rheumatic fever

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E v i d e n c e - b a s e d ,

b e s t p r a c t i c e N

e w Z

e a l a n d

G u i d e l i n e s f o r R h e u m

a t i c F e v e r for

[ Guidelines ]Rheumatic Fever

New Zealand

Evidence-based, best practice

Guidelines on:

1. Diagnosis, Management and

Secondary Prevention

2. Sore Throat Management

3. Proposed Rheumatic Fever

Primary Prevention Programme

1. Diagnosis, Management

and Secondary Prevention

NHF0239 80pp Cover 4C.indd 2 21/6/06 12:12:39



Endorsed by:



Table of Contents

1. Scope and Prpose o Gideline 5

2. Abot the Gideline 5

Disclaimer 5

Outlineogradingmethodologyused 6

Endorsingorganisations 6

Organisationsconsulted 6

NewZealandguidelines:Writinggroup 7

Otherreviewersandcontributors 8

Secreteriatsupport 8

3. Introdction 9

Keypoints 9

Pathogenesis 9

Epidemiology 9 CosttoNewZealand 11

Populationprojections 11

PreventionoARFandRHD 11

DIAGNOSIS AND MANAGEMENT 13

4. Diagnosis o Acte Rhematic Fever 14

Importanceoaccuratediagnosis 14

Currentapproachestodiagnosis 14

Clinicaleaturesoacuterheumaticever-majormaniestations 16

Clinicaleaturesoacuterheumaticever-minormaniestations 18

EvidenceoaprecedinggroupAstreptococcalinection 19

Otherlesscommonclinicaleatures 19

Echocardiography 19

Dierentialdiagnosis 22

Investigations 23

5. Management o ARF 24

Observationandgeneralhospitalcare 28

Discharge 29

SECONDARY PREVENTION 31

6. Prophylaxis Regimes 32

Penicillin 32

Dose 32 Frequency 32

Secondaryprophylaxiswhilebreasteeding,inpregnancyandwhileonoralcontraceptives 33

Secondaryprophylaxisinanti-coagulatedcases 34

7. Dration o Secondary Prophylaxis 34

8. Protocol or Secondary Prophylaxis Delivery 36

9. Anaphylaxis 37

10.Improving Adherence to Secondary Prophylaxis 37

ReducingthepainoBPGinjections 37

Education 38

ARFRegisters 38

KeydataelementsoARF/RHDregisters 40

Outreachandout-o-town 42

Non-compliance 42

11. Rotine Review and Strctred Care Planning 43

NHF0239 80pp Inside.indd 2 21/6/06 12:11:52



1

Evidence-based, best practice

New Zealand Guidelines for Rheumatic Fever

1. DIAGNOSIS, MANAGEMENT

AND SECONDARY PREVENTION

He korokoro ora he manawa ora,

Mo tatou katoa

( A healthy throat, a healthy heart for us all)

JUNE 2006

NHF0239 80pp Inside.indd 1

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3

12. Prevention o Inective Endocarditis 44

13. Case Finding: Srveillance and Screening 44

Surveillance 44

Screeningorrheumaticheartdisease 45

Suggestedindicatorsorevaluation 46

14. Implementation 47

15. Algorithms 50

Algorithm1.GuideorthediagnosisoARF 50

Algorithm2.GuideortheuseoechocardiographyinARF 52

Algorithm3.Guideorthedurationosecondaryprophylaxis 53

16. Reerences 54

17. Appendices 63

AppendixA:Guidelinedevelopmentprocess 63

AppendixB:JonescriteriaorthediagnosisoARF 64

AppendixC:UseoechocardiographyinARF 66 AppendixD:MedicationsusedinARF 68

AppendixE:Comparisonointramuscularpenicillinandoralpenicillinorsecondaryprevention 70

AppendixF:Anaphylaxisrecognitionandmanagement 71

AppendixG:Protocolorollow-uponon-compliantcases 72

AppendixH:Walletcardorinectiveendocarditisprevention 73

18. Glossary 74

19. Notes 75




List of Tables

Table1. Levelsoevidenceorclinicalinterventionsandgradesorecommendation 6

Table2. NewZealandguidelinesorthediagnosisoARF 15

Table3. MajormaniestationsoARF 16

Table4. MinormaniestationsoARF 18

Table5. UpperlimitsonormalorserumstreptococcalantibodytitresusedinNewZealandorARFdiagnosis 19

Table6. Minimalechocardiographiccriteriatoallowadiagnosisopathologicalvalvularregurgitation 20

Table7. SeverityoARFcarditis 21

Table8. DierentialdiagnosesocommonmajormaniestationsoARF 22

Table9. InvestigationsinsuspectedARF 23

Table10. Prioritiesinmanagingacuterheumaticever 24

Table11. Guidelinesorgeneralin-hospitalcare 28

Table12. Recommendedantibioticregimensorsecondarypreventionoacuterheumaticever/rheumaticheartdisease 33

Table13. NewZealandrecommendationsorthedurationosecondaryprophylaxis 34 Table14. Suggestedprotocolorthedeliveryosecondaryprophylaxisbycommunitynurses 36

Table15. MeasuresthatmayreducethepainobenzathinepenicillinGinjections 37

Table16. PrimaryaimsoARFregistersystems 39

Table17. Recommendedelementsoregister-basedcontrolprogramme 39

Table18. Datasetoracuterheumaticeverregister 40

Table19. RecommendedroutinereviewandmanagementplanorARFandRHD 43

Table20. RecommendedelementsoascreeningprogrammeinNewZealand 45

Table21. ProposedindicatorsorevaluatingARF/RHDcontrolprogrammes 46

Table22. UsesoechocardiographyinARF 66

Table23. DiagnosticandclinicalutilityosubclinicalrheumaticvalvedamageinARF 67

Table24. MedicationsusedinARF 68

Table25. Recommendeddoseoadrenalineinanaphylaxis 71




5

[1. Scope and Purpose of Guideline]

[2. About the Guideline]

Thisguideline has been developedby The National Heart Foundation oNew Zealand and the CardiacSociety oAustralia and

New Zealand. Thisguideline will be complemented by urther guidelineson appropriate sore throat management and primary

preventionoacuterheumaticever(ARF).

Theobjectivesothisguidelineare:

• toidentiyandpresenttheevidenceorbestpracticeinARFdiagnosis

• toidentiythestandardocarethatshouldbeavailabletoallpeopleinNewZealand

• toidentiyareaswherecurrentmanagementstrategiesmaynotbeinlinewithavailableevidence

• toensurethathigh-riskpopulationsreceivethesamestandardocareasthatavailabletoother

NewZealanders.

Thisguidelinewasdevelopedbyawritinggroupcomprisedoexpertsinrheumaticever.SelectedindividualswithexperienceinARF

andrelevantstakeholderswerealsoinvolved.Theseincludedarangeogeneralandspecialistclinicians,alliedhealthproessionals,

Ma-oriandPacicproessionals,andlayrepresentativegroups.

ThisguidelinehasbeenproducedorNewZealandandisendorsedbyNewZealandorganisations.

ThechairsotheguidelinewritingcommitteewereinvolvedinthedevelopmentoasimilardocumentortheAustralianpopulation,withtheunderstandingthattheAustralianguidelineswouldbeadaptedortheNewZealandsetting.Wearegrateulorthecontribution

oourAustraliancolleagues.

Thedevelopmentprocessisdescribedin AppendixA.

Disclaimer

Thisdocument hasbeen producedby TheNationalHeart Foundationo NewZealandand theCardiacSocietyo Australiaand

NewZealandor healthproessionals.The statementsandrecommendationsitcontainsare,unlesslabelledas“expertopinion”,

basedonindependentreviewotheavailableevidence.Interpretationothisdocumentbythosewithoutappropriatehealthtraining

isnotrecommended,otherthanattherequesto,orinconsultationwith,arelevanthealthproessional.

Inaddition,therecommendationsinthisguidelinearenotintendedtoreplaceclinicaljudgmentoeachindividualcase.Treatment

should take into account comorbidities, drug tolerance, liestyle, living circumstances, cultural sensibilities and wishes. When

prescribingmedication,cliniciansshould observeusual contra-indications,be mindulo potentialadverse druginteractionsand

allergies,monitorresponsesandensureregularreview.




Endorsing organisations

• TheCardiacSocietyoAustraliaandNewZealand

• TheNationalHeartFoundationoNewZealand,alongwith:

•TeHotuManawaMa-ori

•PacicIslandsHeartbeat

•PaediatricSocietyoNewZealand

•TheRheumaticFeverTrust.

Organisations consulted

• AustralasianSocietyorInectiousDiseases

• AustralasianFacultyoPublicHealthMedicine

• NationalHeartFoundationoAustralia

• NewZealandNursesOrganisation

• NewZealandMinistryoHealth

• PasikaMedicalAssociationoNewZealand

• RoyalAustralasianCollegeoPhysicians

• TeOhuRataoAotearoa-Ma-oriMedicalPractitionersAssociation.

Richbodyohigh-qualityRCTdata

LimitedbodyoRCTdataorhigh-qualitynon-RCTdata

Noevidenceavailable—panelconsensusjudgment

LEVEL OFEVIDENCE

STuDY DESIGN GRADE OF RECOMMENDATION

I A

II B

III-I B

III-2 B

III-3 C

IV C

D/I

Table 1. Levels o Evidence or Clinical Interventions and Grades o Recommendation

Outline of grading methodology used

Thereviewincludeslevelsoevidenceandaccompanyinggradesorecommendation( Table1).

Evidenceobtainedromasystematicreviewoallrelevantrandomisedcontrolledtrials(RCT)

Evidenceobtainedromatleastoneproperlydesignedrandomisedcontrolledtrial

Evidenceobtainedromwell-designedpseudo-randomisedcontrolledtrials(alternateallocation orsomeothermethod)

Evidenceobtainedromcomparativestudieswith concurrentcontrolsandallocationnotrandomised(cohortstudies),case-controlstudies,orinterruptedtimeserieswithacontrolgroup

Evidenceobtainedromcomparativestudieswithhistoricalcontrol,2ormoresingle-armstudies,orinterruptedtimeserieswithaparallelcontrolgroup

Evidenceobtainedromcaseseries,eitherpost-testorpre-testandpost-test

Insucientevidenceavailable–expertopinionor panelconsensusjudgment

Note: The levels o evidence and grades o recommendations are adapted rom the National Heart Foundation o Australia Rheumatic Fever

guidelines. (Details can be ound at www.nh.com.au)




7

New Zealand guidelines: Writing group

Proessor Diana Lennon (Co-chair)ProessoroPopulationChild&YouthHealth,UniversityoAuckland

Dr Nigel Wilson (Co-chair)

PaediatricCardiologist,StarshipChildren’sHospital

Dr Polly Atatoa-Carr

PublicHealthMedicineRegistrar

Dr Bruce Arroll

AssociateProessoroGeneralPractice,UniversityoAuckland

Ms Elizabeth Farrell

PublicHealthNurse,CountiesManukauDistrictHealthBoard

Dr Jonathan Jarman

MedicalOceroHealth,NorthlandDistrictHealthBoard

Dr Melissa Kerdemelidis

RheumaticFeverTrustResearchFellow

Mr Henare Mason

ProjectManager,CountiesManukauDistrictHealthBoard

Dr Johan Morreau

Paediatrician,RotoruaHospital

Dr Ross Nicholson

Paediatrician,KidzFirstHospital,MiddlemoreHospital

Dr Briar Peat

SeniorLecturerinGeneralMedicine,UniversityoAuckland

Ms Heather Spinetto

SpecialistCardiacNurse,StarshipChildren’sHospital

Dr Lesley Voss

PaediatricianinInectiousDiseases,StarshipChildren’sHospital.




Other Reviewers and Contributors

DrRohanAmeratunga MsEricaAmon DrJeremyArmishaw MsCatherineAtkinson

DrAnitaBell DrCatherineBremner MsJosephineCottrell ProessorBartCurrieDrAlanFarrell DrTomGentles DrDavidGraham MsMichelleHooker

DrDavidJamison ProessorEdwardKaplan DrAndrewKerr MsTraceyKunac

DrGraemeLear MsLindsayLowe MsMaoiteleLowen MrJohnKristiansen

DrChrisMansell DrFraserMaxwell DrMalcolmMcDonald DrMargotMcLean

MsAndreaMockord DrPhilipMoore DrChrisMoyes MsMarthaNgawaka

MsMaureenO’Halloran DrTeuilaPercival DrNeilPoskitt MsKathyRennie

DrJanSinclair DrWarrenSmith MsReneeStreateld DrRichardTalbot

DrCraigThornley MsLupeToilolo DrWendyWalker MsJoannaWilliams

DrElizabethWilson MsIsabelleTeokotaiWhite

AucklandDistrictNursingGroup.

Secretariat Support

MrsShaelynnSchaumkel.

Australian Guidelines Writing Group

DrAlexBrown;AssociateProessorJonathanCarapetis(Chair);DrKeithEdwards;DrCliveHadeld;ProessorDianaLennon;Ms

LynettePurton;DrAndrewTonkin;DrWarrenWalsh;DrGavinWheatonandDrNigelWilson.

Australian Guidelines reviewers and contributors

DrLeslieEBolitho;DrAndrewBoyden;DrChristianBrizard;DrRichardChard;MsEleanorClune;DrArthurCoverdale;DrSophie

Couzos;ProessorBartCurrie;DrJamesEdward;DrTomGentles;ProessorMarciaGeorge;DrJeeryHanna;DrNoelHayman;

DrAnaHerceg;DrMarcusIlton;DrJennierJohns;DrJohnKnight;DrJohnMcBride;DrMalcolmMcDonald;DrJohanMorreau;

DrMichaelNicholson;DrRossNicholson;MsSaraNoonan;DrBriarPeat;DrPeterPohlner;DrJimRamsey;DrJennyReath;

MsEmmaRooney;DrWarrenSmith;DrLesleyVoss;DrMarkWenitong;MrChrisWilson;DrElizabethWilsonandDrKeithWollard.

Declaration

Noconfictsointerestwereinvolvedinthedevelopmentothisguideline.DrPollyAtatoa-Carrwhocoordinatedthewritingothis

guidelinewasundedbyTheNationalHeartFoundationoNewZealandandtheAustralasianFacultyoPublicHealthMedicine.




9

Key points

• Acute rheumatic fever, an auto-immune response to group A streptococcus infection of the upper respiratory tract,

may result in damage to the mitral and/or aortic valves and therefore rheumatic heart disease. Recurrences are likely

in the absence of preventative measures and may cause further cardiac valve damage

• Although acute rheumatic fever is rare in industrialised countries, it is a significant cause of disease among Ma-ori

and Pacific children in New Zealand. The incidence of rheumatic heart disease is also high among these

populations, with significant rates of procedures and death among young adults

• Appropriate treatment of sore throats in high risk populations will eliminate group A streptococcus in most cases,

and prevent individual cases of acute rheumatic fever

• Prevention of recurrences, and therefore rheumatic heart disease prevention, with intramuscular penicillin is both

effective and highly cost-effective.

Acuterheumaticever(ARF)isanauto-immuneconsequenceoinectionwiththebacteriumgroupAstreptococcus(GAS).Itcauses

anacutegeneralisedinfammatoryresponseandanillnessthataectsonlycertainpartsothebody,mainlytheheart,joints,brain

andskin.IndividualswithARFareotenseverelyunwell,ingreatpainandrequirehospitalisation.Despitethedramaticnatureothe

acuteepisode,ARFleavesnolastingdamagetothebrain,jointsorskin.1

However,thedamagetotheheart,ormorespecicallythemitraland/oraorticvalves,mayremainoncetheacuteepisodehas

resolved.Thisisknownasrheumaticheartdisease(RHD).PeoplewhohavehadARFpreviouslyaremuchmorelikelythanthewider

communitytohavesubsequentepisodes.2TheserecurrencesoARFmaycauseurthercardiacvalvedamage.HenceRHDsteadily

worsensinpeoplewhohavemultipleepisodesoARF.

Becauseoitshighprevalenceindevelopingcountries,RHDisthemostcommonormopaediatricheartdiseaseintheworld.In

manycountriesitisthemostcommoncauseocardiacmortalityinchildrenandadultsagedlessthan40years.3

Pathogenesis

ARFhas been shownto developin approximatelyone tothreepercent othose in an epidemicsituationo untreatedexudative

pharyngitisand/oraculturepositiveorGAS.Despitethehighincidenceinsomeethnicgroups(suchasMa-oriandPacicpeoplein

NewZealand),ageneticpredispositiontoARFremainsunproven. 1SomestrainsoGAShavebeenrepeatedlyidentiedascausative

inARF (andthereorelabelled “rheumatogenic”)andotherrheumatogenicstrainscontinueto appear. Theroleoskin inections

remainsuncertain.4,5

FollowingGASinection,thereisalatentperiodaveragingthreeweeksbeorethesymptomsoARFbegin.Bythetimethesymptoms

develop,theinectingstrainoGAShasusuallybeeneradicatedbythehostimmuneresponse.

Epidemiology

Theburden oARF inindustrialised countriesdeclined dramaticallyduring the20thCentury,due mainlyto improvementsin living

standards(andhencereducedtransmissionoGAS)andbetteravailabilityomedicalcare.6,7InmostafuentpopulationsARFisnow

rare.RHDisalsorareinyoungerpeopleinindustrialisedcountries,althoughitisstillseeninsomeelderlypatients,alegacyoARF

halacenturyearlier.

Bycontrast,ARFandRHDremaincommoninmanydevelopingcountries. Arecentreviewo theglobalburdenoGAS-related

diseaseestimatedthatthereisaminimumo15.6millionpeoplewithRHD,another1.9millionwithahistoryoARFbutnocarditis

whostillrequirepreventivetreatment,470,000newcasesoARFeachyearandover230,000deathsduetoRHDannually. 8Almostall

casesanddeathsoccurindevelopingcountries.Theseguresarealllikelytobeunderestimatesothetrueburdenothedisease.

[3. Introduction]

Key points

• Acute rheumatic fever, an auto-immune response to group A streptococcus infection of the upper respiratory tract,

may result in damage to the mitral and/or aortic valves and therefore rheumatic heart disease. Recurrences are likely

in the absence of preventative measures and may cause further cardiac valve damage

• Although acute rheumatic fever is rare in industrialised countries, it is a significant cause of disease among Ma-ori

and Pacific children in New Zealand. The incidence of rheumatic heart disease is also high among these

populations, with significant rates of procedures and death among young adults

• Appropriate treatment of sore throats in high risk populations will eliminate group A streptococcus in most cases,

and prevent individual cases of acute rheumatic fever

• Prevention of recurrences, and therefore rheumatic heart disease prevention, with intramuscular penicillin is both

effective and highly cost-effective.




10

ThereissubstantialregionalvariationintheburdenoARFandRHD.Thehighestdocumentedratesin theworldhavebeenound

inMa-oriandPacicpeopleinNewZealand,AboriginalAustraliansandthoseinPacicIslandnations.9,10,11TheprevalenceoRHDis

alsohighinSub-SaharanArica,LatinAmerica,theIndiansubcontinent,theMiddleEastandNorthernArica.8

NewZealandhashadsustainedhighratesoARFandRHDormanydecadeswithRHDbeingasignicantcauseoprematuredeath

inthiscountry. 12,13,14AnumberosurveysoARFandRHDincidencehavebeenconductedsincetheearly1900sinNewZealand.In

the1920s,surveysoschoolrecordsinNewZealanddeterminedanapproximateannualtotalpopulationincidenceoARFo65per

100,000.9From1956to1973,theWairoaCollegeStudydeterminedthatthedeclineinincidenceoARFseeninotherdeveloped

countrieswas notevident in NewZealand and thosepocketso the country whichexperienced isolationand socio-economic

deprivationhadsignicantlyhigherratesobothARFandRHD.15

From1995to 2000, around 100cases oARF werenotied annually in NewZealand,with an incidenceo 13.8 per100,000

populationin5to14yearolds.14From1993to1999,theAucklandRegisterrecordedanincidenceo21.9per100,000population

in5to14yearolds.Aucklandaccountsor60%otheactivecasesonNewZealandregisters.16,17

ARFispredominantlyadiseaseochildrenagedbetween5to14years,withapeakataroundeightyears.ItisraretodiagnoseARF

undertheageothree(beoreullmaturationotheimmunesystem).18,19AsRHDrepresentsthecumulativeheartdamageoprevious

ARFepisodes,theprevalenceoRHDpeaksinthethirdandourthdecadesolie.20,21Thereore,althoughARFisadiseasewithits

rootsinchildhood,itseectsareeltthroughoutadulthood,especiallyintheyoungadultyearswhenpeoplemightotherwisebeat

theirmostproductive.

