Andersons pediatric cardiology 1457

FIG.55.3 Patternofnativerheumaticheartdiseasein2475childrenand
adultswithnopercutaneousorsurgicalinterventionintheinternational
REMEDYregistryforrheumaticheartdisease.AVD,Aorticvalvedisease;
MAVD,mixedAVD;MMAVD,mixedmitralandAVD;MMVD,mixedmitral
valvedisease;MR,mitralregurgitation;MS,mitralstenosis.

Patientsyoungerthan10yearspredominantlyhavepureMR.15,18Bythe
seconddecadeoflife,RHDischaracterizedbymixedMVdisease.Inthethird
decadeoflife,upto5%ofRHDpatientswilldeveloppureMS.15,18Insome
regionsofSub-SaharanAfricaandIndia,pureMSismorecommonandoften
occursearlierinlife.19ConcomitantAVdiseaseincreaseswithageandispresent
inover50%ofpatientsbytheseconddecade(seeFig.55.3).15LikeMVdisease,
aorticdiseaseintheyoungischaracterizedbyregurgitation.Bythesecond
decadeoflife,mixedaorticdiseasedevelopsinsome;however,pureaortic
stenosisisgenerallynotseenuntilthefourthorfifthdecadeoflife.15


DiagnosticCriteriaforRheumaticHeart
Disease
ThemajorityofpatientswithRHDarediagnosedlate,whenindividualspresent
withcomplicationsofRHDincludingheartfailure,infectiveendocarditis,
tachyarrhythmias,stroke,pregnancy-relatedcomplications,orsuddendeath.15
PatientswithRHDoftenhavealonglatentphaseofasymptomaticvalvarheart
disease,oftenwithoutanyprecedingsymptomsofARF.Theglobalregistryof
RHD,REMEDY,suggeststhateveninhigh-incomepopulations,only59%of
patientswithRHDhadaprecedinghistoryofARF,andthisdropsto22%in
low-incomepopulations.15Thereasonforthisislikelymultifactorial,andaccess
tohealthcareandpublicawarenessareimportantfactors.ThecurrentARF
guidelinesmaynotbesufficientlysensitivetodetectARFinhigh-risk
populations.Patientsmaynotpresenttolocalhealthfacilitieswithwhatmight
beseenasrelativelyminorsymptomsor,alternatively,medicalofficersmaynot

beequippedtomakethediagnosis.IncontrasttothearthritisofARF,acute
carditismaynotcausesymptoms.ConsequentlytheearlydiagnosisofRHD
remainschallenging.Thisisespeciallysoinresource-poorsettings.

DiagnosisofRHDintheSettingofa
DocumentedEpisodeofARF
InindividualswithadocumentedhistoryofARF,onceacuteinflammation
subsides,thepersistenceofpathologicregurgitationofthemitraland/orAVson
echocardiographyissufficienttoconfirmchronicRHD.Theechocardiographic
criteriatodiagnosepathologicmitralandaorticregurgitation(AR)aredetailed
inBox55.1.20Incircumstanceswhereechocardiographyisnotavailable,the
persistenceofamitraland/oraorticregurgitantmurmurissufficient,although
auscultationhasalowpositivepredictivevalueandhencelowdiagnosticutility
indetectingmildregurgitation.21


Box55.1


WorldHeartFederationCriteriaforthe
EchocardiographicDiagnosisofRheumatic
HeartDisease
EchocardiographicCriteriaforRHDa
DefiniteRHD(EitherA,B,C,orD):
A.PathologicMRandatleasttwomorphologicfeaturesofRHDoftheMV
B.MSmeangradient≥4mmHg
C.PathologicARandatleasttwomorphologicfeaturesofRHDoftheAV
D.BorderlinediseaseofboththeAVandMV
BorderlineRHD(EitherA,B,orC):
A.AtleasttwomorphologicfeaturesofRHDoftheMVwithoutpathologic

MRorMS
B.PathologicMR
C.PathologicAR

EchocardiographicCriteriaforPathologic
Regurgitation(AllFourDopplerCriteriaMustBe
Met)
PathologicMR
1.Seenintwoviews
2.Inatleastoneviewjetlengthis≥2cmb
3.Peakvelocity≥3m/s
4.Pansystolicjetinatleastoneenvelope
PathologicAR
1.Seenintwoviews