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Andersons pediatric cardiology 1460

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EchocardiographyandRheumaticHeart
Disease
Echocardiographyisanoninvasiveportablediagnostictoolthathasbecome
affordableeveninresource-poorsettings.Inmostoftheworld,
echocardiographicdiagnosishasreplacedtheclinicaldiagnosisofRHD.21
Echocardiographyisessentialto

■Diagnoseacuterheumaticcarditisinanindividual
withsuspectedARF
■ConfirmthediagnosisofRHDinindividuals
withoutaclinicalhistoryofARF
■Gradetheseverityofvalvardysfunction
■AssessserialLVsizeandfunction
■Guidethetimingandnatureofsurgicalintervention
■Determinethedurationofsecondaryprophylaxis
■ScreenforlatentandsubclinicalRHDthatwould
otherwisebemissedonclinicalexamination
MitralValveDisease
ChronicMVdiseaseisthemostcommonmanifestationofRHD.Thenatural
historyofrheumaticMVdiseasehasbeendescribedasapendulumswinging
fromregurgitationtocompleteresolutionwithoutevidenceofheartdiseasein
somecasesbutprogressiontomixedMR/MSorpurevalvarstenosisinothers.18
Inchildren,isolatedpureMRisthemostcommonformofchronicRHD.
Earlyinthediseaseprocesstheleafletsremainrelativelythin,chordaeelongate,
andtheannulusdilates,leadingtovalvarregurgitation.Withtime,scarringand
fibrosisoftheleafletapparatusresultinrigidityandrestrictedmovementofthe
leaflets.TheappearanceoftheanteriorMVleafletisoftendescribedasa“dog
leg”or“hockeystick”deformity(Figs.55.4and55.5).Withtimetheleaflets


thicken,retract,andcalcify.Thereisalsocommissuralfusionaswellaschordal


shortening,thickening,andfusionleadingtoMS(Figs.55.6and55.7).
ProgressivecalcificationoftheMVapparatusmaydevelop.Thisprocessoften
takesmanydecades25,35;however,insomeregionsoftheworldwhereARF
remainshyperendemic,progressiontosevereMScanbeveryrapidandaffect
childrenasyoungas5to10yearsofage.Thisisoftenreferredtoasjuvenileor
malignantMS.19




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