The disparity o ethnicity in rheumatic ever populations has been described in many world centers where population groups

experiencinglowsocio-economicstatusandlivinginovercrowdedsituationspresentwithahighincidenceoARF. 19InNewZealand,

Ma-oriandPacicpeopleshavethehighestburdenobothARFandRHD.Despitethesignicantissuesregardingtheaccuracyo

ethnicitydatainpastmorbidityandmortalitystatistics,theratesoARFinMa-orihavealwaysbeenreportedassignicantlygreater

thanthoseseeninnon-Ma-ori.Forexample,rom1949to1953thereportedincidenceoARFinMa-orichildren(ratesogreater

than1000per100,000)was11timesthatothenon-Ma-oripopulation.9Theage-specicannualnoticationratesorARFbetween

1990to1995orchildrenaged10to14yearswas77.7per100,000orPacicchildren,30.4per100,000orMa

-

orichildrenand1per100,000orEuropeanchildren. 14Aucklandalsodisplaysthispattern:theannualincidenceoARFin5to14yearoldMa-ori

childrenrom1993to1999was41.2per100,000population,Pacicchildren83.7per100,000population/yearandtherestothe

population1.4per100,000population/year. 21Dependingontheyearanalysed,thePacichospitalisationratesareatleastninetimes

thatoEuropeans/others.TheMa-orihospitalisationrateisjustovervetimesthatoEuropeans/others.23

As well as higher rates o initial ARF incidence, Ma-oriandPacicpeoplealsohavethehighestratesoARFrecurrence.From

1973to1982(priortotheintroductionosystematicprophylaxisdelivery)recurrenceratesinMa-oriwere40%comparedto22%in

non-Ma-ori.24AreviewocasesintheAucklandrheumaticeverregisterrom1993to1999oundthatalthoughthetotalrecurrence

rateshaddroppedsignicantlyromthe1980s(22%to5.5%),allotherecurrencesoundwereinMa-oriandPacicpeople.16,17Itis

thereorenotsurprisingthatMa-oriandPacicpeoplehavemuchhigherratesocarditis,RHDandconsequentheartailure,asthe

riskothesecomplicationsincreaseswitheachattackoARF.

IthasnotbeenproventhatMa-oriandPacicpeoplehaveincreasedgeneticsusceptibilitytorheumaticever.Itismorelikelythatthe

over-representationothesesectorsothepopulationrefectsacombinationoovercrowdedconditions,socio-economicdeprivation,

anincreasedincidenceoupperrespiratoryinectionswithGAS,anddierenttreatmentoptionsoropportunitiesorappropriateand

eectivehealthcare.11,19,22




11

Cost to New Zealand

There are signicant personal, community and national costs associated with ARF and RHD. These result rom repeated and

prolonged hospitalisation, the resources requiredor medical prophylaxis and treatment,surgicalintervention, negative physicaland psychologicalexperience, disruption o the lives o casesand theiramiliesand oten prematuredeath.25 In 1991,itwas

estimatedthatthetotalcostoARFandRHDtotheAucklandhealthservicealonewas$3.6million,withchronicRHDaccountingor

71%othecosts.CostsinvolvedwerethedirectcostsoGPandoutpatientvisits,prescriptioncharges,travel,radiologyandthe

costsoinormalcaregivenbyhouseholdmembers.20Inadditiontothesedirectcosts,thereareanumberoindirectcostsoARF

andRHD,whichareotendiculttomeasure.Theseincludenotonlythelossoquantityolie(ithasbeenestimatedthatvetoten

youngpeopledieeachyearasadirectresultoARForRHD),butalsothelossoqualityolie.Thisoccursthroughtimeawayrom

educationandoccupation,impactsonphysicaldevelopmentandamilyrelationships,psychologicaleectsandthelossoabilityor

childrenandyoungadultstorealisetheirullpotential.12,20

Population projections

CurrentlyMa-oriandPacicpeopleinNewZealandmakeupasizeablepercentageothechildhoodpopulation.In2001,approximately

37%oMa

-

oriand40%oPacicpeopleinNewZealandwereundertheageo15(comparedto23%European).ThemedianageoEuropeanswas36.8years,whileortheMa-oriandPacicethnicgroupsthecomparablegureswere21.9and21.0yearsrespectively.26

Itis reasonableto predictthattheNew Zealandpopulationin theuturewillrepresenthighgrowthanda sustainedyouthulage

structureintheMa-oriandPacicpopulationswithmany(particularlychildren)livinginpoorsocio-economiccircumstance.26Allthese

eatureshavesignicantimplicationsorARFincidence,prevalenceandprevention.

Prevention of ARF and RHD

Primary prevention

In the uture, a cost-eectivevaccineor group A streptococcimay be the ideal solutionor the primary prevention oARF.27,28

Scienticproblemshavesoarpreventedthedevelopmentosuchavaccine, 28andcurrentlypreventionoaninitialattackoARF

requiresthepromptandaccuratediagnosisandadequateantibiotictreatmentoGASthroatinections.28,29,30ARFcanbepreventedi

theprecedingthroatinectionistreatedinatimelyandeectiveway. 3,31,32Recommendedtreatmentostreptococcalthroatinectionis

intramuscular(IM)benzathinepenicillinoraten-daycourseooralphenoxymethylpenicillin,bothowhicheradicatethestreptococciromthepharynx.Theoraltreatmentisotenusedbecauseitissae,inexpensiveandlesspainul.

Secondary prevention

Overthelast30yearsoneothemajorsuccessesinARFmanagementhasbeenthemarkeddeclineinrecurrent(andotendisabling)

attacksorheumaticever,duetotheavailabilityoeectiveantibioticsorsecondaryprophylaxis.32SecondarypreventionoARFis

denedasthecontinuousadministrationoantibiotics(usuallyparenteralbenzathinepenicillinevery28days)tocaseswithprevious

ARForwell-documentedRHD.28TheaimosecondarypreventionistostoprecolonisationorreinectionothethroatwithgroupA

streptococciandtherebypreventingrecurrenceoARF. 3TheriskoARFatertherstattackogroupAstreptoccociisapproximately

0.3-3%,butwithsubsequentinectionthisriskrisesto25-75%. 2Inaddition,thosewhosuercarditisduringtheirinitialattackare

signicantlymorelikelytodevelopurthercarditiswithsubsequentstreptococcalthroatinections. 33Thesystematicuseoregular

antibioticprophylaxisinknownARFcaseshasbeenshowntoreducetheincidenceorecurrentrheumaticever,reducetheneedor

hospitalisationandsurgery,decreasetherapidityandseverityoRHDandimprovequalityolie. 28,34Furthermore,nationalprevention

programmesbasedonsecondarypreventionhavethepotentialorconsiderablecostsavings,andhavebeenoundtobeacost-eectivemethodoreducingmortalityandmorbidityromARFinternationallyandinNewZealand.20,22,28,35




1




13

Diagnosis and

Management][




1

[4. Diagnosis of Acute Rheumatic Fever (ARF)]Importance of accurate diagnosis

ItisimportantthatanaccuratediagnosisoARFismadeas:

• over-diagnosiswil lresultintheindividualreceivingbenzathinepenicil linG(BPG)injectionsunnecessari ly

everyourweeksoraminimumotenyears

• under-diagnosisoARFmayleadtotheindividualsueringaurtherattackoARF,cardiacdamageand

prematuredeath.

ThediagnosisoARFreliesonhealthproessionalsbeingawareothediagnosticeatures,particularlywhenpresentationisdelayed

oratypical.InAucklandorexample,between1993and1999,ourpatientsdiagnosedwithsepticarthritisbygeneralmedicineand

orthopaedicphysicians,subsequentlydevelopedacuterheumaticever.16,17

Currently,thereisnolaboratorytestdiagnosticorARF,sodiagnosisremainsaclinicaldecision.Thepre-testprobabilityordiagnosisoARFvariesaccordingtolocationandethnicity.Forexample,inaregionwithhighincidenceoARF(suchastheNorthernhalo

theNorthIsland),apersonwitheverandarthritisismorelikelytohaveARFthanoneinalowincidenceregion(suchastheSouth

Island).Ma-oriandPacicpeoplearealsomorelikelythannon-Ma-oriandPacicpeopletohaveARF.

Current approaches to diagnosis

TheJonescriteriaorthediagnosisoARFwereintroducedin1944.36ThecriteriadividetheclinicaleaturesoARFintomajorand

minormaniestations,basedontheirprevalenceandspecicity.Majormaniestationsarethosethatmakethediagnosismorelikely,

whereasminormaniestationsareconsideredtobesuggestive,butinsucientontheirown,oradiagnosisoARF.Theexception

tothisisinthediagnosisorecurrentARF.

TheJones criteriahave beenperiodically modiedand updated. The1992 updateis currentlythe mostwidelyusedand quotedversion.37

ThereareimportantcircumstanceswhereARFcanbediagnosedwithoutstrictlyadheringtotheJonescriteriaandtheseinclude:

• choreaastheonlymaniestationoARF

• indolentcarditis(carditisoinsidiousonsetandslowprogression)astheonlymaniestationoARF.37

Boththesetypesopatientsmayhaveinsucientsupportinghistorical,clinicalorlaboratoryndingstoulltheJonescriteria.

The 1992Jones criteria are intended only or the initial attack oARF. Further discussiono the Jones criteria can be ound in

AppendixB.

EachchangetotheJonescriteriawasmadetoimprovespecicityattheexpenseosensitivity,largelyinresponsetotheallingincidence

oARFinAmerica.Asaresult,thecriteriamaynotbesensitiveenoughtopickupdiseaseinhighincidencepopulations,suchas

Ma-oriandPacicpeople.Insuchpopulations,theconsequencesounder-diagnosisarelikelytobegreaterthanthoseoover-diagnosis.

AllcasesosuspectedARFshouldbejudgedagainstthemostrecentversionotheJonescriteria,butthecriterianeednotberigidly

adheredtowhenARFisthemostlikelydiagnosis.

AnexpertgroupconvenedbytheWorldHealthOrganisation(WHO)hasrecentlyprovidedadditionalguidelinesastohowtheJones

criteriashouldbeappliedinprimaryandrecurrentepisodes.38

The main modication made to the Jones 1992 criteria or these New Zealand guidelines is the acceptance o

echocardiographic evidence o carditis as a major maniestation. In addition there is greater emphasis that monoarthritis

may be a presenting eature i there is a history o non-steroidal anti-infammatory drug (NSAID) use that is likely to have

aborted classical ARF migratory polyarthritis.




15

DIAGNOSTIC REQuIREMENTS

InitialepisodeoARF

InitialepisodeoARF

InitialepisodeoARF

RecurrentattackoARF inacasewithknownpastARForRHD

2majoror1majorand2minormaniestationsplus

evidenceoaprecedingGASinection *

StrongclinicalsuspicionoARF,butinsucientsignsand

symptomstoulldiagnosisodeniteorprobableARF

Minormaniestations(see Table4orkeypointsinidentiyingminormaniestations)

FeverRaisedESRorCRPPolyarthralgia§

ProlongedP-RintervalonECG.

CATEGORY

DeniteARF

ProbableARF

PossibleARF

Categoriesodenite,probableandpossibleARFcanbedeterminedbytheapplicationotheNewZealandcriteriatoeachcase

( Table2).

Table 2. New Zealand Guidelines or the Diagnosis o ARF

All categories assume that other more likely diagnoses have been excluded. Please see additional tables or details about specic maniestations.

CRP = C-reactive protein; ECG = electrocardiogram; ESR = erythrocyte sedimentation rate; GAS = group A streptococcus; RHD = rheumatic heart

disease

*

**

***

#

§

Elevated or rising antistreptolysin O or other streptococcal antibody ( Table 5 ), is sucient or a diagnosis o denite ARF. A positive throat

culture or rapid antigen test or GAS alone is less secure as 50% o those with a positive throat culture will be carriers only. Thereore, a positive

culture alone demotes a case to probable or possible ARF

Most cases o recurrence ull the Jones criteria. However in some cases (such as new carditis on previous RHD) it may not be clear.

Thereore in order to avoid under-diagnosis, a presumptive diagnosis o rheumatic recurrence may be made where there are several minor

maniestations and evidence o a preceding GAS inection in a person with a reliable history o previous ARF or established RHD. In addition,

WHO (2004) recommendations state that where there is established RHD, a recurrent attack can be diagnosed by the presence o two minor

maniestations plus evidence o a preceding group A streptococcal inection 28

Acceptance o echocardiographic evidence o carditis as a major criterion is a modication to the Jones (1992) update

When carditis is present as a major maniestation (clinical and/or echocardiographic), a prolonged P-R interval cannot be considered an

additional minor maniestation in the same person

Other causes o arthritis/arthralgia should be careully excluded, particularly in the case o monoarthritis e.g. septic arthritis

(including disseminated gonococcal inection), inective or reactive arthritis and auto-immune arthropathy (e.g. juvenile

chronic arthritis, infammatory bowel disease, systemic lupus erythematosus, systemic vasculitis and sarcoidosis. Note that i

polyarthritis is present as a major maniestation, polyarthralgia cannot be considered an additional minor maniestation in the

same person.

1majorand2minorwiththeinclusionoevidenceoaprecedingGASinection*asaminormaniestation(Jones,1956)39

2majoror1majorand2minororseveral**minorplus

evidenceoaprecedingGASinection *(Jones,1992)37

Carditis(includingevidenceosubclinicalrheumaticvalvediseaseonechocardiogram)#

Polyarthritis§(orasepticmonoarthritiswithhistoryoNSAIDuse)Chorea(canbestand-aloneorARFdiagnosis)ErythemamarginatumSubcutaneousnodules

Majormaniestationsmodied *** romJones1992(see Table3orkeypointsinidentiyingmajormaniestations)

Special consideration should be given to high-risk population groups such as Ma-ori and Pacic people, and those residing in poor socio-economiccircumstances. In these cases, it may be important to err on the side o diagnosis and prophylaxis.




1

Clinical features of acute rheumatic fever - major manifestations

ThemajormaniestationsoARFandeaturesortheirdiagnosisarepresentedin Table3.

Table 3. Major Maniestations o ARF

Arthritis* •MostcommonpresentingsymptomoARF(occurringinupto75%orstattacks)

•Classiedasswellingothejointinthepresenceotwoormoreotheollowing: limitationomovement,hotnessothejointandpaininthejointand/ortenderness.42Typically,thearthritiso ARFisextremelypainul

•Largejointsareusuallyaected,especiallykneesandankles

•Polyarthritisisusuallyasymmetricalandmigratory(onejointbecominginfamedasanothersubsides)butcanbeadditive(multiplejointsprogressivelybecominginfamedwithoutwarning)

•HighlyresponsivetosalicylateandNSAIDtherapy-usuallyrespondswithin3days

•MonoarthritismaybeapresentingeatureithereisahistoryoNSAIDuseearlyinthecourseotheillness(prematurelyabortingthemaniestationopolyarthritis).**ThisdiagnosisisbestmadebyaphysicianexperiencedinARF

• Thediagnosisoarthritisothehipisacceptedbyhistoryopainprecludingweightbearingand/orlimitationomovementonexamination

•Inordertosatisypolyarthritisasamaniestation,atleastonejointshouldhavebeenobservedinaclinicalsettingaccompaniedbyadenitehistoryoarthritisinotherjoints( GradeD)

Carditis • Valvulitisusuallypresentsclinicallyasanapicalholosystolicmurmurwithorwithoutamid-diastolicfowmurmur(Carey-Coombsmurmur)oranearlydiastolicmurmuratthebaseotheheart(aorticregurgitation)

• Althoughpericarditisandmyocarditismayoccur,cardiacinfammationinARFalmostalwaysaectsthevalves,especiallythemitralandaorticvalves 43,44

•Earlydiseaseleadstovalvularregurgitation,whereasprolongedorrecurrentdiseasemayleadtoincreasedvalvularregurgitationorstenoticlesions 43

• Therheumaticaetiologycanusuallybeconrmedbyatypicalappearanceonechocardiography(see Tables6and7)

•InNewZealand,echocardiographicevidenceosubclinicalcarditiscanalsobeacceptedasamajormaniestation

•CongestiveheartailureinARFresultsromvalvulardysunctionsecondarytovalvulitisandisnotduetoprimarymyocarditis45

•Thenaturalhistoryovalveregurgitationisa25-50%improvementbyoneyear 46

•Ipericarditisispresent,therictionrubmayobscurevalvularmurmurs.

POINTS FOR DIAGNOSIS

MAJOR

MANIFESTATION

Patientswhodonotullthesecriteria,butinwhomtheclinicianremainssuspiciousthatthediagnosismaybeARF,shouldbe

maintainedonoralpenicillinandreviewedintwotoourweekswitharepeatechocardiogramtodetecttheappearanceonew

lesions.40,41Ithereisevidenceorheumaticvalvediseaseclinicallyoronechocardiogram,thediagnosisisconrmedandlong-term

secondaryprophylaxiscanbecommenced.IthereisnoevidenceocarditisandnoalternativediagnosishasbeenoundthenARFmaybethediagnosisbyexclusion.Thosewithepidemiologicalriskactors(Ma-ori,Pacicandlowsocio-economicstatus)shouldbe

commencedonsecondaryprophylaxiswithdueconsiderationoanalternativediagnosis(suchasrheumatological)andtheneedor

ongoingreview.




17

Sydenham’schorea •Consists o jerky, uncoordinated movements, especially aecting the hands, eet, tongue and ace. Themovementsdisappearduringsleep.Theymayaectonesideonly(hemichorea)

•Useulsignsinclude: 47

•the“milkmaid’sgrip”(rhythmicsqueezingwhenthepatientgraspstheexaminer’sngers) •“spooning”(fexionothewristsandextensionothengerswhenthehandsareextended) •the“pronatorsign”(turningoutwardsothearmsandpalmswhenheldabovethehead) •inabilitytomaintainprotrusionothetongue

•Thismaniestationaectsemalespredominantly,particularlyinadolescence 48,49

•Chorea may occur ater a prolonged latent period ollowing GAS inection50,51,52 thereore no additionalmaniestations(includingraisedantibodytitres)arerequiredinordertomakeadiagnosisoARF

•Choreahasastrongassociationwithcarditis,***henceechocardiographyisessentialorassessmentoallpatientswithchorea,regardlessothepresenceocardiacmurmurs( LevelIV,GradeC).Andingosubclinicalcarditisbyechowillurthersupport thediagnosiso choreaasa maniestationo ARF(GradeD).Evenintheabsenceoechocardiographicevidenceocarditis,patientswithchoreashouldbeconsideredatriskosubsequent cardiacdamage.53 Thereore,they shouldall receive secondary prophylaxis, andbe careullyolloweduporsubsequentdevelopmentoRHD

•ChoreaistheARFmaniestationmostlikelytorecurandisotenassociatedwithpregnancyororalcontraceptiveuse.Thevastmajorityocasesresolvewithin6months(usuallywithin6weeks)althoughrarecaseslastingaslongas3yearshavebeendocumented 47

Subcutaneousnodules

•Rare(lessthan2%ocases)buthighlyspecicmaniestationoARF 54

• Theyare0.5-2.0cmindiameter,round,rm,reelymobileandpainlessnodulesthatoccurincropsoupto12overtheelbows,wrists,knees,ankles,Achillestendon,occiputandposteriorspinalprocessesovertebrae

•Tendtoappear1-2weeksatertheonsetoothersymptoms,lastonly1-2weeks(rarelymorethan1month)

•Stronglyassociatedwithcarditis

•Subcutaneous nodulesarerarelyseenas thesolemajor criterionin ARFandshould beaccompaniedbyadditionalmajorcriteriainordertomakethediagnosis

Erythemamarginatum

•Rareaswellasdiculttodetect(especiallyindark-skinnedpeople)

•Occursascircularpatternsobrightpinkmaculesorpapulesthatblanchunderpressureandspreadoutwardsinacircularorserpiginouspatternonthetrunkandproximalextremities(almostneveronace).Therashmaybemoreapparentatershowering

•Notitchyorpainulandnotaectedbyanti-infammatorymedication

•Mayrecurorweeksormonths,despiteresolutionotheothereaturesoARF

•ErythemamarginatumisrarelyseenasthesolemajorcriterioninARFandshouldbeaccompaniedbyadditionalmajorcriteriainordertomakethediagnosis.

