Andersons pediatric cardiology 1466

MedicalManagementofChronic
RheumaticHeartDisease
BenzathinePenicillinG
Secondaryprophylaxis,thecontinuousadministrationofbenzathinepenicillinG
intheformofthreetofourweeklyintramuscularinjections,remains
fundamentaltoRHDmanagement.56,57ItpreventsARFrecurrencesandhalts
theprogressionofRHD;insomecircumstances,italsoallowsforresolution.
AlthoughbenzathinepenicillinGisoneoftheoldestandcheapestantibiotics,
beingontheWorldHealthOrganization'slistofessentialmedicines,there
continuestobeaworldwideshortage.58Manufacturingandqualityassurance
protocolsarelackingorinsufficient.58
SecondaryprophylaxisisbestdeliveredaspartofacomprehensiveregistrybasedRHDpreventionprogram.56YetinmuchoftheworldwhereRHDremains
endemic,nosuchprogramsexist;consequentlytheoveralldeliveryoflong-term
antibioticstopreventARFrecurrencesremainssuboptimal.59Therecommended
durationofsecondaryprophylaxisisdetailedinTable55.1.
Table55.1
DurationofSecondaryProphylaxisforRheumaticFeverand
RheumaticHeartDisease
DurationofProphylaxis
WHO
PatientCategory
Recommendations56
ARFonly
For5yearsafterthelast
attack,oruntil18yearsold
(whicheverislonger)
ARFwithcarditis
For5yearsafterthelast
butnoresidualheart attack,oruntilage18years
disease
(whicheverislonger)

MildRHD
For10yearsafterthelast
attack,oratleastuntilage
25years
ModerateRHD
For10yearsafterthelast
attack,oratleastuntilage
25years
SevereRHD,
Lifelong
includingthosewho

AustralianRecommendations53
Minimumof10yearsaftermost
recentepisodeofARForuntilage21
years(whicheverislonger)
Minimumof10yearsaftermost
recentepisodeofARForuntilage21
years(whicheverislonger)
Minimumof10yearsaftermost
recentepisodeofARForuntilage21
years(whicheverislonger)
Continueuntilage35years

Continueuntilage40yearsorlonger

AHA/ACC
Recommendations39
5yearsoruntilpatient
is21yearsold

(whicheverislonger)
10yearsoruntil
patientisage21years
(whicheverislonger)
10yearsoruntil
patientisage40years
(whicheverislonger)
10yearsoruntil
patientisage40years
(whicheverislonger)
10yearsoruntil
patientisage40years


arepostintervention

(whicheverislonger)

ACC,AmericanCollegeofCardiology;AHA,AmericanHeartAssociation;ARF,acuterheumatic
fever;RHD,rheumaticheartdisease;WHO,WorldHealthOrganization.

MedicalManagementofChronicValveDisease
PatientswithasymptomaticchronicRHDgenerallydonotbenefitfromheart
failuremedications.39Althoughmedicaltherapyforheartfailure,forexample
diuretics,angiotensin-converting-enzyme(ACE)inhibitors,andβ-blockers.May
amelioratesymptoms,thereisnoevidencethattheychangethenaturalhistoryof
chronicRHDinthesettingofnormalcontractility.39
Inpatientswithsymptomaticvalvardisease,cardiacmedicationshaveonlya
temporizingroleinoptimizingthehemodynamicprofilepriortosurgical
intervention.Inthissetting,theremaybearequirementtocombinemedications

withfluidrestrictionaswellasinotropicand/orventilatorysupport.39

AtrialArrhythmias
TheonsetofAFisfrequentlyassociatedwithabruptworseningofpulmonary
congestivesymptomsandmayleadtopulmonaryedemaand/orcardiogenic
shock.Thisistheconsequenceofashorteneddiastolicfillingtimeandlossof
atrialsystole,whichresultinincreasedleftatrialpressure,pulmonaryvenous
congestion,andlowcardiacoutput.Patientspresentingwithpulmonaryedema
orwithhemodynamicinstabilityrequirepromptelectriccardioversion.More
stablepatientscanbetreatedwithβ-blockers,calciumchannelblockers,or
amiodaroneiftheformertwoagentscannotbeusedorwereineffective.
Intravenousdigoxinislesseffectivebutisusefulinpatientswithlowblood
pressureorpoorventricularfunction.39,60
Instablepatients,ratecontrolisusuallythestrategyofchoice,asmaintenance
ofsinusrhythmisfrequentlydifficulttoachieve,giventhedegreeofleftatrial
hypertension,dilatationandfibrosisinthesepatients.Oralβ-blockersorcalcium
channelantagonistsarethedrugsofchoice.Thelatter,however,shouldbe
avoidedinpatientswithdepressedventricular(rightorleft)function.Digoxinis
reservedforpatientsinwhomβ-blockersorcalciumchannelantagonistsare
contraindicatedorwhentheventricularrateremainsuncontrolled.39,60
AlthoughAFisthemostcommonatrialtachyarrhythmiaencountered,a
minorityofpatientsmaysufferfromatrialtachycardiaoratrialflutter.Medical


treatmentissimilartothatforAF,butradiofrequencyablationmaybe
consideredinrefractorycases.Ontheotherhand,theroleofradiofrequency
ablationforAFcomplicatingrheumaticMVdiseaseisoftenlimitedtoa
concomitantprocedureatthetimeofMVsurgery.
AnticoagulationwithavitaminKantagonistismandatoryinpatientswithMV
diseaseandAForflutter,giventheirveryhighriskofstroke.Patientswitha

prioremboliceventorwithaleftatrial/leftatrialappendagethrombusarealso
indicatedforanticoagulation.Anticoagulationmaybealsobeneficialinpatients
withsignificantleftatrialdilatationorspontaneousechocontrast,butthereis
currentlyinsufficientevidencetomakethisrecommendation.Neweroral
anticoagulantshavenotbeenstudiedinpatientswithrheumaticMVdisease,and
vitaminKantagonistsremaintheonlyapprovedanticoagulantinthissubsetof
patients.39,60

PregnancyandAnticoagulation
Pregnancyisassociatedwithaphysiologicincreaseincardiacoutput,which
resultsinanexponentialincreaseingradientsacrossstenoticvalvesaspredicted
fromtheGorlinequation.Pregnancyisalsoassociatedwithanincreaseinheart
rate,whichreducesthediastolicfillingtime.Collectivelythesechangesleadto
worseningofsymptoms,andadequateheartratecontroliscrucialtoavoid
clinicaldeterioration.Thiscanbeachievedwiththeuseofβ-blockers
(preferablywithselectiveβ-1action)ordigoxin.Restrictionofphysicalactivity
anddiureticsareoftennecessarytomitigatesymptoms,butoverdiuresisshould
beavoidedtopreventplacentalhypoperfusion.Pregnancyisalsoa
hypercoagulablestate;thereforemeticulousanticoagulationinpatientswith
AF/flutterisrequired.Approvedregimensincludeunfractionatedheparinor
low-molecular-weightheparin(withdailymonitoringoffactorXalevels)inthe
firsttrimesterfollowedbywarfarinuntilthe35thweekofgestation,then
switchingbacktoheparinuntildelivery.Low-dosewarfarin(<5mg)is
associatedwithalowriskofembryopathyandisaviablethirdoptionifthe
targetinternationalnormalizedratioisreachedwithsuchlowdoses.The
regimenofchoiceshouldbedecidedonafterthoroughdiscussionwiththe
patientregardingtheprosandconsofeachoftheaforementionedalternatives.61