Andersons pediatric cardiology 1458

2.Inatleastoneviewjetlengthis≥1cmb
3.Peakvelocity≥3m/s
4.Pandiastolicjetinatleastoneenvelope

MorphologicFeaturesofRHD
MV
1.AMVLthickening≥3mmc
2.Chordalthickening
3.Restrictedleafletmotion
4.Excessiveleaflettipmotionduringsystole
AV
1.Irregularorfocalthickening
2.Coaptationdefect
3.Restrictedleafletmotion
4.Prolapse
BorderlineRHDcategoryonlyappliestoindividualsyoungerthan21years.
AR,Aorticregurgitation;AMVL,aorticmitralvalveleaflet;AV,aorticvalve;
MR,mitralregurgitation;MS,mitralstenosis;MV,mitralvalve;RHD,rheumatic
heartdisease.

aCongenitalanomaliesmustbeexcluded.
bAregurgitantjetlengthshouldbemeasuredfromthevenacontractatothelast

pixelofregurgitantcolour(blueorred)onnonmagnified(nonzoomed)images.
cAMVLthicknessshouldbemeasuredduringdiastoleatfullexcursion.

Measurementshouldbetakenatthethickestportionoftheleafletandshouldbe
performedonaframewithmaximalseparationofchordaefromtheleaflet
tissue.

DiagnosisofRHDWithoutaPreviousHistoryof
ARF
ThemajorityofpatientswithRHDworldwidefitintothecategoryofhaving
RHDwithnopriorhistoryofARF.Thereasonforcardiologyreferralor
assessmentmayincludeevaluationofcardiacmurmur,symptomaticstatus,
complicationsofRHD,orabnormalitydetectedonechocardiographicscreening.
The2012WorldHeartFederationechocardiographiccriteriawereestablished
tofacilitateearlydiagnosisofRHDinindividualswithoutaprevioushistoryof
ARF.22Box55.1detailstheminimumdiagnosticcriteria.Inthissetting,
pathologicregurgitationisinsufficienttodiagnoseRHD.Bothmorphologic
featuresofRHDandpathologicregurgitationmustbepresentor,alternatively,
theremustbemultivalvediseaseofboththeMVsandAVs.
Ifechocardiographyisnotavailable,thenthediagnosisreliesonauscultatory
findingsofvalvardysfunction.Naturallyauscultationalonedoesnotdetermine
theetiologyofdisease.Inthissetting,thepretestprobabilityofRHDwill
determinediagnosticandmanagementstrategies.


DiseaseProgression
TheoutcomeandprogressionofRHDrelatetotheseverityatdiagnosis,
exposuretoARFrecurrences,andaccesstotertiarymedicalcare.11,23
ThosewithmildRHDatdiagnosisandgoodadherencetosecondary
prophylaxishaveexcellentlong-termoutcomes.8,11Thosewithseverediseaseat
diagnosishaveableakprognosisandwilllikelyrequirecardiosurgical
interventionwithin2yearsofdiagnosisorwillsuccumb.23,24
IntheUnitedStates,beforetheintroductionofsecondaryprophylaxis,20-year
mortalityduetoRHDwasashighas30%to80%,withmostaffected
individualsdyingbeforetheageof30years.25,26Similarfindingsarestill
observedinmanylow-andmiddle-incomecountries,withannualmortalityrates
of3.0%to12.5%.InNigeriaandEthiopia,themeanageatdeathisbelow25

years.27,28InNewZealand,ahigh-incomecountry,theoutcomeofRHDismore
favorable,withamedianlifeexpectancyof56years.29TheNewZealanddata
mayreflectthetailofRHDinEuropeanadultswhohadmilderformsofRHD
fromearlierdecades,whenARFaffectedadultsofEuropeanethnicity,oritmay
reflectbetteraccesstomedicalcare.Finally,therearelimitationstousing
InternationalClassificationofDiseases(ICD)dischargedatawhennonspecified
valvardiseaseisattributedarheumaticetiology.
Overall,itcanbesaidthatthediseaseprogressionandmortalityduetoRHD
isgreatestinlow-incomecountries,andwithinthosecountriesitisthemost
disadvantagedgroupsthathavetheworstoutcomes.