ARF should always be considered in the dierentia l diagnosis o patients presenting with arthritis in high-risk populations. In the hospital

setting, physic ians and surgeons should collaborate when the diagnosis o arthritis is unclear. Patients with sterile joint aspirates in the

absence o previous antibiotic exposure should never be treated speculatively or septic arthritis without urther investigation, particularly in

areas with high ARF/RHD prevalence

Note that in New Zealand, NSAIDs are now readily available over the counter and have thereore oten been used prior to presentation

During recent outbreaks o ARF in the USA, up to 71% o patients with chorea had carditis. 55 Even though clinically evident carditis

increases the risk o later development o RHD, prior to cardiac echocardiography approximately 25% o patients with “pure” chorea also

eventually developed RHD.53,56 This is explained by the nding that over 50% o patients with chorea, but without cardiac murmurs, have

echocardiographic evidence o mitral regurgitation.5

*

POINTS FOR DIAGNOSIS

MAJOR

MANIFESTATION

***

**




1

Clinical features of acute rheumatic fever - minor manifestations

TheminormaniestationsoARFandeaturesortheirdiagnosisarepresentedin Table4.

POINTS FOR DIAGNOSISMINOR

MANIFESTATION

Arthralgia •MaysuggestARFithearthralgiaoccursinthesamepatternasrheumaticpolyarthritis(migratory,asymmetricalandaectinglargejoints)

•I polyarthritis is present asa majormaniestation,polyarthralgiacannot beconsidered anadditional minormaniestationinthesameperson

• Alternativediagnoses(assuggestedin Table8)shouldbeconsideredinapatientwitharthralgiathatisnottypicaloARF

Fever •MostmaniestationsoARFareaccompaniedbyever(withtheexceptionochorea)

•InNew Zealand, anoral, tympanic orrectal temperature greater than or equal to38°C onadmission, ordocumentedduringthecurrentillness,shouldbeconsideredasever( LevelIV,GradeC)

•Fever,likearthritisandarthralgia,isusuallyquicklyresponsivetosalicylate/NSAIDtherapy

Elevatedacutephasereactants

•InNewZealand,aserumCRPlevelo ≥30mg/LorESRo≥50mm/hmeetsthisdiagnosticcriterion( GradeD)

• TheESRinARFistypically>80mm/hr,usuallyremainselevatedor>4weeks,andmayremainelevatedor3-6monthsdespiteamuchshorterdurationosymptoms

• TheserumCRPconcentrationrisesmorerapidlythantheESRandalsoallsmorerapidlywithresolutionotheattack

ProlongedP-Rinterval •Anelectrocardiogram(ECG)shouldbeperormedinallcasesosuspectedARF( LevelIV,GradeC)

• TheP-Rintervalincreasesnormallywithagethereoreneedstobeage-adjusted.TheollowingupperlimitsonormalareusedinNewZealand: *

• Age3-12years:0.16seconds • Age12-16years:0.18seconds • Age17+years:0.20seconds

• AprolongedP-Rintervalisoccasionallyanormalvariant,butonethatresolvesovertheensuingdaystoweeksmaybeauseuldiagnosticeatureoARFincaseswheretheclinicaleaturesarenotdenitive. **Inthesecases,arepeatECGater1-2monthsmaybeuseul

•Extremerstdegreeblocksometimesleadstoajunctionalrhythm,usuallywithaheartratesimilartothesinusrate

•Seconddegree,andevencompleteheartblock,canoccurand,iassociatedwithaslowventricularrate,may

givethealseimpressionthatcarditisisnotsignicant

•Intheabsenceoclinicalorechocardiographiccarditis,asecondorthirddegreeblockaccompaniedbyothermaniestationsoARFishighlysupportiveothediagnosis( GradeD)

•Whencarditisispresentasamajormaniestation(clinicaland/orechocardiographic),prolongedP-Rintervalcannotbeconsideredanadditionalminormaniestationinthesameperson.

Table 4. Minor Maniestations o ARF

*

**

Adapted rom Park M K. 58 p42.

In a recent resurgence o ARF in the USA, 32% o patients had abnormal AV conduction, usually a prolonged P-R interval. A small

proportion had more severe conduction abnormalities, which were sometimes ound by auscultation or echocardiography in the

absence o evidence o valvulitis.57




19

Evidence of a preceding group A streptococcal infection

GASareisolatedromthroatswabsinlessthantenpercentoARFcasesinNewZealand5andlessthanvepercentocasesin

AboriginalAustralians.54

ThislatterguremaybearesultolaterpresentationoARF,as28%oAboriginalAustralianshavebeenoundtopresentaschorea59comparedtosixpercentoARFcasesinAuckland(1993-1999). 22,23Apositiveculturewithoutsupportiveantibody

elevationmaybecarriageinupto50%ocases. 37Streptococcalantibodytitresarethereorecrucialinconrmingthediagnosis.The

mostcommonlyusedtestsaretheplasmaantistreptolysinO(ASO)andtheantideoxyribonucleaseB(anti-DNaseB)titres.Theserum

ASOtitrereachesamaximumataboutthreetosixweeksaterinectionandtheserumanti-DNaseBtitrecantakeuptosixtoeight

weekstoreachamaximum.60Therateodeclineotheseantibodiesvariesenormously,withtheASOtitrestartingtoallsixtoeight

weeksandtheanti-DNaseBtitrethreemonthsaterinection.61Intheabsenceoreinection,theASOtitreusuallyapproachespre-

inectionlevelsatersixto12months,whereastheanti-DNaseBtitretendstoremainelevatedorlonger.62Thereerencerangeorthese

antibodytitresvarieswithageandgeographicallocation.Inapopulationwithahighrateostreptococcalinections,manychildrenwill

havehighbackgroundstreptococcaltitres.Theupperlimitonormalapproachattemptstodeterminearaisedtitreoverandabove

thisbackground,andthereoreselectoutthosechildrenwhohavehadarecentstreptococcalinection.63InNewZealand,anASO

titreogreaterthanorequalto480and/oranantiDNaseBtitreogreaterthanorequalto680isacceptedassignicant(GradeD)

Table5.

Established rom residual sera rom children (under 15 years) hospitalised in Auckland in 1982. Lower levels may be acceptable in the

very young or those over the age o 15 years. A two-tube (two-old) rise or all in antibody titres ater 10-14 days would also be diagnostic.

Note that evidence o a preceding GAS inection is not necessary or the diagnosis o chorea as ARF.

TITRE (Iu/ML)

≥480

ANTIBODY TEST

ASO(anti-streptolysinO)

Anti-DNaseB ≥680

AllcasesosuspectedARF(choreaisanexception)shouldhaveelevatedserumstreptococcalserologydemonstrated.Itheinitial

titreisbelowtheupperlimitonormal,testingshouldberepeated10to14dayslater(GradeD).

Other less common clinical features

Theseincludeepistaxis,abdominalpain,rheumaticpneumonia(pulmonaryinltratesinpatientswithacutecarditis),mildelevationso

plasmatransaminaselevelsandmicroscopichaematuria,pyuriaorproteinuria.NoneisspecicorARFbutepistaxisandabdominal

painoccurcommonly.

Echocardiography

Prior to the introduction o echocardiography, the diagnosis o rheumatic carditis relied on clinical evidence o valvulitis orpericarditis,supportedbyradiographicevidenceocardiomegaly.Today,allpatientswithsuspectedordeniteARFshouldundergo

echocardiography,ipossible,toidentiyevidenceocarditis(GradeC).

InNewZealand,echocardiologyacilitiesarereadilyavailableinthelargercentersorpopulationsathigh-riskoARF.Theuseo

echocardiography asa majorcriterionor ARFdiagnosisrequires expert interpretationadhering toechocardiographicdiagnostic

standards.ThesestandardsconcurwithrecentWHOechocardiographiccriteriaorARFandaresummarisedin Table6(LevelIV).

Thesecriteriacanreadilydistinguishasmallcolourjetophysiologicalregurgitationinanormalchildrompathologicalregurgitation

inachildwithRHD.

Table 5. Upper Limits o Normal or Serum Streptococcal Antibody Titres Used in New Zealand or ARF Diagnosis




0

Table 6. Minimal Echocardiographic Criteria to Allow a Diagnosis o Pathological Valvular Regurgitation

Echocardiography allows the operator to comment on the appearance o valves that are aected by rheumatic infammation. The degree

o thickening gives some insight into the duration o valvulitis, no signicant thickening occurs in the rst weeks o acute rheumatic carditis

( Level IV )

Only ater several months is immobility o the subchordal apparatus and posterior leafet observed. Several other ndings have also been

reported, including acute nodules, seen as a beaded appearance o the mitral valve leafets.64 Although none o these morphological

eatures is unique to ARF, the experienced echocardiographic operator can use their presence as supportive evidence o a rheumatic

aetiology o valvulitis.

InNewZealand,ARFcarditisisclassiedmild,moderateorsevere( Table7)andthesecategoriesareusedtoguidethedurationo

secondaryprophylaxis(seeSection7and Table13).

MITRAL REGuRGITATION

•Colour:

Substantialcolourjetseenin2planesextendinggreaterthanorequalto2cmbeyondthevalveleafets

•ContinuouswaveorpulsedDoppler:

Holosystolicwithwell-denedhighvelocityspectralenvelope

•Bothmitralandaorticvalveshavepathologicalregurgitation

•Themitralregurgitantjetisdirectedposteriorly,asanteriormitralvalveprolapseismorecommonthanposteriorvalveprolapse

•Multiplejetsomitralregurgitation

•ThepresenceomorphologicaloranatomicalchangesconsistentwithRHD(seetext),butexcludingslightthickeningovalveleafets:

•Denitethickeningomitralvalveleafets,indicativeochronicRHD*

•Elbowordoglegdeormity**oanteriormitralvalveleafet.

Itheaetiologyo aorticor mitralregurgitationonDoppler echocardiographyis notclear, theollowing eaturessupport adiagnosisorheumaticvalvedamage:

AORTIC REGuRGITATION

*

**

•Colour:

Substantialcolourjetseenin2planesextendinggreaterthanorequalto1cmbeyondthevalveleafets

•ContinuouswaveorpulsedDoppler:

Holodiastolicwithwell-denedhighvelocityspectralenvelope

Source: Adapted with permission rom Wilson N J. & Neutze J M.65 These criteria urther evolved as part o the development o the Australian guidelines

on rheumatic ever diagnosis and the WHO working groups on echocardiography.66




1

MODERATE CARDITIS

• Anyvalvelesionomoderateseverityclinically(e.g.mildormoderatecardiomegaly),or

•Anyechocardiographicevidenceocardiacchamberenlargement or

• Anymoderateseverityvalvelesiononecho**

•Mitralregurgitationisconsideredmoderateithereisabroadhigh-intensityproximaljetllinghaltheletatriumora

lesservolumehigh-intensityjetproducingprominentbluntingopulmonaryvenousinfow41

•Aorticregurgitationisconsideredmoderateithediameterotheregurgitantjetis15%to30%othediameterothe letventricularoutfowtractwithfowreversalinupperdescendingaorta41

SEVERE CARDITIS

•AnyimpendingorpreviouscardiacsurgeryorRHD, or

•Anyseverevalvelesionclinically(signicantcardiomegalyexpected,and/orheartailure), or

•Anyseverevalvelesiononecho:

•AbnormalregurgitantcolourandDopplerfowpatternsinpulmonaryveinsareaprerequisiteorseveremitral

regurgitation41

•Dopplerreversalinlowerdescendingaortaisrequiredorsevereaorticregurgitation.41

Valvular regurgitation is usually relatively mild in the absence o pre-existing disease; in rst episodes o ARF, severe mitral and aortic

regurgitation occurred in less than 10% o patients in New Zealand 41

When there is both mitral and aortic regurgitation, one must be moderate by echo criteria in order or the carditis to be classied

o moderate severi ty.

Ivalvulitisisnotoundatpresentation,itmayappearwithintwoweeks,40oroccasionallywithinonemonth41butnolonger.ThusanequivocalinitialechocardiographshouldbeollowedupintwotoourweeksithendingswouldalterthediagnosisoARF.

Usuallyitisnotpossibletocondentlydistinguishbetweenacutecarditisandpre-existingrheumaticvalvediseasebyechocardiography.

InapatientwithknownpreviousRHD,thediagnosisoacutecarditisduringarecurrenceoARFreliesonaccuratedocumentation

othecardiacndingsbeoretherecurrence,sothatnewclinicalorechocardiographiceaturescanbeconrmed.But,inapatient

withnopriorhistoryoARForRHD,itmakeslittledierencewhetherechocardiographicchangesareneworold.

FurtherdetailsontheuseoechoinARFcanbeoundin AppendixC.

MILD CARDITIS*

•Mildmitraloraorticregurgitationclinicallyand/oronecho(ulllingtheminimalechostandardsin Table6)withnoclinicalevidence oheartailureandnoevidenceocardiacchamberenlargementonCXR,ECGorechocardiography

Table 7. Severity o ARF Carditis

*

**

Tricuspid and pulmonary regurgitation graded mild or greater may be seen in people with normal hearts who have ever, volume overload or pulmonary

hypertension. For this reason a diagnosis o carditis should not be based on right-side regurgitation alone. Although pulmonary and tricuspid

regurgitation are oten seen in association with le t-sided lesions in ARF, pressure and volume overload must be excluded beore attributing even

moderate tricuspid regurgitation to valvuli tis. I both let and right-sided lesions coexist in ARF carditis, then the predominant infuence or diagnosis

is the severity o the let-sided lesion.




Table 8. Dierential Diagnoses o Common Major Maniestations o ARF

Source: Adapted rom Lennon D. 2004,32 and Carapetis J et al. 2005.67

POLYARTHRITIS AND FEVER

•Otherinections*(includinggonococcal)

•Connectivetissueandotherauto-immunedisease **

•Reactivearthropathy

•Sicklecellanaemia

•Inectiveendocarditis

•Leukaemiaorlymphoma

•Goutandpseudogout

•Henoch-Schonleinpurpura

•Post-streptococcalreactivearthritis ***

•Other,e.g.HIV/AIDS,leukaemia

CARDITIS CHOREA

D I F F E R E N T I A L D I A G N O

S E S

•Innocentmurmur

•Mitralvalveprolapse

•Congenitalheartdisease

•Inectiveendocarditis

•Hypertrophiccardiomyopathy

•Myocarditis—viraloridiopathic

•Pericarditis—viraloridiopathic

•Systemiclupuserythematosus

•Drugingestion(extrapyramidalsyndrome) #

•Wilson’sdisease(usuallyadultonset)

•Ticdisorder§

•Congenital,e.g.hyperbilirubinaemia

•Choreoathetoidcerebralpalsy

•Encephalitis

•Familialchorea(includingHuntington’s)

•Intracranialtumour

•Hormonal‡

•Metabolic,e.g.Lesch-Nyhan, hyperalanaemia,ataxia,telangiectasia

•Antiphospholipidantibody

Differential diagnosis

ManyotheclinicaleaturesoARFarenon-specic,soawiderangeodierentialdiagnosesshouldbeconsideredasshownin

Table8.32,67

Includes bacterial arthritis (e.g. Staphylococcus aureus, Neisseria gonorrhea), infuenza b, cytomegalovirus, Epstein-Barr Virus, mycoplasma,

rubella (also post-vaccination), hepatitis B, parvovirus, Yersinia spp and other gastrointestinal pathogens

Includes rheumatoid arthritis, juvenile chronic arthritis, infammatory bowel disease, systemic lupus erythematosus, systemic vasculitis,

sarcoidosis and others

Some patients present with arthritis not typical o ARF, but with evidence o recent streptococcal inection and are said to have post-

streptococcal reactive arthritis. In these cases the arthritis may aect joints that are not commonly aected in ARF (such as the small joints

o the hand), and is less responsive to anti-infammatory treatment. These patients are said not to be at risk o carditis, and thereore do not

require secondary prophylaxis. However, some patients diagnosed with post-streptococcal reactive arthritis have developed later episodes

o ARF, indicating that the initial diagnosis should have been atypical ARF ( Level IV )68,69

It is recommended that the diagnosis o post-streptococcal reactive arthritis should rarely, i ever, be made in high-risk populations, and

with caution in low-risk populations ( Grade C ). Patients so diagnosed should receive secondary prophylaxis or at least 5 years ( Grade D ).

Echocardiography (see algorithm 2) should be used to conrm the absence o valvular damage in all o these cases beore discontinuing secondary prophylaxis ( Grade D )

Drugs and toxins include anticonvulsants, antidepressants, lithium, scopolamine, calcium channel blockers, methylphenidate, theophylline

and antihistamines

Some cases o chorea are mild or atypical and may be conused with motor tics or the involuntary jerks o Tourette’s syndrome. There may

thereore be conusion between Sydenham’s chorea and these conditions. The term PANDAS (Pediatric Auto-immune Neuropsychiatric

Disorder Associated with Streptococcal inection) reers to a subgroup o children with tic or obsessive-compulsive disorders (OCD), whose

symptoms may develop or worsen ollowing GAS inection.

Five criteria have been used to dene the PANDAS subgroup: 70,71

• The presence o a Tic disorder and/or OCD• Pre-pubertal age o onset (usually between 3 and 12 years o age)• Abrupt symptom onset and/or episodic course o symptom severity • Temporal association between symptom exacerbations and streptococcal inection (approx 7-14 days)• Presence o neurologic abnormalities during periods o symptom exacerbation (typically adventitious movements

or motoric hyperactivity)

*

**

***

#

§




3

Investigations

Therecommended investigationsin ARFare listedin Table9. Otherinvestigationsmaybe appropriatedependingontheclinical

pictureandpotentialdierentialdiagnoses.

•Whitebloodcellcount

•Erythrocytesedimentationrate(repeatweeklyoncediagnosisconrmed)

•C-reactiveprotein

•Bloodculturesiebrile

•Electrocardiogram(repeatasnecessaryiconductionabnormalitymorethanrstdegree)

•Chestx-rayiclinicalorechocardiographicevidenceocarditis

•Echocardiogram(repeatasnecessaryin2-4weeksiequivocaloriseriouscarditis)

•Throatswab(preerablybeoregivingantibiotics)—cultureorgroupAstreptococcus

•Anti-streptococcalserology:bothanti-streptolysinOandanti-DNaseBtitres,iavailable(repeat10-14dayslateri1sttestnot conrmatory)

•Serologyandauto-immunemarkersorauto-immuneorreactivearthritis(includingANA-AntiNuclearAntibody)

•Repeatedbloodculturesipossibleendocarditisorsepticarthritis

•Jointaspirate(microscopyandculture)orpossiblesepticarthritis *

•JointX-ray

•Copper,caeruloplasmin,anti-nuclearantibody,drugscreen,andconsiderCT/MRIheadorchoreiormmovements. **

RECOMMENDED FOR ALL CASES

TESTS FOR ALTERNATIVE DIAGNOSES, DEPENDING ON CLINICAL FEATuRES

Typically, the synovial fuid in joints aected by ARF contains 10,000 to 100,000 white blood cells/mm 3 (predominantly neutrophils). The

protein concentration is approximately 4g/dL, glucose levels are normal, gram stain negative and a good mucin clot is present 72

The chorea o ARF can be readily diagnosed on the basis o history, physical examination and laboratory evaluation. Neuroimaging is

seldom necessary and should be reserved or cases who have an atypical presentation such as hemichorea. 73

Table 9. Investigations in Suspected ARF

However, the evidence supporting PANDAS as a distinct disease entity has been questioned.71 Hence, in New Zealand populations with a

high prevalence o ARF, clinicians should rarely (i ever) make a diagnosis o PANDAS, and should rather err on the side o over-diagnosis

o ARF and secondary prophylaxis ( Grade D ). I ARF is excluded, secondary prophylaxis is not needed, but such cases should be careully

ollowed up to ensure that they do not develop carditis in the long term

‡ Includes oral contraceptives, pregnancy (chorea gravidarum), hyperthyroidism and hypoparathyroidism.

