisofthemitralortricuspidvalveandatrialmyxoma.ThecontinuousmurmursaremorecommonlyproducedbyPDA,venoushum,oraorto-pulmonaryshuntprocedures.Therearemanyotherlesscommoncauses.Carefulauscultationandotherfindingsinhistory,physicalexamination,chestroentgenogram,andECGwillfrequentlyhelpcomeupwithanaccuratediagnosis.Echo-Dopplerstudiesarevaluableandconfirmatoryinmakingthediagnosis,inquantifyingtheproblem,andareveryusefulindirectingthetypeofandtimingofmanagement.DECLARATIONSAcknowledgmentsTheauthorwishestothanknumerouspatients(andtheirparents)whomtheauthorhadtheprivilegetoexamineatmultipleinstitutionsoverthelastfivedecades.Theexperiencesogainedresultedinformulatingthisreview.Authors’contributionsTheauthorcontributedsolelytothearticle.AvailabilityofdataandmaterialsNotapplicable.FinancialsupportandsponsorshipNone.ConflictsofinterestTheauthordeclaredthattherearenoconflictsofinterest.EthicalapprovalandconsenttoparticipateNotapplicable.ConsentforpublicationNotapplicable.Copyright©TheAuthor(s)2022.References1.Thapar MK,Rao PS,Rogers JH,Moore HV,Strong WB.Changingmurmurofpatentductusarteriosus.JPediatr1978;92:939-41.DOIPubMed2.Rao PS.Auscultationisstillavalidtoolintheevaluationofcardiacdefectsinchildren.CongenitalCardiologyToday2018;16:1-6.3.Rao PS.Anapproachtothediagnosisofcyanoticneonatefortheprimarycareprovider.NeonatologyToday2007;2:1-7.4.Rao PS.Anapproachtothediagnosisofcyanoticneonatefortheprimarycareprovider.In:RaoPS,VidyasagarD,editors.Amultidisciplinaryapproachtoperinatalcardiology.NewCastleuponTyne,UK:CambridgeScholarsPublishing;2021.p.294-312.5.Rao PS.Evaluationofcardiacmurmursinchildren.IndianJPediat1991;58:471-89.PubMed6.Austin AV,Owens DS,Prutkin JM,etal.Do'pathologic'cardiacmurmursinadolescentsidentifystructuralheartdisease?BrJSportsMed2021:bjsports-2019.DOIPubMed7.Gonzalez VJ,Kyle WB,Allen HD.Cardiacexaminationandevaluationofmurmurs.PediatrRev2021;42:375-82.DOIPubMed8.Levine SA.Clinicalheartdisease.4thed.Philadelphia:W.B.SaundersCo.;1951.p.229-40.9.Leatham A.Auscultationoftheheart.Lancet1958;272:703-8.DOIPubMed10.Kostopoulou E,Dimitriou G,Karatza A.Cardiacmurmursinchildren:achallengefortheprimarycarephysician.CurrPediatrRev2019;15:131-8.DOIPubMed11.Butter J,McGaghie WC,Cohen ER,Kaye M,Wayne DB.Simulation-basedmasterylearningimprovescardiacauscultationskillsinmedicalstudents.JGenInternMed2010;25:780-5.DOIPubMedPMC12.McKinney J,Cook DA,Wood D,Hatala R.Simulation-basedtrainingforcardiacauscultationskills:systematicreviewandmeta-analysis.JGenInternMed2013;28:283-91.DOIPubMedPMC13.Perlini S,Salinaro F,Santalucia P,Musca F.Simulation-guidedcardiacauscultationimprovesmedicalstudents'clinicalskills:thePaviapilotexperience.InternEmergMed2014;9:165-72.DOIPubMed14.Power JE,Toft LEB,Barrett M.Themurmuronlinelearningexperience(MOLE)curriculumimprovesmedicalstudents'abilitytocorrectlyidentifycardiacmurmurs.MedEdPORTAL2020;16:10904.DOIPubMedPMC15.Vogelpocl L,Schrire V.Auscultatoryandphonocardiographicassessmentofpulmonarystenosiswithintactventricularseptum.Circulation1960;22:55-72.DOI16.Viviers PL,Kirby JH,Viljoen JT,Derman W.Thediagnosticutilityofcomputer-assistedauscultationfortheearlydetectionofcardiacmurmursofstructuraloriginintheperiodichealthevaluation.SportsHealth2017;9:341-5.DOIPubMedPMC17.Takahashi K,Ono K,Arai H,Adachi H,Ito M,Kato A,Takahashi T.Detectionofpathologicheartmurmursusingapiezoelectricsensor.Sensors(Basel)2021;21:1376.DOIPubMedPMC18.Lim GB.AIusedtodetectcardiacmurmurs.NatRevCardiol2021;18:460.DOIPubMed19.Ip HL,Menahem S.Doesechocardiographyhavearoleinthecardiologist'sdiagnosisofinnocentmurmursinchildhood?HeartLungCirc2020;29:242-5.DOIPubMed20.Rees AH,Rao PS,Rigby JJ,Miller MD.Echocardiographicestimationofleft-to-rightshuntinisolatedventricularseptaldefects.EuropeanJCardiol1978;7:25-33.PubMed21.Blalock A,Taussig HB.Thesurgicaltreatmentofmalformationsoftheheart:inwhichthereispulmonarystenosisorpulmonaryatresia.JAMA1945;128:189-202.DOI22.deLevalMR,MckayR,JonesM,StarkJ,MacartneyFJ.Modifiedblalock-taussigshunt.JThoracCardovascSurg1981;81:112-9.DOI23.Sano S,Ishino K,Kawada M,etal.Rightventricle–pulmonaryarteryshuntinfirst-stagepalliationofhypoplasticleftheartsyndrome.JThoracCardiovascSurg2003;126:504-9.DOIPubMed24.Sano S,Ishino K,Kado H,etal.Outcomeofrightventricle-to-pulmonaryarteryshuntinfirst-stagepalliationofhypoplasticleftheartsyndrome:amulti-institutionalstudy.AnnThoracSurg2004;78:1951-7;discussion1957-8.DOIPubMed25.Kulangara RJ,Strong WB,Miller MD.Differentialdiagnosisofheartmurmursinchildren.PostgradMed1982;72:219-28.DOIPubMedCiteThisArticle Rao PS.Diagnosisofcardiacmurmursinchildren. VesselPlus 2022;6:22.http://dx.doi.org/10.20517/2574-1209.2021.105 Views552Downloads55Citations 0Comments00 Comments CommentsmustbewritteninEnglish.Spam,offensivecontent,impersonation,andprivateinformationwillnotbepermitted.IfanycommentisreportedandidentifiedasinappropriatecontentbyOAEstaff,thecommentwillberemovedwithoutnotice.Ifyouhaveanyqueriesorneedanyhelp,[email protected]. Post Login 0 Cancel Loginwith OAEMESAS tostartadiscussion! Tip× Ok Citation Rao PS.Diagnosisofcardiacmurmursinchildren. 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