*

**




Ideally,allthosewithsuspectedARF(rstepisodeorrecurrence)shouldbehospitalisedassoonaspossibleateronsetosymptoms(GradeD).Thisensuresthatallinvestigationsareperormedand,inecessary,observationscompletedoraperiodpriortocommencingtreatmenttoconrmthediagnosis.Hospitalisationalsoprovidesanidealopportunityoreducation

Observationpriortoanti-infammatorytreatment(paracetamol[1stline]oreverorjointpain)

Investigations(asper Table9)

TREATMENT

All cases

CONFIRMATION OF THE DIAGNOSIS

ADMISSION TO HOSPITAL*

[5. Management of ARF]

Antibiotics**

•OralpenicillinV(250mgtwicedaily)shouldbecommencedinallcaseswhilethediagnosisisbeingestablished.Toreliablyeradicate GAS,oralpenicillinshouldbegivenortheull10days

•Oralerythromycinusedincaseswithreliablydocumentedpenicillinallergy***(10daysoerythromycinethylsuccinate(EES)40mg/kg perdayin2-4divideddoses,maximum1g/dayinchildrenor400mgtwicedailyinadolescentsandadults).76EESiscurrentlythe onlyullysubsidisedoralerythromycininNewZealand

•Intravenousantibioticsarenotindicated.Roxithromycinisnotrecommendedbecauseothelimitedavailableevidencethatitisnot aseectiveaserythromycinineradicatingGASromtheupperrespiratorytract77

• TherstdoseointramuscularbenzathinepenicillinG(BPG1,200,000Uor600,000Uilessthan20kg)shouldalsobegiveninhospitalinassociationwitheducationabouttheimportanceosecondaryprophylaxis.OncetherstdoseoBPGisgiven,theoralpenicillinisstopped

Arthritis/arthralgia

Salicylates/NSAIDS

•ThearthritisoARFhasbeenshownincontrolledtrialstoresponddramaticallytosalicylatesandhasalsobeennotedtorespondto otherNSAIDtherapy,78,79,80otenwithinhoursandalmostalwayswithin3days (LevelII)

•SalicylatesarerecommendedasrstlinetreatmentbecauseotheextensiveexperiencewiththeiruseinARF.38,81,82Theyshould becommencedincaseswitharthritisorseverearthralgiaassoonasthediagnosisoARFhasbeenconrmed(GradeB),butthey shouldbewithheldithediagnosisisnotcertain.Insuchcases,paracetamolorcodeineshouldbeusedinsteadorpainrelie

•Aspirinshouldbestartedatadoseo60-100mg/kg/day(4-8g/dayinadults)in4-5divideddoses.Ithereisanincompleteresponse within2 weeks,the dose may beincreasedto125mg/kg/day, butwiththesehigher dosescareul observation oreatureso salicylatetoxicityisadvised.Iacilitiesareavailable,bloodlevelsmaybemonitoredeveryewdays,andthedoseincreaseduntil serumlevelso20-30mg/100dLarereached.Toxiceects(tinnitus,headache,hyperpnoea)arelikelyabove20mg/100dLbutoten resolveateraewdays

•Mostcasesrequire10daysorlessoaspirintherapyandthereorebloodlevelmonitoringisseldomnecessary.Manyneedaspirin oronly1-2weeks,althoughsomeneeditorupto6weeks.Insuchcases,thedosecanotenbereducedto60-70mg/kg/day

Themajorpriorityinthe rstewdaysaterpresentationinARFisconrmationothediagnosis.Exceptinthe caseoheartailuremanagement,noneothetreatmentsoeredtocaseswithARFhavebeenproventoaltertheoutcomeotheacuteepisodeorthe

amountodamagetoheartvalves. 74,75Thus,thereisnourgencytobegindenitivetreatment.TheprioritiesinmanagingARFare

outlinedin Table10.

Table 10. Priorities in Managing Acute Rheumatic Fever




5

TREATMENT CONTINuED

Activity Arthritis

alone

Mild carditis Moderate carditis Severe carditis

In hospital 1-2weeks 2-3weeks 4-6weeks 2-4months

Mobilisereelyastolerated

House arrest(activity and

school work

at home)

1-2weeksaterdischarge

2-3weeks 4-6weeks 2-4months

School 2weeks 2-4weeks 1-3months 2-3months

Gradualreturntoullactivity Avoidsportandphysicaleducation

Full activity

(sport)

Ater6weeks

Ater3months Ater3-6months Variable

Urgentechocardiogram

Anurgentechocardiogramandcardiologyassessmentarerecommendedorallcaseswithheartailure

atertheinitial1-2weeks.32Asthedoseisreduced,orwithin3weeksodiscontinuingaspirin,jointsymptomsmayrecur.Thisdoes notindicaterecurrence,andcanbetreatedwith anotherbriecourseo high-doseaspirin. MostARFepisodessubsidewithin 6 weeks,and90%resolvewithin12weeks.Approximately5%ocasesrequire6monthsormoreosalicylatetherapy83

• ThereisalsotheriskoReye’ssyndromeinchildrenreceivingsalicylateswhodevelopcertainviralinections,particularlyinfuenza.Itisrecommendedthatchildrenreceivingaspirinduringtheinfuenzaseason(autumn/winter)alsoreceiveaninfuenzavaccine( GradeD)

•Naproxen hasbeen used(10-20mg/kg/day) successullyin thosewith ARF, including onesmall randomisedtrial,and hasbeen advocatedasasaeralternativetoaspirin(LevelIII-I).84,85Ithastheadvantageoonlytwice-dailydosing,lesshepatotoxicity,anditis alsoavailableinanelixiror youngchildren. Theexperiencewiththismedicationislimited,sotherecommendationcurrentlyisto restrictittothoseintoleranttoaspirin,ortouseitasastep-downtreatmentoncecasesaredischargedromhospital(GradeD)

Paracetamol

•Mildarthralgiaandevermayrespondtoparacetamolalone

Fever

Low-gradeeverdoesnotrequirespecictreatment.Feverwillusuallyresponddramaticallytosalicylatetherapy.Feveralone,oreverwithmildarthralgiaorarthritis,maynotrequiresalicylates,butcaninsteadbetreatedwithparacetamol

Carditis/heart ailure

Bedrest

Inthepre-penicillinera,prolongedbedrestinthosewithrheumaticcarditiswasassociatedwithshorterdurationocarditis,ewerrelapsesandlesscardiomegaly.86Ambulationshouldbegradualandastoleratedincaseswithheartailure,orsevereacutevalvedisease,especiallyduringtherst4weeks,oruntiltheserumCRPlevelhasnormalisedandtheESRhasnormalisedordramatically

reduced.Thosewithmilderornocarditisshouldremaininbedonlyaslongasnecessarytomanageothersymptoms,suchasjointpain(GradeD).

Aguideoractivitylevelsisshownbelow(AdaptedromLennonD.200432)(GradeD).




Anti-ailuremedication

•Diuretics/fuidrestrictionormild-moderateailure

•ACEinhibitorsormoresevereailure,particularlyiaorticregurgitationpresent

•Glucocorticoids§optionalorseverecarditis 74

•Digoxiniatrialbrillationpresent

•Thereislittleexperiencewithbeta-blockersinheartailureduetoacutecarditis,andtheiruseisnotrecommended (GradeD)

Detailed recommendations orthe management o heart ailure can be ound in a separate Heart Foundation clinical guideline(availableathttp://www.nzgg.org.nz)

Valvesurgery

Surgeryisusuallydeerreduntilactiveinfammationhassubsided.Rarely,valveleafetorchordaetendinaeruptureleadstosevere

regurgitationwhichrequiresemergencysurgery.Thiscanbesaelyperormedbyexperiencedsurgeons,althoughtheriskappearstobeslightlyhigherthanwhensurgeryisperormedateractiveinfammationhasresolved. 87Valvereplacement,ratherthanrepair,isusuallyperormedduringtheacuteepisode,becauseothetechnicaldicultiesorepairingriable,infamedtissue.Nevertheless,veryexperiencedsurgeonsmayachievegoodresultswithrepairinthissituation

Chorea

Sydenham’schoreaissel-limited.Mostcaseswillresolvewithinweeksandalmostallcaseswithin6months,88althoughrarecasesmaylastaslongas2-3years. 59,89,90Mildormoderatechoreadoesnotrequireanyspecictreatment,asideromrestandacalmenvironment.Over-stimulationorstresscanexacerbatethesymptoms.Sometimeshospitalisationisuseultoreducethestressthatamiliesaceindealingwithabnormalmovementsandemotionallability

Becausechoreaisbenignandsel-limiting,andanti-choreamedicationsarepotentiallytoxic,treatmentshouldonlybeconsideredithemovementsintereresubstantiallywithnormalactivities,placethepersonatriskoinjuryorareextremelydistressingtothepatient,amilyandriends.Aspirinandglucocorticoidtherapydonothaveasignicanteectonrheumaticchorea 47

Smallstudiesointravenousimmunoglobulin(IVIG)havesuggestedmorerapidrecoveryromchorea,buthavenotdemonstratedreducedincidenceolong-termvalvediseaseinnon-choreaARF.41,91Untilmoreevidenceisavailable,IVIGisnotrecommended,exceptorseverechoreareractorytoothertreatments( LevelII/IV,GradeC)

Carbamazepine‡andvalproicacid+arenowpreerredtohaloperidol,whichwaspreviouslyconsideredtherst-linemedicaltreatmentorchorea.92,93Asmall,prospectivecomparisonothese3agentsrecentlyconcludedthatvalproicacidwasthemosteective 94

Otheranti-choreamedicationsshouldbeavoidedbecauseopotentialtoxicity.Becauseothesmallpotentialorlivertoxicitywithvalproicacid,itisrecommendedthatcarbamazepinebeusedinitiallyorseverechorearequiringtreatment,andthatvalproicacidbeconsideredorreractorycases(LevelIII2,GradeB).Aresponsemaynotbeseenor1-2weeks,andsuccessulmedicationmayonlyreduce,butnoteliminate,thesymptoms.Medicationshouldbecontinuedor2-4weeksaterchoreahassubsidedandthenwithdrawn.Recurrencesochoreaareusuallymildandcanbemanagedconservativelybut,insevererecurrences,themedicationcanbere-startedinecessary

CLINICAL FOLLOW-uP

•Allcasesshouldreceiveregularreviewandoutpatientollow-upshouldbeinitiatedpriortodischarge

• Therequencyanddurationoreviewisdependentontheindividualclinicalneedsandlocalcapacityandshouldbecomemorerequentintheeventosymptomonset,symptomaticdeteriorationorachangeinclinicalndings

•Particularcareshouldbetakenwhencasesaretranserredrompaediatrictoadultservices.Acasecanbemadeormaintaininglessseverecasesinthepaediatricservicesuntildischargeatage21inordertoensurecontinuityoollow-up

•Jointcardiologyandgeneralpaediatric/physicianmanagementorcaseswithseverecarditisarerecommended

•Furtherinormationregardingrequencyandnatureoroutinereviewcanbeoundin Section11




7

COMMENCEMENT OF LONG-TERM PREVENTIVE MEASuRES

*

**

***

#

Secondaryprophylaxis

•Obtainconsentromcaregiver/caseorIMpenicillintreatment

•Firstdoseosecondaryprophylaxisshouldbedeliveredinhospital

Notication

•CaseshouldbenotiedtoalocalARFregisteriavailable(see Section10.3).Thereshouldbeaneasymeansto dothis,viaa standardnoticationorm,telephonecallorotherwise

•Inaddition,asARFisanotiablediseaseinNewZealand,eachcaseshouldbenotiedtothelocalpublichealthunitornational inectiousdiseasesurveillance

Contactcommunityservicestoensureollow-up

•Theregistercoordinator(iavailable)shouldnotiycommunityhealthstaaboutARFcasesintheirarea.Thenotiyingmedical practitionershouldalsomakedirectcontactwiththoseinthecommunityresponsibleorprophylaxisdeliveryinordertoensurethat theyareawareothediagnosis,theneedorsecondaryprophylaxisandanyotherspecicollow-uprequirements.Thismayinclude districtnurses,publichealthnurses,medicalocerohealthandotherpublichealthsta

•Acommunitynurseand/orcommunityhealthworkerortheareawherethecaseresidesshouldalsodoawardand/oramilyvisiti possiblebeoredischarge

•Whererelevant,itisalsoimportantorconsenttobeobtainedromthecase(orcaregiver)ortheirlocalMa -oriorPacicproviderto knowabouttheillness

Education

•Atthetimeodiagnosis,itisessentialthatthediseaseprocessbeexplainedtothepatientandtheiramilyinaculturallyappropriate way,usingavailableeducationalmaterialsandinteractivediscussion.Furthereducation,usingculturallyappropriateeducational materialsshouldollowoncethecasehasreturnedhome

•Forurtherinormationregardingeducationsee Section10.2

Organisedentalcheckandongoingdentalcare

•Thisiscriticalinthepreventionoendocarditis.Asthosewithoutrheumaticvalvedamagemaystillbeatlong-termriskodeveloping RHD,particularlyintheeventorecurrentepisodesoARF,dentalcareisessential,regardlessothepresenceorabsenceocarditis

•Eachcaseshouldbenotiedtotheappropriateschooldentalserviceordentist

Contactmanagement

•Allsymptomaticandasymptomatichouseholdcontactsotheindexcaseaged3yearsandoldershouldhaveathroatswabithe contactwasnolongerthanonemonthbeoretheonsetoARFintheindexcase.Thisshouldbeorganisedthroughtheappropriate publichealthunitandallcontactswithpositiveGASculturesshouldbeoeredantibiotictreatment.Streptococcalacquisitionrates o25%orgreaterhavebeenrecordedinamilycontactsostreptococcalpharyngitis78,95,96

Opportunisticcare

•ItisimportanttonotethisopportunitytoprovideinormationandotherservicesorARFcases,whomrequentlyhaveotherchallenges totheirgeneralwellbeing. This may include promotinga healthy diet,exerciseandhygiene,as well asassistancewithsocio- economicstressors,andtheopportunityorongoingsupport.

Occasionally, when the diagnosis has already been confrmed and the case is not unwell (e.g. mild recurrent chorea in a child with no other

symptoms or signs), outpatient management may be appropriate. In such cases health sta must ensure that investigations, treatment,

health education, registration (where available) and notifcation are all completed and prophylaxis commenced

Controlled studies have ailed to show that treating ARF with large doses o penicillin aects the outcome o rheumatic valvular lesions 1

year later.97,98 Despite this, most authorities recommend a course o penicillin, even i throat cultures are negative, to ensure eradication o

streptococci that may persist in the upper respiratory tract ( Grade D )

Most people labelled as being allergic to penicillin are not. Because penicillin is the best antibiotic choice or secondary prophylaxis it is

recommended that those with stated penicillin allergy be investigated careully, preerably with the help o an allergist, beore being accepted

as truly allergic ( Grade D ) ( Section 6 )

I the symptoms and signs do not remit substantially within 3 days o commencing anti-infammatory medications, a diagnosis other than

ARF should be considered




The use o glucocorticoids and other anti-infammatory medications in rheumatic carditis has been studied in two meta-analyses.74,75 All

o these studies o glucocorticoids were perormed more than 40 years ago, and did not use drugs in common use today. These meta-

analyses ailed to suggest any benet o glucocorticoids or IVIG over placebo, or o glucocorticoids over salicylates, in reducing the risk o

long-term heart disease ( Level I ). The available evidence suggests that salicylates do not decrease the incidence o residual RHD ( Level

IV ).78,79,80 Thereore, salicylates are not recommended to treat carditis ( Grade C ). Glucocorticoids may be considered or those with heart

ailure in whom acute cardiac surgery is not indicated ( Grade D ). This recommendation is not supported by evidence, but is made because

many clinicians believe that glucocorticoids may lead to more rapid resolution o cardiac compromise, and even be lie-saving in severe

acute carditis.75,99 The potential major adverse eects o short courses o glucocorticoids, including gastrointestinal bleeding and worsening

o heart ailure as a result o fuid retention, should be considered beore they are used. I glucocorticoids are used, the drug o choice is

oral prednisone or prednisolone (1-2mg/kg/day, to a maximum o 80mg once daily or in divided doses). Intravenous methyl prednisolone

may be given in very severe cases. I a week or less o treatment is required, the medication can be ceased when heart ailure is controlled,

and infammatory markers are improving. For longer courses (usually no more than 3 weeks is required), the dose may be decreased by

20-25% each week. Treatment should be given in addition to the other anti-ailure treatments outlined below. Mild to moderate carditis

does not warrant any specic treatment. As glucocorticoids will control joint pain and ever, salicylates can usual ly be discontinued, or the

dose reduced, during glucocorticoid administration. Salicylates may need to be recommenced ater glucocorticoids are discontinued to

avoid rebound joint symptoms or ever

Side eects o carbamazepine include CNS adverse reactions (dizziness, headache, ataxia, drowsiness, atigue and diplopia); gastrointestinal

disturbances (nausea and vomiting), as well as allergic skin reactions. Uncommon side eects include abnormal involuntary movements

(e.g. tremor, asterixis, dystonia and tics) and nystagmus. Rarely carbemazapine can cause oroacial dyskinesia, oculomotor disturbances,

speech disorders (e.g. dysarthria and slurred speech), choreoathetotic disorders, peripheral neuritis, paresthesia, muscle weakness and

paretic symptoms100

Side eects o valproic acid include pancreatitis, hepatic toxicity, hyperammonaemia and thrombocytopaenia.100

Observation and general hospital care

Guidelinesorgeneralin-hospitalcareareprovidedin Table11(GradeD).

Table 11. Guidelines For General In-Hospital Care

NuRSING RECORDINGS

•Temperature,pulse,RR,BP4timesdaily•Sleepingpulse(e.g.0200hrs)•Ipulse>100bpm,recordapicalHR

DIET

•Freefuids(inoheartailure)•Normaldiet(limitextras)•Earlydietaryadviceioverweightandinailure,toavoidurtherweightgain•Weeklyweight

BED REST AND GENERAL CARE

•Examinedailyorthepatternoarthritisandthepresenceoheartmurmur,choreiormmovements,skinrashandsubcutaneous nodules•Iclinicalcarditispresent: •Documentcardiacsymptomsandsigns •Dailyweightandfuidbalancechart •Medicationsasappropriate(see Table10and AppendixD) •Seegeneralguidelinesorbedrest( Table10) •Cardiologyopinion•Repeatinvestigationsasnecessary•Provideculturalsupport(asrelevant)•Plancaretoproviderestperiods•Provideage-appropriateactivities•Notiyschoolteacher

•Involveamilyincare.

§

‡

+

Source: Adapted rom Lennon D. 2004.32

Note: RR = respiratory rate; BP = blood pressure; HR = heart rate.




9

Discharge

Timing of discharge

Thedurationotreatmentisdictatedbytheclinicalresponseandimprovementininfammatorymarkers(ESRandCRP).MostcasesoARFwithoutseverecarditiscanbedischargedromhospitalaterapproximatelytwoweeks.Thelengthoadmissionwillpartly

dependonthesocialandhomecircumstances. Icasescomeromremotecommunitiesorothersettingswithlimitedaccessto

highqualitymedicalcare,itisadvisabletodiscussdischargetimingwiththeperson,amilyandthelocalprimaryhealthcareteam

(particularlyMa-oriorPacichealthproviderswherepossible).Insomecases,itmaybeadvisabletoprolongthehospitalstayuntil

recoveryiswelladvanced.

Advice on discharge

Allcasesshouldhaveagoodunderstandingothecauseorheumaticeverandtheneedtohavesorethroatstreatedearlyinother

amilymembers.Contactmanagement(asper Table10)shouldbediscussed.

CasesandtheiramiliesshouldunderstandthereasonorsecondaryprophylaxisandtheconsequencesomissingaBPGinjection.

TherstdoseoBPGisusuallygiveninahospitalsetting.Arrangementsortherstinjectionpostdischargeshouldbemade.Theyshouldbegivenclearinormationaboutwheretogoorsecondaryprophylaxisoncedischarged,knowwhotocontactwithquestions

concerningtheirollow-uporsecondaryprophylaxis,andbegivenwritteninormationonappointmentsorollow-upwiththeirlocal

medicalpractitioner,physician/paediatricianandcardiologist(ineeded).Theyshouldbeadvisedotheappropriateactivityleveluntil

theirnextclinicappointment.

Casesandtheiramiliesshouldalsoberemindedotheimportanceoadditionalantibioticprophylaxisordentalandotherprocedures

toprotectagainstendocarditis( AppendixH).

Copiesothedischargesummaryshouldgototheollowingservices:communitynursingstaresponsibleorprophylaxisdelivery

(suchasdistrictnurse,publichealthnurse),rheumaticeversecretaryorstaresponsibleortheregister(whereapplicable),primary

careproviderandtheamily.




30




31

[SecondaryPrevention]




3

[6. Prophylaxis Regimes]

SECONDARY PREVENTION

Secondarypreventionorheumaticeverisdenedasthecontinuousadministrationoantibioticstocaseswhohavehadaprevious

attack oARFor well-documented RHD. The purpose isto preventinection o the upperrespiratory tractwithGASand thedevelopmentorecurrentrheumaticever.28

Theregularadministration oantibioticsto preventinectionwithgroupAstreptococcal(GAS) andrecurrentARF isrecommended

orallpeoplewithahistoryoARForRHD.3Thisstrategyhasbeenproveninrandomisedcontrolledtrialstopreventstreptococcal

pharyngitisandrecurrentARF.

PenicillinInearlystudiesoARFprophylaxisusingsulphonamides,1.5%otreatedcasesdevelopedARFrecurrences,comparedto20%o

untreatedcases.Subsequently,penicillinwasoundtobemoreecaciousthansulphonamides(LevelI).35,83

Arecent Cochranemeta-analysis101concludedthattheuseopenicillin(comparedtonotherapy)isbenecialinthepreventiono

recurrentARF,andthatintramuscularbenzathinepenicillinG(BPG)issuperiortooralpenicillininthereductionobothrecurrentARF

(87–96%reduction)andstreptococcalpharyngitis(71-91%reduction)(LevelI)( AppendixE).

SecondaryprophylaxisalsoreducestheseverityoRHD.Itisassociatedwithregressionoheartdiseaseinapproximately50-70%

othosewithadequateadherenceoveradecade(LevelIII2),56,102,103andreducesmortality(LevelIII2).104

Dose TheinternationallyacceptedstandarddoseoBPGorthesecondarypreventionoARFinadultsis1,200,000U.3,38,105Thedoseor

childrenislessclear.InNewZealand,itisrecommendedthat1,200,000UoBPGshouldbeusedorsecondaryprophylaxisorall

personsweighing20kgormore(LevelIII-2,GradeB),and600,000Uorthoseweighinglessthan20kg(GradeD).106

Frequency

While BPG is usually administered every our weeks (28 days), serum penicillin levels may be low or undetectable 28 days

ollowingadoseo1,200,000U.107 FewerstreptococcalinectionsandARF recurrencesoccurredamongthosereceivingthree-

weeklyBPG(LevelI).101,108,109Moreover,thethree-weeklyregimenresultedingreaterresolutionomitralregurgitationinalong-term

randomisedstudyinTaiwan(66%vs46%)( LevelII).110ProspectivedataromNewZealandhowever,showedthatrecurrences

were rare among people who were ully adherent to a our-weekly BPG regimen. In Auckland (1993 to 1999), the rate o

recurrenceinullyadherentindividualsona28dayregimewas0.07per100patientyears.Failureontheprophylaxisprogramme

(i.e.including those whowere lessthan ully adherent) was1.4 perpatient years.16,17 Thiscompares avourably to prophylaxis

ailure reportedin Taiwan o 0.25 (21-day programme)and 1.29 (28-dayprogramme) per 100patientyears.111 Furthermore,a

our-weekly regime is preerable to a three-weekly regime because o the resource and compliance implications (Grade D).

InNewZealand,threeweekly(21-day)BPGisrecommendedonlyorthosewhohaveconrmedrecurrentARFdespiteulladherence

toour-weekly(28-day)BPGdelivery(GradeC).16,17

Analternativestrategy istheadministrationolarger dosesoBPG,leadingtoa higherproportionopeoplewithdetectableserum

penicillinlevelsourweeksaterinjection.112However,untilmoredataareavailable,thisstrategycannotberecommended.




33

Table 12. Recommended Antibiotic Regimens or Secondary Prevention o Acute Rheumatic Fever/Rheumatic Heart Disease

ANTIBIOTIC

First line

DOSE ROuTE FREQuENCY

BenzathinepenicillinG(BPG)

1,200,000U≥20kg600,000U<20kg

4-weekly(28days),or3-weeklyorthosewhohavehadconrmedrecurrentARFdespiteulladherenceto4-weeklyBPG

BPGismosteectivelygivenasadeepintramuscularinjection 38

Second line (Iintramuscularrouteisnotpossibleorreused) *

Phenoxymethylpenicillin(PenicillinV)

250mg TwicedailyOral

Following documented penicillin allergy**

Erythromycin(EES) 40mg/kgperday(children)

2-4divideddoses(maximum1g/day)Oral

400mg(adolescentsandadults)

TwicedailyOral

Oral penicillin is less ecacious than BPG in preventing GAS inections and subsequent recurrences o ARF.38,83,113,114 Twice-daily oral

regimens are also likely to result in poorer rates o adherence over long periods o time115 and less predictable serum penicillin concentrations,

when compared to intramuscular BPG.35 In addition, oral penicillin V incurs a cost to the patient, while IM BPG is ree when provided through

an ARF prevention programme. Oral penicillin should be reserved or cases who reuse intramuscular BPG ( Level II, Grade B ). I a patient isoered oral penicillin, the consequences o missed doses must be emphasised and adherence careully monitored ( Grade D )

The benets o long-term BPG administration outweigh the rare risk o serious allergic reactions to penicillin and atality as a result o

anaphylaxis.35,108,116,117 The rates o allergic and anaphylactic reactions to monthly BPG are 3.2% and 0.2%, respectively, and atal reactions

are exceptionally rare.117,118 There is no increased risk with prolonged BPG use. A prospective study o 1,790 ARF/RHD patients ound

similar rates o allergic reactions in those receiving long-term penicillin therapy and those receiving short-term therapy or sexually transmitted

diseases ( Level III-2 ).118 Beore commencing penicillin treatment, cases should be careully questioned about known allergies to penicillin

and other beta-lactam antibiotics. When patients state they are allergic to penicillin or when a non-specic reaction has been reported but

there is no unequivocal evidence, they should be investigated or penicillin allergy, preerably in consultation with an allergist. The options

include skin testing118 or a supervised challenge test. Most o these patients are not truly allergic. Penicillin desensitisation is not applicable

to these patients, even with a regime o more requent injections, as it would have to be repeated beore each dose o BPG.119,120 A RAST

(RadioAllergoSorbent Test) may be used as a screening tool only. Because this is a specic but not very sensitive test, a negative RAST test

must be ollowed up in all cases with penicillin skin testing and/or consideration o a graded challenge i appropriate ( Grade D ).

NewZealandhasbeenaectedbyinconsistentsupplyobenzathinepenicillinoverrecentyears.Thisposespotentialriskstothose

requiringour-weeklyprophylaxis.Organisationalapproachestosecondarypreventionshouldseektoensureconsistentsupplyat

thenational,regionalandlocallevels.However,whenbenzathinepenicillinisunavailable,oralpenicillinorerythromycincanbegiven

(asper Table12).

Secondary prophylaxis while breast feeding, during pregnancy and while on oral

contraceptives

Asthereisnoevidenceoteratogenicity,penicillinprophylaxisshouldcontinueorthedurationopregnancyorthepreventionorecurrent

ARF(GradeD).38Erythromycinisalsoconsideredsaeinpregnancy,althoughcontrolledtrialshavenotbeenconducted.100

Penicillinisalsogenerallyconsideredtobesaetouseduringbreasteeding.Concentrationslowerthanplasmalevelsareexcreted

inbreastmilk.Noadverseeectshavebeenreported.121Erythromycinisalsoexcretedintobreastmilk,buttherearenoreportsoadverseeectsininantsanditisconsideredsaetouse(GradeD).121

*

**




3

* Denition o categories:

• Mild carditis:

• Any valve lesion(s) graded mild clinically, or by echocardiography, with no clinical evidence o heart ailure and no

evidence o cardiac chamber enlargement on CXR, ECG or echo

• Moderate carditis:

• Any valve lesion o moderate severity clinically (e.g. mild or moderate cardiomegaly), or

• Any moderate severity valve lesion on echocardiography, or

• Any echocardiographic evidence o cardiac chamber enlargement

DuRATION OF PROPHYLAXIS

Minimumo10yearsatermostrecentepisodeARForuntilage21years(whicheverislonger) **

Minimumo10yearsatermostrecentepisodeARForuntilage30years ***(whicheverislonger)

Minimumo10yearsatermostrecentepisodeARForuntilage30years(whicheverislonger),andthenspecialistrevieworconsiderationotheneedorcontinuationoprophylaxis,probablylielong.

AllpersonswithARFwithnoormildcarditis

AllpersonswithARFwithmoderatecarditis

AllpersonswithARFwithseverecarditis

CATEGORY*

Oralcontraceptivesarestillrecommendedorwomenochild-bearingagewhileonBPGprophylaxis.Progesterone-onlyoral

contraceptivesdonotinteractwithBPGtherapy.122,123,124,125 An interaction between IMBPGandthecombinedoralcontraceptive

ispossible,althoughthisinteractionissuggestedtoonlybeosignicanceorshortcoursesoantibiotictherapy(lessthanthree

weeks).Inaddition,theriskointeractionwithantibioticsissmallenoughthatitmaynotbeidentiableromtheonetothreepercentriskooralcontraceptiveailure(GradeC).126Cautionisadvisedwhenconsideringtheuseothecombinedoralcontraceptive

pillin womenwith complicated rheumatic heartdisease/valvediseaseor atrial brillation, especially orcases alsoon wararin.

A levonorgestrel-releasing intra uterine contraceptive device (such as Mirena) would be more suitable (i in a stable relationship)

(GradeD).

Secondary prophylaxis in anti-coagulated cases

IntramuscularbleedingromBPGinjections,usedinconjunctionwithanticoagulationtherapyinNewZealand,israre.Thus,BPG

injectionsshouldbecontinuedorthosewhoareanti-coagulated,unlessthereisevidenceouncontrolledbleedingortheinternational

normalisedratio(INR)isoutsidethedenedtherapeuticwindow( GradeD).Casesdischargedromhospitalonoralpenicillinollowing

valvesurgeryshouldrecommenceBPGassoonasispractical.

Theappropriatedurationosecondaryprophylaxisdependsonanumberoactors.Theseinclude:

• age(ARFrecurrenceislesscommonatertheageo25anduncommonatertheageo30)16,17,38

• clinicalpattern(presenceorabsenceocarditisorRHDandseverityocarditisorRHD)

• environment(particular lythelikelihoodoongoingexposuretoGAS)

• timeelapsedsincelastepisodeoARF(ARFrecurrencesarelesscommongreaterthanveyearssince

lastepisode).16,17,38

Basedontheseactors,therecommendeddurationosecondaryprophylaxisisoutlinedin Table13.Thedurationoprophylaxis

recommendedisalsooutlinedin Algorithm3.

Table 13. New Zealand Recommendations or the Duration o Secondary Prophylaxis

[7. Duration of Secondary Prophylaxis]




35

**

***

• Severe carditis:

• Any severe valve lesion clinically (signicant cardiomegaly expected, and/or heart ailure), or

• Any severe valve lesion on echocardiography, or

• Any impending or previous cardiac surgery or RHD

• Pure aortic stenosis is rarely due to ARF and in such cases an alternative diagnosis should be considered

• When there is both mitral and aortic regurgitation, one o them must be graded moderate by echo standards in order or the carditis to be classied

o moderate severity

• Tricuspid and pulmonary regurgitation graded mild or greater may be seen in people with normal hearts who have ever, volume overload or

pulmonary hypertension. For this reason a diagnosis o carditis should not be based on right-side regurgitation a lone. Although pulmonary and

tricuspid regurgitation are oten seen in association with le t-sided lesions in ARF, pressure and volume overload must be excluded beore attributing

even moderate tricuspid regurgitation to valvulitis. I both let and right-sided lesions coexist in ARF carditis, then the predominant infuence is the

severity o the let-sided lesion

Beorestoppingprophylaxis,recipientswhoareknowntohavehadcarditisshouldbeevaluatedorsymptomaticdeteriorationandthe

stabilityandseverityovalvelesions.Thisshouldincludeechocardiographicassessment(GradeD).Wherelimitedechocardiography

isavailable,preerenceshouldbegiventothosewithahistoryomoderateorgreatercarditis,ahistoryooneormoreARFrecurrences

orclinicalevidenceocarditis(e.g.amurmur)( GradeD).Theanticipatedandactualdatesocessationshouldbedocumentedin

themedicalrecordsandontheARFregisterwherepossible,(seeSection10.3).Thedateocessationmaybereviewedithere

isachangeinclinicalorechocardiographicseverity,specialistrecommendation,achangeinenvironmentalexposuretoGAS,ora

recurrenceoARF(GradeD).

A review o data rom the Auckland Acute Rheumatic Fever Register (1993-1999) in New Zealand ound that recurrences occurred up to 21 years ater completion o prophylaxis programmes. 77% were within the rst seven years, and 30% were greater than ten years. The

mean overall recurrence interval between last attack and recurrence was 8.6 years. O the cases that received ten years prophylaxis, there

were two ARF recurrences ater discharge and an estimated 2,200 patient years o ollow-up (0.1/100 patient years). Two “breakthrough”

recurrences occurred in this series in cases who were inadvertently discharged early o prophylaxis (aged 16 and 17 years).16,17 This data

suggest that in the New Zealand environment, maintenance o prophylaxis to 21 years o age in cases with absent or mild heart disease is

sae and eective ( Level IV, Grade C )

O the Auckland (1993-1999) cases, only ve recurrences occurred ater the age o 30. 16,17 Thereore it is reasonable to cease secondary

prophylaxis at that age, except when individual circumstances warrant continuing (e.g. when cases wish to reduce even a small chance o

a recurrence) ( Level IV, Grade C )

• Individuals working or living with children or in a living situation where there is overcrowding or close proximity to others (such as boarding schools,

barracks, and hostels) have a higher risk o exposure to GAS and subsequent development o ARF. In these cases, consideration should be given

to extending the duration o prophylaxis ( Grade D )

• For those presenting at an older age (over the age o 21 years), with no or mild carditis, it is possible to consider discharge rom prophylaxis ater 5

years ( Grade D )

• The duration o prophylaxis presented here reers to ‘denite’ and ‘probable’ cases o ARF (see Section 4 ). For those with ‘possible’

ARF (where there is strong clinical suspicion, but insucient signs and symptoms to ull the diagnosis), a minimum o 5 years prophylaxis should

be considered, with regular review ( Grade D )

• For those presenting with RHD or whom no initial episode o ARF can be identied, the decision to commence and cease penicillin prophylaxis

should be taken on an individual basis with regard to the age o the case, severity o the disease, possible age o rst attack and risk o exposure

to GAS.




3

DuRATION OF PROPHYLAXIS

[8. Protocol for Secondary Prophylaxis Delivery]

*

**

***

PREPARATION

•Identiyclient(ullnameanddateobirth) *

•Conrmthatconsent**hasbeengivenorBPGdeliveryatschool(iappropriate)•Checkallergystatus,isymptomsoallergyreportedrompreviousinjectionthenwithholdinjection,documentandreporttoGPandspecialist

•Noteappropriatedoseoadrenalinerequiredorcurrentageinecessaryorananaphylacticreaction(see AppendixF)•Checktheprescription:date,requencyanddose•Checkweightorchildrenon0.6megaunits(mU)obicillintoensuredoseornextinjectionisappropriate(i.e.remainat<20kg).Record

weightinprogressnotes.Idoseshouldchange,documentandinormthelocalprescriberandregistercoordinatortoensurethedoseischangedorthenextdelivery

•Giveullexplanationtoclient•Positionclientlyingoraspreerred•Washhands•PrepareBPG(bicillincartridgeandTubexisthecurrentsystem).I0.6mUdoseisrequireddisposeohalthesyringecontentspriorto

administration.Warminhands•Alcoholswabinjectionsite,allowtodry

DELIVERY***

•Applypressuretoinjectionsiteor10secondsandconsiderothermeasurestoreducepain( Table15)•Administerbicillinslowlyintoventrogluteal,dorsoglutealareaobuttockorvastuslateralisorthigh(orasperlocalareapolicy)•DisposeotheusedsyringeinasharpscontaineraterremovingTubex

OBSERVATION

•Observeclientoraminimumo10minutesateradministrationobicillinoranysignsandsymptomsoanallergicreaction.Localpolicymaysuggesta20minuteobservationperiod

EVALuATION

•Completerecordoadministration•Revieweducationneeds/knowledge•Conrmnextappointmentandanyotherollow-upneeds•Consideropportunitytoreviewbroaderhealthissuessuchasmobilityandactivitylevels,nutritionalstatusanddietaryhabits,dentalhygiene

andsupport.

IntheNewZealandenvironment,itisrecommendedthatsecondaryprophylaxisisdeliveredbycommunitynursingstaatschools,

intheworkplaceorathome( Table14).

In eacharea thisdelivery should be supportedby thepresenceo a rheumatic ever register (seeSection 10.3),anditisalso

recommendedthatineachareaspecicmedicalstasignthree-monthlydesignatedauthorisationorthenursestodeliverBPG.The

generationotheseprescriptionswillalsobeassistedbyaregistersystem.

Table 14. Suggested Protocol or the Delivery o Secondary Prophylaxis by Community Nurses

I under 16: conrm identication with another responsible person (i.e. caregiver, school receptionist)

Consent or the duration o BPG delivery will have been obtained in hospital prior to the initiation o prophylaxis ( Table 10 ). A separate

consent needs to be obtained rom the caregiver/parent only or delivery at school

I the client is not available, and the ull syringe has been maintained in cold chain, it can be returned to the medicine ridge. I the ull syringe has not been maintained within the cold chain, then it needs to be discarded.




37

Anaphylaxisis anuncommonreaction ollowingIM orIV antibiotics,immunisation orother medicines. Anaphylaxisto benzathinepenicillinisrare.Inaprospectiveinternationalstudyater32,430injectionsduring2,736patientyearsoobservation,57(3.2%)o

the1,790patientshadanallergicreactionandour(0.2%or1.2per10000injections)hadanaphylaxis.Thelong-termbenetso

prophylaxisthereorearoutweighthepotentialriskoaseriousallergicreaction. 117Theresponsetoananaphylacticreactiontoa

BPGdoseandthemanagementoanaphylaxiscanbeoundin AppendixF.

ThepersistenceorecurrentARFinsomeareasoNewZealandhighlightsthecontinuedailureosecondaryprevention.

FailuretopreventrecurrentARFinastudyromtheGisbornearea,wasthoughttobeduetoarangeoactorsincludingalacko

recognitionotheecacyoparenteralBPGcomparedtooralregimens,inadequateadherence,unreliabledatacollectionandthe

lackolong-termcontinuityocare.127

Improvedadherencetoprophylaxisisseenwithactiveollow-upocaseswhenBPGdosesaremissed,theidenticationolocaldedicatedstamembersresponsibleordeliveryosecondaryprophylaxis,developingapersonal

rapportwitheachcaseandcoordinatingroutinecare.Eectivecommunicationbetweenhealthstaandamiliesisimportant.InNew

Zealand,itisparticularlyimportanttosupportandutilisetheexpertise,experience,communityknowledge,cultureandlanguageskills

oMa-oriandPacichealthworkersinordertoassistwithadherencetosecondaryprophylaxis.

Threemethodsorimprovingcompliancewillbediscussedurtherinthisguideline:

•reducingthepainotheBPGinjection

•education

•theuseorheumaticeverregisters.

Reducing the pain of BPG injections

ThepainoBPGinjectionsisusuallynotacriticalactorindeterminingadherencetosecondaryprophylaxis.Nonetheless,techniques

thatsaelyreduceinjectionpain( Table15)shouldbepromoted.

A smaller gauge needle and increasing the volume o injection to 3.5ml improved acceptability in Taiwan110

Direct application o pressure to the injection site has been shown to decrease pain o intra-muscular injections 128

As these measures are logical and benign they are recommended, despite lack o evidence ( Grade D )

Although merely a topical agent, some cases have reported reduced pain and bruising ollowing the appropriate use o ethylchloride spray

( Grade D )

•Deliverinjectionveryslowly(preerablyoveratleast2–3mins) ***

•Distractiontechniquesduringinjection(e.g.withconversation)

•Goodrapportwiththecase,assistedbyhavingadesignated nurseoreachcase,isasignicantaidtoinjectioncomort, compliance,andunderstanding.***

[9. Anaphylaxis]

[10. Improving Adherence to Secondary Prophylaxis ]

*

**

***

#

•Usea23-gaugeneedle*

•Applypressurewiththumbor10secondsbeore insertingneedle**

•Warmsyringetoroomtemperaturebeoreusing ***

•Allowalcoholromswabtodrybeoreinsertingneedle ***

•Useoethylchloridespraypriortoinjection #

Table 15. Measures That May Reduce the Pain o Benzathine Penicillin G Injections

The addition o 0.5-1.0 ml o 1% lignocaine is used elsewhere. It signicantly reduces pain immediately and in the rst 24 hours ater injection, while

not signicantly aecting serum penicillin concentrations.129 Procaine penicillin added to BPG also reduces pain and local reactions. The combination

is eective or the treatment o streptococcal pharyngitis, but the ormulations tested to date have not sustained adequate serum penicillin levels

or long enough or secondary prophylaxis.130,131 However, with the pre-loaded (“Tubex”) system syringes currently used in New Zealand it is not

recommended or possible or community sta to add lignocaine or procaine penicillin ( Grade D ).




3

Education

Healtheducationiscriticalatalllevels.38LackoparentalawarenessothecausesandconsequencesoARF/RHDwasakeycontributor

topooradherenceamongchildrenonlong-termprophylaxisinEgypt.132

InanumberoregionsinIndia,comprehensivehealtheducationhasimprovedcommunityawarenessosorethroat,ARFandRHD 133andassistedcaseidentication. 134Comprehensive

healtheducationandpromotionwasalsoakeycomponentinthesuccessulcontroloRHDintheFrenchCaribbean. 135Improved

healthstaawarenessothediagnosisandmanagementoARFandRHDisnecessaryinordertoimprovecasendings,encourage

compliancewithprophylaxisandtoimprovethequalityanddeliveryohealtheducationdeliveredtocasesandtheiramilies.

Education provided to the case and their family should cover:

•thecauseandcomplicationsoARF

•thereasonorsecondaryprophylaxisandthesignsandsymptomsorecurrence

•thepreventionoendocarditisandthedierencesbetweenthisandsecondaryprophylaxisoARF

•sorethroatmanagement

•theimportanceomedicalanddentalol low-up

•howtocontacttherelevantpeopleoragenciesshouldtheyrequireurtherinormationorassistance.

TheNationalHeartFoundationoNewZealandproducesabookletcalled“WhatisRheumaticFever?”toassistineducationprovision

tocasesandtheiramilies.Thisisavailabletoorderromwww.heartoundation.org.nz

ARF Registers

RegistersopeoplewithRHDorahistoryoARFareakeyelementinARFrecurrenceandRHDcontrolatanindividual,community

andnationallevel.14,15,16,25,136

In1978,theWHOpromotedtheuseodiseaseregistersaspartocommunityprogrammestohelpcoordinatepreventionoARF

recurrencesandoRHD.137Theuseotheseregistershasbeenproveninbothdevelopinganddevelopedcountriestoenhancethe

impactosecondarypreventionstrategiesorARFandtoeectivelyreducemorbidityandmortality.13,28,138

Register-based RHDcontrol programmes havebeen successulin NewZealand. Bythe early 1980s,ARF registershad been

implementedinWaikato,Northland,Auckland,GisborneandRotorua.Despitesimilarities,eachprogrammedevelopedindependently

oanynationalramework,andeachwasshowntobeeectiveatreducingadmissionsorARFrecurrences.13InNewZealand,ARF

becameanotiableconditionunderthenationalsurveillanceandmanagementrameworkin1986.13

In2001,asurveydescribingregister-basedARFpreventionprogrammesinNewZealandwasconducted.Twotypesoregisters

weredescribed:managementandsurveillance.Register-based‘management’programmesusearegistertocoordinatecommunity-

based prophylaxisprovidedpredominantly bydistrictnursingservices,collateinormationonprophylaxisdeliveryandencourage

parenteralprophylaxis. Managementprogrammesalsousetheirregisterstoperorma varyingrangeootherunctionsincluding

inorminghealthcareworkers(suchasdentistsandGPs)othosewhoarereceivingprophylaxis,generatingorpromptingpenicillin

prescriptionsandaccumulatingdataorevaluation.Sixregister-basedmanagementprogrammeswereoperatinginNewZealandin

2001(predominantlythroughpublichealthunitsincollaborationwithclinicians).ThesewerebasedinNorthland,Auckland(district

nursesinassociationwithpaediatricians),Rotorua(establishedbyanassociationoGP’s),Gisborne,HawkesBayandLowerHutt.

Collectively,these programmes coverednine health districtscontaining51.1%othe population and81.9%o ARFnotications

between1995and2000.Aurtherthree‘surveillance’programmes,withoutclinicianinput,weredescribedinWhakatane,Wanganui

andPalmerstonNorth.Theseprogrammesmaintainedarecordocasesreceivingprophylaxis,butdidnothavearoleincoordinating

theprovisionoprophylaxis.13Intotal,theseregistersystemscovered94%onotiedARFcases,andtheywereconsideredlargely

responsibleorreducingARFrecurrencerom22%(oallARFepisodes)between1972and1981toonly6%between1982and

1992.139

TheAuckland AcuteRheumatic FeverRegister,establishedin1982, isa population-basedregistercovering60% oNewZealand

ARFregistrations.Theregisterisusedbothasasurveillanceregisterandatooltogeneratedentalreerralsanddelegatedauthority

prescriptionstoaidpenicillindeliverybythedistrictnursingservice.Thosewhomisstheirprophylaxisareactivelysoughtorthreeto

sixmonthsbeorebeinginactivatedontheregister.Communitynursesromotherareascanalsoreerconrmedcasestotheregister

orongoingprophylaxis.ArecentstudyevaluatedtheeectivenessotheAucklandARFRegisterando28daypenicillinprophylaxis

byauditingrecurrencesnotiedtotheregisterinthistimeperiodorthosewithmildorabsentheartdiseasewithoutactiveollow-up

ateratleasttenyears.Inthisstudy,anoverallprogrammeailurerateo1.4per100patientyearswasdeterminedwithapenicillin

ailurerateo0.07per100patientyears. 16,17Earlierauditsothesameregisterrom1972to1981(1.5per100patientyears)and

1982to1992(0.6per100patientyears)reachedsimilarconclusions.24,140

Theseratesoprogrammeailurearehighlyacceptablewhencomparedtootherpublisheddata(0.0-2.8per100patientyears).35,56,109,110,111,137,141,142,143,144,145




39

•Increaseuptakeoandadherencetosecondaryprophylaxis 34,146

•ReducerecurrencesoARF 13,34,139,146,147,148anddeceasehospitalisationsromARF/RHD( LevelIII)34,146

•Improvecasedetection 34,135,146,149,150

•Recordprophylaxisdelivery

•EmployrecallandremindersystemsorARFcases,identiyindividualswithpooradherencetolong-termtherapyortargetededucational activitiesandotherinterventions

•MonitorthemovementoARFcases(whoaretypicallyhighlymobile),whileconormingtoprivacylegislationandpatientcondentiality

•Improvethecoordinationoongoingcarerequirementsandollow-up

•IdentiyandregisternewcasesoARFandRHD

•Usedatatoimproveprogrammestrategiesanddeterminechangesindiseaseepidemiology

•Fulllegalrequirementsodiseasenotication

•Improveawarenessamongsthealthproessionals

•Centralisedregisterscanalsosupporttheprovisionoprophylaxisorthosewhomovebetweencommunities. 149

Table 16. Primary Aims o ARF Register Systems

ItisrecommendedthatallregionsoNewZealandwithsubstantialpopulationswithARForRHDestablishacoordinatedARFregister

(preerably computerised) whichprovides individualand community reports,recall lists, reportson ARF/RHD epidemiology and

monitorstheeectivenessothelocalpreventionprogramme(GradeC).

ThemainaimsoARFregistersaresummarisedin Table16.

Theregistercanthenbeusedasthebasisoracoordinatedcontrolprogramme.ThisisthemosteectiveapproachtoimprovingBPGadherenceandclinicalollow-upopeoplewithRHD,includingspecialistreviewandechocardiography(LevelIII-3).Elements

osuchaprogrammearelistedin Table17(GradeC).

A dedicated coordinator with data entry support is critical to the success o the programme. This person should have skills in data management, basic epidemiology, and clinical medicine, or ready access to clinical expertise when individual case management issues

arise. To ensure that the programme continues to unction well despite stang changes, activities must be integrated into the established

health system.

•Alocal(preerablycomputerised)ARFregister,establishedwithinexistinghealthcarenetworksorpublichealthunits,withallthe propertiesanddataasdescribedin Tables16and18

•Commitmentromregionalandlocalservices,particularlytoensurelong-termunding

•Activitiesguidedbylocallyrelevant,evidence-basedguidelines

•Acoordinatororeachregisterprogramme *

•AcommitmenttopartnershipsbetweencliniciansandpublichealthservicesinordertosupporttheneedsopeoplewithARF/RHDand

thecommunity

•Anabilitytoassessandmonitortheburdenodisease

•Provisionoeducationorhealthpractitioners,thecommunity,thosewithrheumaticeverorrheumaticheartdiseaseandtheiramilies

•Provisionorsupportortheprovisionohealtheducationwithinthelocalcommunity,communityhealthserviceandorcommunityhealth workers

•Aollow-upsystem(suchasdedicatedARF/RHDclinics)thatensuresthatongoingcareisdelivered,particularlytothoseathighestrisk

•Amechanismormonitoringdeliveryosecondaryprophylaxisandongoingcare,programmereportingandindependentevaluation

•Someareasmayalsobeabletohaveaneectiveadvisorycommitteethatmayincludecardiologists,paediatricians,generalpractitioners, physicians,epidemiologists,nurses,publichealthpractitionersandrelevantcommunityrepresentative.

Table 17. Recommended Elements o a Register-Based Control Programme

*




0

Key data elements of ARF/RHD registers

ApossibledatasetorARF/RHDregistersisoutlinedinTable18.

Table 18. Dataset or Acute Rheumatic Fever Register*

DOMAIN

Demographics

DATA ELEMENTS

NationalHospitalIndexandname(s)

Dateobirth

Gender

Addressandphonenumbers(includingcellphoneortextmessagecontacting),alternateaddress

Detailsoparents/caregivers

Ethnicity

GPdetails

Schoolatdiagnosis(whererelevant) **

InitialARFdiagnosis Dateandplaceodiagnosisanddateoadmissiontohospital

Denite,probableorpossiblediagnosis

Medicationstakenpriortopresentation/admission

Majorcriteria:

•Presence(andseverity)ocarditis

•Presence(andsite)oarthritis

•Presenceochorea

•Presenceoerythemamarginatumand/orsubcutaneousnodules

Minorcriteria:

•Fever

•Acutephasereactants

•P-Rinterval

EvidenceoaprecedingGASinection:

•Historyosorethroat

•Throatswab

•Titres

ARFrecurrences*** Onsetdate

Presenceandseverityocarditis

Othersymptomsandsignsateachrecurrence

Prophylaxisstatusattimeorecurrence




1

DOMAIN DATA ELEMENTS

RHDdiagnosis Onsetdate/dateodiagnosis

DocumentedhistoryoARF

Valvulardysunctionanddiseaseseverityattimeodiagnosis

Surgery

Dentist

Secondaryprophylaxis Antibioticused

Doseandrequency

Datecommencedonprophylaxis

Dateolastdose

Dateonextexpecteddose

Designatedauthority

Expecteddateocessation

Annualadherencedata

Follow-up/recall Dateandplaceolastreview

Dateandplaceonextscheduledreviewbyeachprovider(cardiologist,paediatrician,physician,surgeon,echocardiography)

RecallsystemormissedBPG

Recallsystemormissedappointment

Mortality Dateandcauseodeathaccordingtoagreedcriteria(e.g.duetoRHD,notduetoRHD).

This dataset is an amalgamation o systems currently in use in New Zealand. Some o the unctions may be ullled elsewhere. Other

inormation such as details o surgical procedures and medical management may also be included

To acilitate the set-up o school-based primary prevention programmes

It is recommended that each ARF recurrence notied to the register is thoroughly investigated to determine i any changes in the system o

prophylaxis delivery need to be made to prevent such recurrences rom occuring in the uture.

*

**

Table 18. (continued)

***




Outreach and out-of-town

The non-compliantand the non-presentinggroupscontinue to be a major challenge to secondary prophylaxis. Transient living

patternsorshitingwithoutnotiyingstaoaorwardingaddresscancreateollow-updiculties.InAuckland(1993to1999),13peoplesuered14recurrencesbecausepenicillinhadbeendiscontinuedprematurely.16,17

Asthepopulationsatthehighest riskoARFareMa-oriandPacic,theinvolvementoMa-oriandPacichealthworkers,withtheir

skillsinoutreachandtheircommunityknowledge,isimportant.

Inaddition,thepresenceolocalARFregistersinNewZealandallowsorinter-registerreerral(otennursetonurse)odiagnosedARF

cases.Thisensurescontinuityocareandprophylaxiswhencasestransertoanewarea.

Non-compliance

Iacaseisnon-compliant,itisrecommendedthatanumberomethodsocontact,overanumberomonthsareattempted.Every

eortshouldbemadetoutilisecommunitycontactsinthearea,andaperiod“onhold”withcontinuedattemptstocontact,should

beusedpriortoconsideringdischarge.InAucklandearlydischargeoprophylaxisduetopersistentnon-compliance,israre.

Aprotocolorthemanagementonon-compliantcasescanbeoundin AppendixG.




3

AstructuredcareplanshouldbedevelopedandrecordedinthenotesoallpersonswithahistoryoARF,orwithestablishedRHD. Table19liststherecommendedreviewrequency(GradeD).Thisschedulemaybetailoredtotheneedsotheindividualandmay

alsodierdependingonlocalresources.Auckland,orexample(with60%oNewZealandARFcases),maynotbeabletoseecases

asrequentlyasispossibleinotherareaswithasmallercaseload.

CLASSIFICATION

Lowrisk

CRITERIA

ARFwithnoevidenceoRHD

or

Trivialtomildvalvulardisease

REVIEW AND MANAGEMENT PLAN

Secondaryprophylaxis(BPG)

Doctorreview

Echocardiography

Mediumorhighrisk#

Anymoderateorseverevalvelesion

or

Mechanicalprostheticvalves

or

Tissueprostheticvalvesandvalverepairs

Secondaryprophylaxis(BPG)

Infuenzavaccination

Dentalreview§

Cardiologist/physician/paediatricianreviewwithechocardiography

Endocarditisprophylaxis

Polysaccharidepneumococcalvaccination(Pneumovax23)

Additionalconsiderations

Followingvalvesurgery MedicalassessmentECGChestradiograph

EchocardiographyFullbloodcountUrea,creatinine,electrolytesINRiindicated

[11. Routine Review and Structured Care Planning]

Table 19. Recommended Routine Review and Management Plan or ARF and RHD*

Review requency should be determined according to individual needs and local capacity. Most critically, review should become more

requent in the event o symptom onset, symptomatic deterioration or a change in clinical ndings

In New Zealand, 4-weekly BPG is recommended unless conrmed recurrent ARF has occurred despite ull adherence to prophylaxis. In this

case, 3-weekly BPG is recommended ( Grade D )

Close supervision until stable

Anyone with severe valvular disease or moderate to severe valvular disease with symptoms should be reerred or cardiological and surgical

assessment as soon as is possible ( Grade D )

Routine dental care is critically important in cases with a history o ARF and/or RHD. All patients should receive education about oral

hygiene, and should be reerred promptly or dental assessment and treatment when required. This is especially important prior to valvular

surgery, when all oral/dental pathology should be investigated and treated accordingly ( Grade D ).

*

**

***

#

§

FREQuENCY

4-weekly**

3-5yearly,ormorerequently

dependingonlocalresource

Childrenonclinicalchange Adultsondischarge

4-weekly**

Yearly

6monthly

6-24monthly***

Asrequired

5-yearly(max3doses)

3-4weekspost-discharge




Surveillance

PassivesurveillanceoARFusuallydependsoncaseidenticationromhealthcareproviders.InNewZealand,ARFandrecurrent

ARF are notiable conditions.14 Historically however, relianceon notication hasunder-estimated theburden odiseasedue to

inaccuraciesandincompleteness. 152Inunder-resourcedsettings,thedecienciesopassivesurveillanceareexacerbatedbyhigh

turnoverohospitalandprimarycarestaandlackoawarenessoARFbymanyhealthcareproviders.

Ideally,activesurveillanceshouldbe usedtoaugmentpassivesurveillance(GradeD).153Thisentailsestablishingmechanismsto

identiynewcasesoARFandtoupdateinormationaboutexistingcases.Thiscouldinclude:

• mechanismsallowingaccesstohospitalcodingdata

• echocardiographyreports

• specialistreviewcorrespondence

• primaryhealthcareinormation.

Where possible, theseprocessesshould beautomated (includingregular downloads oinormationregardingcasesadmittedto

hospitalwithadiagnosisoARF).ThiswouldhavetobecompliantwiththeHealthInormationPrivacyCode1994.

RHDisnotanotiablecondition,andisunlikelytobeinthenearuture.Itisimportantto notehoweverthatrelyingonlyonARF

noticationwould not identiya number o Ma-ori and Pacic people with RHD. Furthermore,there isgreat potential or RHD

noticationto improveoutcomesorpeoplewithRHDbecause,unlikeormostnotiablediseases,thereisa simple,cheapand

provenintervention—secondaryprophylaxis.

[12. Prevention of Infective Endocarditis]

[13. Case Finding Surveillance and Screening]

InectiveendocarditisisadangerouscomplicationoRHD.38,151

Althoughtheeectivenessoadditionalantibioticprophylaxispriortodentalorsurgicalprocedureshasnotbeenproven,itsuseissupportedbyanimalmodelsoendocarditisandempiricalobservations,

suchasthereductionobacteraemia.38,151

Thereore,personswithestablishedRHDorprostheticvalvesshouldreceiveantibioticprophylaxispriortoproceduresexpectedto

producebacteraemia.IndividualswithahistoryoARFbutnovalvulardamagedonotrequireantibioticprophylaxis.Thosealready

receivingpenicillinorsecondaryprophylaxisshouldbeoeredadierentantibioticorprophylaxisoendocarditis.

Recommendations or the procedures that require endocarditis prophylaxis and the appropriate antibiotics are currently being

updated.ThesecanbeoundonTheNationalHeartFoundationoNewZealandwebsite(http://www.nh.org.nz).Someothese

recommendationsarealsooutlinedonawalletcardtobecarriedbycases( AppendixH).




5

Screening for rheumatic heart disease

NewZealandcriteriaorassessingscreeningprogrammesareasollows( Table20):

Table 20. Recommended Elements o a Screening Programme in New Zealand

• Theconditionisasuitablecandidateorscreening.Theconditionshouldbeanimportanthealthproblemrombothanindividualandacommunityperspective.Theepidemiologyandnaturalhistoryothecondition,includingdevelopmentromlatenttodeclareddisease,shouldbeadequatelyunderstoodandthereshouldbeadetectableriskactorordiseasemarkerandalatentperiodorpre-symptomaticstage

•Thereisasuitabletest:sae,simple,reliable,accurate,sensitive,andspecic

• Thereisaneectiveandaccessibletreatmentorinterventionortheconditionidentiedthroughearlydetection.Thereshouldbeevidencethatearlytreatmentleadstobetteroutcomesthanlatetreatment

• Thereis high qualityevidence, ideallyrom randomisedcontrolledtrials,thata screeningprogramme iseective inreducingmortalityormorbidity

• Thepotentialbenetromthescreeningprogrammeshouldoutweighthepotentialphysicalandpsychologicalharm(causedbythetest,diagnosticproceduresandtreatment)

• Thehealthcaresystemwillbecapableosupportingallnecessaryelementsothescreeningpathway,includingdiagnosis,ollow-upandprogrammeevaluation

• Thereisconsiderationosocialandethicalissues.Thereshouldbeevidencethatthecompletescreeningprogramme(identicationandinvitation,test,diagnosticproceduresandtreatment/intervention)isclinically,sociallyandethicallyunderstoodandacceptabletohealthproessionalsandthewiderpublic

•Thereisconsiderationocost-benetissues

•Whenconsideringandevaluatingaprospectivescreeningprogramme,itisimportanttoconsiderthedirectbenettoparticipantsandanypublicgoodbenetsthatmayresult

•ScreeningprogrammesneedtospecicallyconsiderandrespondtoMa-ori,itheyaretoensureparticipationbyMa-ori,whichiscrucialto

reducinginequalitiesinmorbidityandmortalityinNewZealand.

Source: National Advisory Committee on Health and Disability (2003)154

IntheMa-oriandPacicpopulationsinNewZealand,RHDullssomeotheseproperties:

TheWHOrecommendsschool-basedscreeningorRHDasatoolorestimatingthediseaseburden,andalsooridentiyingcases

inareaswithahighprevalenceoRHD.38 TheidealmethodoRHDscreeninghoweverisnotknown.TheWHOGlobalProgramme

onRHDundertookauscultatoryscreeningooveronemillionchildren. 146Insomeregions,thiswasaugmentedbyechotoconrm

thediagnosisoRHD.Thesensitivityocardiacauscultationishighlydependentontheskillotheoperator,andthespecicityo

auscultationorrheumaticcarditisislow.Thereore,theadditionoechocardiographytoconrmthediagnosishasbeenproposed.

InNewZealand,a nationalcomprehensiveRHDscreeningprogrammewouldnotbecost-eective. AnyscreeningorRHDhere

wouldhavetotargethigh-riskpopulationsinordertoimprovethepre-testprobability.Itispossiblethatauscultatoryschool-based

screening(suchasatschool-entry,oratage11coordinatedwiththeimmunisationprogramme)coulddiscoverundetectedRHDin

thesepopulations.Whereechocardiographywasnotavailabletoreviewallchildrenwithmurmurs,ahighlyexperiencedauscultator

couldselectallchildrenwithnon-innocentmurmursorechocardiography(GradeD).

• RHDisanimportanthealthprobleminthesepopulations,withsignicantmortality,morbidity,socialand

economicburden.ThenaturalhistoryoRHDiswellunderstood(thankstoclassicstudiesothe20th

Century),99,155withalatentorearlysymptomaticstage

• goodadherencetosecondaryprophylaxispreventsthedevelopmentorworseningoRHDandleadsto

diseaseresolutioninmanycases99,143

• mildervalvelesions,whichareotenasymptomaticandthusthemostcommonlesionsthatwillbedetected

withscreening,aremorelikelytoresolvethanmoreseverelesionsinthosewhoadheretosecondary

prophylaxis41,46,103,155

• auscultationandechocardiographycouldprovideappropriatetestingtoolsthatarehighlysensitiveand

specicorthedisease,aswellasbeingacceptabletothepersonscreened.




Lowschoolattendanceorchildrenohigh-riskgroupsinsomeareasmayinfuencetheeectivenessosuchaprogramme.Apilot

programmetoestimatetheprevalenceoundetectedRHDinaspeciedpopulationmayberequired(GradeD).

Suggested indicators for evaluation

ControlprogrammesorARF/RHDshouldbeevaluatedoncriteriaorroutinecareandkeyepidemiologicalobjectives.Theseinclude

measurementoindividualand communityadherenceto secondaryprophylaxis,indicators osatisactorycarespeciedin best-

practiceguidelinesandratesodiseaseoccurrence,recurrenceandmortality.

Furtherconsiderationshouldbegivento:

• assessingthedeliveryospecialistcardiologyservices

• availabilityandaccessibilityoechocardiography

• reerralpracticesandstructures

• transportationorcases

• supportstructuresandappropriateollow-upprocesses.

Ashasbeenhighlightedthroughoutthedevelopingworld,theavailabilityoandsupportorroutinehealthcareisessentialtocontrolling

ARF/RHD.IndicatorsusedtoevaluateARF/RHDcontrolprogrammesshouldberelevant,structured,measurable,routinelyavailable

andaordable.Inparticular,theyshouldnotoverburdenhealthcareprovidersandshouldleadtoimprovedclinicalresults.Alisto

suggestedindicatorsisprovidedin Table21(GradeD).

Table 21. Proposed Indicators or Evaluating ARF/RHD Control Programmes

Secondary prophylaxis

•TheproportionoscheduledBPGinjectionsdeliveredintheprevious12months

•Individual,communityandregionalgures,expressedas:

•Medianpercentageodosesdelivered

•Proportionwhoreceive80%orlessoscheduleddoses

•Proportionwhoreceive50%orlessoscheduleddoses

Medical review

•Proportionoregisteredindividualswhoaremorethan3monthsoverdueorspecialistorothermedicalreview,asdenedbylocalguidelines

•Proportionoindividualswhohaveechocardiographyperormedwithin3monthsoscheduledtiming

•Mediantimeelapsedbetweenrecommendationandperormanceovalvularsurgery

Epidemiology

•Yearly(orotherappropriatetimerame)age-specicincidenceratesoARF

•ProportionoARFepisodesintheregisterclassiedasrecurrences

•RatesoARFrecurrenceper100patient-years

•Numberodeathsandage-standardisedratesomortalityduetoARF/RHDintheprevious12months(orotherappropriatetimerame)

•Yearlyage-specicandoverallpointprevalenceoRHD

•ProportionoARFcasesnotiedtoandrecordedbypublichealthauthoritiesintheprevious12months(orotherappropriatetimerame)

•ProportiononewlyregisteredindividualswithaninitialdiagnosisbeingestablishedoRHD(ratherthanARF).




7

Therearea numberodriving orcesthatwillassist theimplementationo thisguideline. Thereisnational practitionerdemandorstandardisationo thediagnosisoARFinordertominimiseover-andunder-diagnosisandensurethatthehigh-riskpopulations

receiveappropriatecare.ThereisalsodemandoreectiveandcosteectivemanagementandavoidanceoARFrecurrenceand

subsequentdisablingRHD.Restrainingorcesthathavethepotentialtohindertheimplementationothisguidelineinclude:reduced

accessocasestodiagnostictestsandspecialistservices,limitedresourcesavailable,reluctanceopractitionerstochangecurrent

practice,incompleteunderstandingoARFamongstprimaryandsecondarycareproessionalsandinconsistentaccesstocertain

treatmentsincludingBPG.

Suggested implementation strategies include:

Streamlined processes for the diagnosis, management and prevention of ARF

• ConsistentNewZealandstandardsorARFdiagnosis

• Consistentstandardsorstreptococcalserologymethodology,reportingandassaybetweenlaboratories.

Provision of streamlined specialist services

• Wherepossible,regionshavetheopportunityorregularspecialistrheumaticeverclinics(potentiallyinvolving

both paediatric and medical input, in close association with available cardiology services). These should

coordinatewithrheumaticeverregisters,thecommunityservicesinvolvedinBPGdeliveryandwithprimarycare

providers(particularlyMa-oriandPacic).Thishasthepotentialorreducingcasesthatarelosttoollow-upand

tosecondaryprophylaxisandthereorereducerheumaticeverrecurrence,hospitalisationsandRHD.

Ensure funding for training

• Maintain echocardiography standards or ARF and training o echo technicians in all main centers o

ARFprevalence.

Education

• Proessionaleducationtargetingbothprimaryandsecondarycareproviders,doctors,nurses,dentists,pharmacy,

medicalandnursingstudents

• Increasedunderstandinginprimarycareoearlymanagement,andtheneedorhospitalisationinARF.

Community awareness and health promotion

• Raiseawareness,especiallyinamiliesandcommunitiesathighrisk,othe“sorethroatsdomatter”messageand

othesignsandsymptomsoARF.

Ensure regular supply of benzathine

• ThesupplyoBPGhasbeeninconsistent,withoccasionalperiodswherenoBPGwasavailable.Theseguidelines

providetheopportunitytodiscusswithPHARMACthemeansoensuringanuninterruptedsupplyoBPG,includingthepossibilityohavinganalternativesupplier.

• Discussionswithhospitalspharmacies onstoringback-upsuppliesoBPG couldensurea contingencyplan

shouldsuppliesrunout.

Resource and support for a local ARF register in each area

•Proessionalleadership

•Adequateadministrativesupport.

Case follow-up

• BecauseanumberoARFcases(particularlyinAuckland)involvePacicpeople,thereisopportunityorgreater

linkstobeorgedbetweenNewZealandandthePacicIslands.KeycontactsoneachPacicIslandneed

tobeidentied.TheyshouldbeabletoaccessinormationonNewZealandregistersandprovidereciprocal

inormationtotheNewZealandregisters. Thiswillimprovethecontinuityoprophylaxistherapyandcareor

casesthattravelbetweenthesecountries.

[14. Implementation]




• Inaddition,thereshouldbecontinuedsupportortheoutreachcapacityoprimarycareprovidersin

ordertoreducethenumberocasesthatarenon-compliantordonotpresentorprophylaxis.

Dissemination of guidelines

Itishopedthatthisguidelinewillbeusedwidely.Theollowingaresuggestionsordisseminationothisguideline:

• TheNational HeartFoundation oNew Zealandthroughprinted resources,includingthis guideline

andweb-basedinormation

• TheCardiacSocietyoAustraliaandNewZealand(CSANZ),specicallythelaunchotheguidelines

attheCSANZmeetinginAucklandinMay,2006

• Dissemination by members o the writing group, reviewers and contributors and the endorsing

organisations

• Productionanddistributionoanadditionalresourceconsistingothealgorithmsromthisanduture

guidelines

• Publishedarticles

• Healthpromotioninitiativesanddiscussionothisguidelineinregionsorelativelyhighprevalenceo ARF.




9




51

* Echo here reers to ullling the echocardiographic criteria o ARF. See guidelines or urther details.

Subcutaneous

nodules

Erythema

marginatum

These are rarely seen as sole major

criteria, thereore look or other major:

• Arthritis (inclding mono with

NSAID), or

• Carditis (inclding sbclinical)

Strep.

serology

Strep.

serology

Defnite

ARF

Possible ARF i no other

diagnosis and high-risk grop

Repeat echo at 2-4 weeks, repeat

serology, consider prophylaxis

Consider

alternative

diagnosis

Flfls Jones criteria, bt with

erythema marginatm or

sbctaneos nodles as sole

major, ollow-p or evoltion o

diagnosis

Minor

criteria

or

-ve

-ve +ve

+ve

+ve-ve

Yes No

Abbreviations:

alt. = alternative ARF = acute rheumatic ever dx = diagnosisEcho = echocardiogram

GAS = group A streptococcus mono = monoarthritisNSAID = non-steroidal anti-infammatory drugRHD = rheumatic heart diseaseStrep. = streptococcus

NotealsothatcasescanullltheJonescriteriabutnothaveARF.Discussionwithanexperiencedclinician,withreerencetotheull

guidelineisrecommended.




5

Any person with suspected ARF and a cardiac

murmur, or any case o chorea, should have an

echocardiogram shortly ater admission to hospital

Eqivocal

Repeat at

2-4 weeksNormal

Abnormal

Tables 6 & 7

Prse alternative

diagnoses

Tables 8 & 9

Second echocardiogram

at 2-4 weeks i no alternative

diagnosis. A second echo is

usually unnecessary with a

presentation o chorea

Second echocardiogram

at 4-6 weeks i:

• Signs progress

• medication commenced

• recommended by cardiologist

Normal Abnormal

Tables 6 & 7

Algorithm 2: Guide for the use of echocardiography in ARF




53

Algorithm 3: Guide for the duration of secondary prophylaxis

New Zealand standard recommendations are for four-weekly (28-day) IM BPG prophylaxis. A 21-day schedule

of prophylaxis is recommended only for those cases with ARF recurrence while compliant with the four-weekly

schedule. Refer to the text of the guideline for further details.

See Table 2 or denitions o possible, probable and denite ARF

It is recommended that cases with established valvular disease have regular dental care and ollow the guidelines or endocarditis

prophylaxis

Individuals working or living with children, or in a living situation where there is overcrowding or close proximity to others (such as

boarding schools, barracks, and hostels) have a higher risk o exposure to GAS and subsequent development o ARF.

*

**

***

Possible* ARF Probable* or definite* ARF Established RHD**

No or mild

carditis

Moderate

carditis

Severe

carditis

Consider prophylaxis

Table 13

5 years

prophylaxis with

reglar review

Possible to

consider 5 years

prophylaxis

Prophylaxis or

10 years or ntil

21 (whichever islonger)

Prophylaxis or 10 years or

ntil age 30 (whichever is

longer), then review severity

o disease, GAS

exposre*** and discssconsideration o contined

prophylaxis (probably

lielong)

Consider

contination o

prophylaxis

Stop

prophylaxis

ater 10 years

Ongoing exposreto high-risk GAS

environment***

Low-risk GAS

environment***

Age over 21 Age nder 21

Table 13

Prophylaxis

or 10 years

or ntil age 30

(whichever is

longer), then

review




5

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3

Appendix A: Guideline development process

• RelevantliteratureregardingARFwasidentiedprimarilyusingcomputerisedMedline,CINAHL,ProQuestandotherdatabases.

PublicationswerelimitedtothoseintheEnglishlanguage.Articlesoundthroughthismethodologywerethensearched

orrelevantinormationand urtherarticlesidentiedthroughbibliographicreerences. AsubstantialphysicallibraryoARF

reerencesheldattheSchooloPopulationHealthwasalsoreviewedorkeyarticles.Inadditiontojournalarticlesearches,

regularreviewandsearchesweremadeointernetsitessuchastheWorldHealthOrganisation,NewZealandMinistryo

Health,NewZealandEnvironmentScienticResearch(ESR)andtheNewZealandDepartmentoStatistics

• In2005,asteeringgroupwhicharoseoutotheNewZealandmembersothewritinggrouportheAustralianguidelinesmet

andagreedtodeveloptheNewZealandversionoguidelinesorthediagnosis,managementandpreventionoARF

• awritinggroupcomprisingexpertsintheareareviewedtheAustraliandratandreachedconsensusonareasodisagreement

• selectedindividualsre-wrotetheAustralianguidelinesortheNewZealandcontext,andaccordingtotheoutlinerecommended

bytheNewZealandGuidelinesGroup(NZGG)

• members o the writing group withexperience in ARF/RHD diagnosis, management,and prevention thenreviewedeach

chapterandtheirsuggestionswereincorporatedintoaseconddrat

• thereviseddratwaswidelydistributedtoarangeostakeholders,whoweretheninvitedtocomment

• thestakeholdersreviewedthedratandreachedconsensusonareasodisagreement

• thecommentswerethenincorporatedintoanaldrat,whichwasendorsedbythestakeholders.

[17. Appendices]




Appendix B: Jones criteria for the diagnosis of ARF

Thereisnosinglesymptom,sign,or laboratorytestthatisdiagnosticorARF.TheJonescriteria wereintroducedin1944.Majormaniestations(leastlikelytoleadtoanincorrectdiagnosis)atthattimeincludedcarditis,jointsymptoms,subcutaneousnodulesand

chorea.HistoricalevidenceoARForRHDwasalsoamajormaniestation.Minormaniestations(suggestive,butnotsucientorthe

diagnosis)includedclinicalsignssuchasever,erythemamarginatum,andabdominalpainandlaboratorymarkersoinfammationsuch

asESRandleukocytosis.SinceaprevioushistoryoARFwasconsideredamajorcriterion,casesonlyneededminormaniestations

inordertoullthediagnosis(onemajorandtwominor).21,28

Inordertoimprovespecicity,in1956arthritisreplacedjointsymptomsasamajormaniestation,anderythemamarginatumwas

reconsidered asa majormaniestation. A preceding ARF orRHD was reclassiedas a minormaniestation, and otherminor

maniestationsoarthralgia,andevidenceoaprecedingGASinectionwereadded. 39Insubsequentrevisionsin1965and1984,

evidenceoaGASinectionwasconsideredessential.28,158,159

Maniestations AHAModifed 1956

AHARevised 1965

(1984)

WHO1988

AHA update1992

WHO2003

Carditis

Long PRa

Arthritis

Arthralgia

Sbctaneosnodles

Chorea

Erythemamarginatm

Pre-existingRF/RHD

FeverWBC, ESR, CRPa

Epistaxis,abdominal pain, anemia,plmonary fndings

Recent streptococoalinection

Essential Major Minor Special Consideration

Source: WorldHealthOrganisation(2004).Page20. 28

aPR=PRintervalintheelectrocardiogram;WBC=leukocytosis;ESR=erythrocytesedimentationrate;CRP=C-reactiveprotein.

Original Jonescriteria 1944




5

ThecurrentJonesCriteria(1992)37aredesignedtoestablishthediagnosisotheinitialattackoARFandaprevioushistoryoARF

orRHDisexcludedromthelistominormaniestations.ThesensitivityoARFarthritistoNSAIDsandsalicylates,andthereorethe

potentialortheuseothesemedicationstoaidindiagnosis,isdescribed.Inaddition,the1992criteriadenethreecircumstances

inwhichthediagnosisoARFcanbemadewithoutstrictlyadheringtotheJonescriteria.

Theseare:

• choreaoccuringastheonlymaniestationoARF

• indolentcarditisoccurringastheonlymaniestationoARF

•apresumptivediagnosisorheumaticeverrecurrencemaybemadewhenasinglemajororseveralminor

maniestationsarepresent inapatientwithareliablehistoryoARForestablishedRHD,providedthereis

evidenceoarecentGASinection.37

TheJonescriteria WorkingGroup metagain in2000to reviewthe adequacyo existingguidelines orthe diagnosiso the initial

attackoARF.Theconsensusopinionatthistimewasthatnonewversionothecriteriawasjustied. Itwasreiteratedthatthe

epidemiologicalsettingwherediagnosisisbeingmadeisimportant,andthatstrictadherencetotheJonescriteriainareasohigh

prevalencemayresultinunder-diagnosis.160Thisgroupdeterminedthatechocardiographyisuseulorconrmingclinicalndings,

assessingseverityovalvulardisease,chambersizeandventricularunction,andnotingthepresenceandsizeopericardialeusions.

EchocardiographywasalsonotedtobeuseulorthemanagementoARF,andtoexcludeARFasacauseomurmur.However,the

useoechocardiographyinthediagnosisoARFwasdeterminedbythisworkinggrouptobetoocontroversialtoclassiyasamajor

orminorcriterion.Controversyarosebecauseo‘normal’valvularregurgitation(whichincreaseswithage),regurgitationwithebrile

illnessesunrelatedtoARF,andtheuncertaintyoverthelong-termprognosticsignicanceoechocardiography.160




Appendix C: Use of echocardiography in ARF

Echocardiographyis nowrecommended orallsuspectedcaseso ARF.The usesoechocardiographyinARF arepresentedin

Table22.

Table 22. Uses o Echocardiography in ARF

EchocardiographicevidenceosubclinicalcarditisissucientasamajormaniestationoARF

Conrmingthepresenceoapericardialeusion

Revealinginaudibleorsubclinicalvalvularregurgitationinpresenceoarictionrub

Deningletventricularunction

Conrmingtheseverityovalvulitis(valvulitisisusuallypresentinARFwithheartailure)

Visualisationoanatomyothevalves,especiallyinmitralregurgitation.Thisisparamountinsurgicaldecision-making

Deningtheseverityomitral,aorticand/ortricuspidregurgitation

Deningtheseverityomixedvalvedisease

Identiyingsubclinicalevidenceorheumaticvalvedamage.

DIAGNOSIS

PERICARDITIS

MYOCARDITIS AND CONGESTIVE HEART FAILuRE

VALVuLITIS

TheanatomyandphysiologyoARFasshownbyechocardiographyM-modeand2-dimensionalechocardiography(2DE)areusedin

evaluatingchambersizeandventricularunction.Morecomplexormulaebasedon2DEcanalsobeusedtocalculateletventricular

unction(e.g.singleplaneellipseandSimpson’smethodsodiscs). 452DEallowsvisualisationotheunctionalanatomyoacutemitral

regurgitation.Thedegreeoannulardilatationiseasilyshownbyrelatingannularsizetobodysuracearea.Mitralvalveprolapseis

arequentndingwithgreaterdegreesomitralregurgitation.Chordalelongationandsometimeschordalrupturemayoccurinthe

presenceosignicantvalveprolapse.161,162,163,164

ValvularregurgitationcanbeaccuratelygradedwithpulsedandcolourDopplerechocardiographyasnil,physiological,mild,moderate

andsevereorbothrheumatic40andnon-rheumaticvalvedisease.165,166,167,168ColourDopplerechocardiographyshowsthedirectiono

theregurgitantjet,whichisdirectedposteriorlywithanteriormitralvalveleafetprolapse,andanteriorlywiththelesscommonposterior

leafetprolapse.

Echocardiography and physiological valvular regurgitation

Trivialvalvularregurgitationiscommonlydetectedonechocardiographyasanormalnding.Itcannowbereadilydistinguishedrom

pathologicalregurgitation.First,valveclosureisassociatedwithphysiologicaldisplacementoasmallamountoblood,theclosing

volume,whichisdetectablebycolourfowDopplerimaging.Second,trueregurgitantjets,albeittrivialinnature,maybeobservedin

normalindividualsoallages46,169Theseleaksextendbeyondthevalvecoaptationpoint,butusuallybyonly1cmorless.46,169,170They

mayhaveahighvelocitycomponent,generallyoronlypartosystoleordiastole.




7

Trivialright-sidedregurgitationisverycommon,171buttrivialaorticregurgitationisuncommon,occurringin0-1%onormalsubjects,

exceptinonestudy46whereclosingvolumeswereincluded.ThecharacteristicDopplerechocardiographiceatureotrivialmitral

regurgitationinnormalsubjectsisanaliasingfowpatterninearlysystole,withavelocityusually<1m/s. 46,169,172Onestudyreported

holosystolicfowsignals,buttheywererecordedonlyatthevalveleafets,andhadapoorlydenedspectralenvelope.170Sometimesabriehighvelocitycomponentmaybedetected.170

Subclinical evidence of rheumatic valve damage

InthosewithsuspectedARFandamurmur,relianceonclinicalndingsalonemayresultinmisclassicationocarditis. 38,40,65Some

caseshavebeenshownonechocardiographytohaveaphysiologicalorfowmurmur,orevencongenitalheartdisease.Thelikelihood

omisclassicationhasincreasedinrecentyears,asphysicians’auscultatoryskillshavebecomelessprocient.38Thereisconvincing

evidencethatsubclinicalorsilentrheumaticvalvedamagedetectedbyechocardiographyispartothespectrumorheumaticcarditis

andshouldnotbeignored.Thishasbeenconrmedbyinvestigatorsinmanyregionsaroundtheworldwithhighratesorheumatic

ever, includingNew Zealand 40,41,65Australia,USA,57,173,174Qatar,156,175Brazil,176 Turkey, Chile,177 Tahiti,178Nepal,179Portugal,180Egypt

andIndia.181AsinglereportromIndiadescribing28patientswithpolyarthritisorchoreaailedtodetectanysubclinicalcarditis. 64

In experienced hands, subclinical rheumaticvalve damage canusuallybe dierentiated onechocardiographyrom physiological

regurgitation.40,171,177However,therearesomeauthorswhoadvocateagainsttheconceptosubclinicalrheumaticvalvedamage.182

AWorldHealthOrganisationexpertcommitteeconcurredthat subclinicalrheumaticvalve damageexists.38However,becausethe

clinicalsignicanceothisndingisnotyetknown,theydecidedagainstrecommendingitsinclusionintheJonescriteria. Inthe

opinionothe authorso thisreview, echocardiographicdiagnosiso subclinical valvedamagecan helpexperiencedcliniciansin

makingthediagnosisoARF,orinconrmingthepresenceocarditisincasesoARFwithoutanobviouslypathologicalheartmurmur.

Thereore,itisrecommendedthatechocardiographicallysuggestedvalvedamage(subclinicalorotherwise),diagnosedbyaclinician

withexperienceinechocardiographyopatientswithARF/RHD,beincludedasamajormaniestation( Table3)(LevelIV,GradeC).

SubclinicalvalvedamageinfuencesthediagnosisoARFinrelativelyewindividuals.Mostcaseshaveeithermigratorypolyarthritis,

orclinicallyovertcarditisthat canbe conrmedbyechocardiography. However,therearesome casesin which thendingmay

helptoconrmthediagnosis,andtoreinorceinthemindsocasesandtheiramiliestheimportanceoadherencetoasecondary

prophylacticregimen( Table23).

Erythemamarginatum Nil,becauseclinicalcarditisorpolyarthritisusuallypresent

Subcutaneousnodules Nil,becauseclinicalcarditisorpolyarthritisusuallypresent

Clinicalcarditis Nil Denesinvolvementosecondvalveionly1valvehasclinicalcarditis.

Polyarthritis

MAIN CLINICAL FEATuRES OF ARF IMPLICATIONS OF A FINDING OF SuBCLINICAL VALVE DAMAGE

DIAGNOSTICALLY

Usuallynone, as Jones criteria ullled, butcanincreasecondenceindiagnosisoARF

CLINICALLY

Helps to reinorce the importance o 2°prophylaxis

Monoarthritisorarthralgia May conrm thediagnosisas ARF,as long asothercausesojointdiseaseareexcluded

Chorea ConrmsthediagnosisasARF.Avoidstheneedtoexcludeothercausesochorea.

Table 23. Diagnostic and Clinical Utility o Subclinical Rheumatic Valve Damage in ARF




Appendix D: Medications used in ARF

Table 24. Medications Used in ARF

BenzathinepenicillinGIM

or

MEDICATION

Treatstreptococcalinection

INDICATION

900mg(1,200,000U)>20kg450mg(600,000U)≤20kg

REGIMEN

Singledose

DuRATION

PenicillinVPO

or

250mgbd 10days

ErythromycinethylsuccinatePO

40mg/kgperdayin2-4divideddosesmaximum1g/day(children)

400mgbd(adolescentsandadults)

10days

ParacetamolPO Arthritisorarthralgia-mildoruntildiagnosisconrmed

60mg/kg/day(max4g)givenin4-6doses/day.Mayincreaseto90mg/kg/dayineeded,undermedicalsupervision

UntilsymptomsrelievedorNSAIDstarted

CodeinePO Arthritisorarthralgiauntildiagnosisconrmed

0.5-1.0mg/kg/dose(adults15-60mg/dose)4-6h

AspirinPO Arthritisorseverearthralgia(whenARFdiagnosisconrmed)

80-100mg/kg/day(4-8g/dinadults)givenin4-5doses/day

Reduceto60-70mg/kg/daywhensymptomsimprove

Considerceasinginthepresenceoacuteviralillness,andconsiderinfuenzavaccineiadministeredduringautumn/winter

Untiljointsymptomsrelieved

NaproxenPO Arthritis(iaspirin-intolerant)

10-20mg/kg/day(max1250mg)givenbd

Asoraspirin

PrednisoneorPrednisolonePO

Severecarditis,heartailure,pericarditiswitheusion

1-2mg/kg/day(max80mg).Iused>1week,taperby20-25%perweek

Usually1to3weeks

FrusemidePO/IV(canalsobegivenIM)

Heartailure Children:1-2mg/kgstat,then0.5-1mg/kg/dose6-24hrly(max6mg/kg/dose)

Adults:20-40mg/dose12-24hrlyupto250-500mg/day

Untilailurecontrolledandcarditisimproved




9

SpironolactonePO

MEDICATION

Heartailure

INDICATION

1-3mg/kg/day(max100-200mg/day)in1-3doses.Rounddosetomultipleo6.25mg(quarteroatab)

REGIMEN

Asorrusemide

DuRATION

EnalaprilPO Children:0.1mg/kg/dayin1-2dosesincreasedgraduallyover2wkstomaxo1mg/kg/dayin1-2doses

AdultsInitial:2.5mgdailyMaintenance:

10-20mgdaily(max40mg)

Asorrusemide

LisinoprilPO Heartailure Children:0.1- 0.2mg/kgoncedailyupto1mg/kg/dose

Adults:2.5-20mgoncedaily(max40mg/day)

Asorrusemide

DigoxinPO/IV Heartailure/atrialbrillation

Children:15mcg/kgstatandthen5mcg/kgater6hrs,then3-5mcg/kg/dose(max125mcg)12-hourly.

Adults:125-250mcgdaily

Checkserumlevels

CarbamazepinePO Severechorea 7-20mg/kg/day(7-10mg/kg/dayusuallysucient)giventds.

Untilchoreacontrolledorseveralweeks,thentrialomedication

Heartailure

Seekadviceromspecialist

ValproicacidPO Severechorea(mayaect

salicylatemetabolism)

Usually15-20mg/kg/day(canincrease

to30mg/kg/day)giventds

Asor

carbamazepine.




70

Appendix E: Comparison of intramuscular penicillin and oral penicillin for secondary

prevention

AsearchwasconductedbyManyembaandMayosi(2002).101ThesearchstrategyincludedtheControlledTrialsRegister(Cochrane

LibraryIssue2,2001),Medline(January1996toJuly2000),Embase(January1985toJuly2000),reerencelistsoarticlesand

consulatationwithexperts.

Randomisedandquasi-randomisedstudiescomparing:(i)oralwithintramuscularpenicillin;and(ii)two-weeklyorthree-weeklywith

our-weeklyintramuscularpenicillininpatientswithpreviousARF.Tworeviewersindependentlyassessedthetrialqualityandextracted

thedataosixincludedstudies(1,707patients).

Fourtrials(1,098patients)comparedIMwithoralpenicillinandallshowedthatIMpenicillinwasmoreeectivethanoralinreducing

recurrenceoARFandstreptococcalthroatinections.

Onetrialcomparedtwo-weeklywithour-weeklyIMpenicillin.PenicillingiveneverytwoweekswasbetteratreducingARFrecurrence

(relativerisk(RR)0.52,95%condenceinterval(CI)0.33-0.83)andstreptococcalthroatinections(RR0.60,95%CI0.42-0.85).

Onetrial(249patients)showedthatthree-weeklyIMpenicillininjectionsweremoreeectivethanour-weeklyIMpenicillinatreducing

streptococcalthroatinections(RR0.67,95%CI0.48-0.92).

TheconclusionsmadethereorewerethatIMpenicillinseemedtobemoreeectivethanoralpenicillininpreventingARFrecurrence

andstreptococcalthroatinections.Two-weeklyorthree-weeklyinjectionsappearedtobemoreeectivethanour-weeklyinjections.

However,theevidencewasbasedonpoor-qualitytrialsandtheuseooutdatedormulationsooralpenicillin.101




71

Appendix F: Anaphylaxis recognition and management

Thesignsandsymptomsoananaphylacticreactioninclude:rapidweakpulse,wheeze,tightnessinchest,pruritis,urticaria,giddinessorheadache,fushingand/orperiorbitaloedema.

Response procedure:

•donotleavethepatientalone

•callorassistance

•liepatientinrecoveryposition(maybebettersittingupisevererespiratorydistress)

•ensureairwayisclear,applyoxygeniavailable

•giveadrenaline( Table25)

•ring111orambulance

•checkvitalsigns,notecolour,toneandperusion

•isignsourtherdeterioration,repeatadrenalineater10minutes

•upto3dosesoadrenalinecanbegiven.

Adrenaline dosage:

Table 25. Recommended Dose o Adrenaline in Anaphylaxis*

0.5mlo1:1000adrenaline,deepIMinjection

12 YEARS OF AGE AND OVER

Approximately0.01ml/kgo1:1000adrenaline,deepIMinjection

•Age0-3years:0.1ml




uNDER 12 YEARS OF AGE

Source: StarshipHospitalClinicalPracticeManual,AucklandDistrictHealthBoard(2006).

* Up to 3 doses o adrenaline can be given




7

Appendix G: Protocol for follow-up of non-compliant cases

Case is non-compliant with injections on 3-4 concurrent

occasions. All attempts at contact are clearly documented in

the patients le. These attempts should include the use

of multiple modalities for contact including telephone

calls, visits, texting and the use of the local knowledge of

community health workers

Discss with primary nrse and reer to commnity health worker,pblic health nrse, or other commnity sta as ftting in the area or

ollow p. Note also opportnity to involve sta rom Ma-ori or Pacifc

primary health providers, i appropriate

Commnity health worker (or other commnity sta responsible)

ollows p with case (and amily) to determine reason or non-

compliance. Where necessary and appropriate, provides on going

spport, edcation, and arranges appointments or review at

otpatient clinic

I compliance is no

longer a problem,

contine rotine

secondary prophylaxis

At the end o the holding period, the

primary nrse and commnity health

worker review the case and i

considered appropriate a discharge

letter is to be sent to the case, with a

copy to the patient fle, GP, and rhematicever register (i available)

I non-compliance contines, letter o

planning to discharge is copied to the

case, case fle, and GP ater discssion

with primary nrse and commnity

health worker

Case fle goes “on hold”

or p to six months

(local area policy may sggest

reglar attempts at contact while

case is on hold)




73

Appendix H: Wallet card for infective endocarditis prevention

Information For Patient/

Parents/Guardians

has a heart disorder and therefore

needs antibiotic protection to

be given before some of the

procedures that dentists and

doctors may need to do.

YOU MUST SHOW THIS CARD

TO ANY DENTIST/DENTAL

THERAPIST OR DOCTOR

BEFORE TREATMENT IS

STARTED.

General Advice

1 Regular teeth cleaning and

avoiding sugary foods and

drinks will reduce the need

for dental surgery.

2 Regular dental check ups will

help keep teeth healthy.

HOSPITAL CHECK UPS DO

NOT REPLACE VISITS TO

YOUR LOCAL DENTIST/

DENTALTHERAPIST.

3 Using a mouth guard for

contact sports will help

protect teeth.

4 Antibiotics are not needed for

natural loss of baby teeth.

■Information for

Doctor/Dentist/Dental

Therapist

This patient is at risk of

bacterial endocarditis and

requires prophylaxis as detailed

below. Antibiotic prophylaxis

is necessary for all procedures

involving manipulation/bleeding

of the gingival tissues and any

instrumentation through the apex

of the tooth.

■Dental/Oral/Respiratory

Tract/Oesophageal Procedures

Patients who have not received

Penicillin or Cephalosporin in the

last two weeks and are not on

long term Penicillin:

■Adults and Children Over

10 years

2 g Amoxycillin orally one hour

prior to procedure.

1 g Amoxycillin orally six hours

after the first dose.

PLEASE CARRY THIS CARD WITH YOU

INFECTIVE ENDOCARDITISPROPHYLAXIS

Name:

NHI:

Diagnosis:

GP:

Hospital Doctor: Standard Risk ■ High Risk ■

■Children Under 10 years

Amoxycillin 250 mg in 5 ml, oral

suspension 50 mg/kg (max 2

g) one hour prior to procedure

then,25 mg/kg (max 1 g) six

hours later.

■Patients with Penicillin

allergy or treated with

Penicillin or Cephalosporin

within the last two weeks,

or on long term Penicillin

prophylaxis Adults and

Children Over 10 years

Clarithromycin tab 500 mg orally

one hour prior to procedure.

A single dose only is required.

■Children Under 10 years

Clarithromycin 125 mg/5 ml oral

liquid 15 mg/kg (max 500 mg)

one hour prior to procedure.

A single dose only required.

■Genitourinary and

Gastrointestinal (excluding

oesophageal) procedures

For standard risk patients who

have not received Penicillin/

Cephalosporin in the last two

weeks and are not on long termPenicillin, Amoxycillin as per

previous dosages.

For all other patients including high

risk, discuss with Paediatrician/

Physician/Cardiologist.




7

18. Glossary

2DE.............................................. 2-dimensionalechocardiographyalt................................................ alternative

ANA............................................. antinuclearantibody

anti-DNase B.............................. antideoxyribonucleaseB

ARF............................................. acuterheumaticever

ASO............................................. antistreptolysinO

BP................................................ bloodpressure

BPG............................................. benzathinepenicillinG

CRP............................................. C-reactiveprotein

CSANZ........................................ CardiacSocietyoAustraliaandNewZealand

ECG............................................. electrocardiogram

Echo............................................ echocardiography

ESR............................................. erythrocytesedimentationrate

GAS............................................. groupAstreptococcus

HR............................................... heartrate

IM................................................ intramuscular

INR.............................................. internationalnormalisedratio

IV................................................. intravenous

mU............................................... megaunits

NHF............................................. TheNationalHeartFoundationoNewZealand

NHI.............................................. NationalHospitalIndex

NSAID.......................................... non-steroidalanti-infammatorydrug

PANDAS...................................... paediatricauto-immuneneuropsychiatricdisordersassociatedwithstreptococcalinections

PO............................................... peroral

RAST........................................... RadioAllergoSorbentTest

RHD............................................. rheumaticheartdisease

ULN............................................. upperlimitsonormal

WHO............................................ WorldHealthOrganisation




75

[19. Notes]




7




NHF0239 80pp Cover 4C.indd 4 21/6/06 12:12:43



Endorsed by:



Cardiovascular disease is the leading cause of death in New

Zealand, accounting for 40 percent of all deaths annually (approx.

10,500 people).

Since its inception in 1968, the Heart Foundation has played a majorrole in reducing the high incidence of death from cardiovasculardisease, including:

• Funding vital heart-related medical and scientific research in

New Zealand

• Working with at-risk groups through intervention programmes

• Supporting and implementing cardiac rehabilitation programmes

• Working with food industry groups to promote healthier foods• Providing education programmes promoting healthy eating and

physical activity

• Providing heart health resources to health professionals and the

general public

• Working with Pacific people through Pacific Islands Heartbeat (PIHB).

Without the generosity of New Zealanders’ donations and legacies, theHeart Foundation could not achieve many of these goals. Any help youcan give is greatly appreciated.

For more information on heart health and/or

supporting the Heart Foundation, visit our websitewww.heartfoundation.org.nz or please contact:

The National Heart Foundation of New Zealand

PO Box 17-160, Greenlane, Auckland, 1546

Tel: 0064 9 571 9191

Fax: 0064 9 571 9190

Email: [email protected]

Published June 2006

ISBN: 0-9582743-0-4

rheumatic fever

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