Andersons pediatric cardiology 2257

formativeyears.IntheeventCHDissuspected,facilitiesforaccuratediagnosis
intheformofechocardiographymachineswithpediatricandnewborn
transducersandskilledpersonnelarequitelimited.Itisalsonotuncommonfor
echocardiogramstobeperformedbyadultcardiologists.Asaresult,erroneous
reportsarecommon.
Therearemanybarrierstothetimelyreferralofpatientstoapediatricheart
programevenwhenCHDissuspectedorrecognized.Primarycaregivers,
includingpediatricians,maynotoftenperceivetheurgencyinreferringofmany
criticalCHDs.ThereisalsoageneralperceptionthattreatingCHDisafutile
exercise,contributingtofailureoftimelyreferral.Geographicbarriersresult
fromasevereshortfallofcomprehensivepediatricheartprogramsinLMICs.
Mostprogramsarelargelyclusteredinandaroundlargecities.19,21Geographic
barriersincombinationwithpovertyandignoranceconspiretoensurethatmost
newbornswithheartdiseasearenotreferredinatimelyfashion.Thisis
particularlytrueformostofAfrica,24largepartsofeasternandnorthernIndia,
Afghanistan,Pakistan,Iraq,andthecentralAsiancountries.Insomecountries,
suchasIndiaandChina,thereisgenderbiasfavoringboys,andthisisa
significantbarriertogirlswithCHDgettingattention.25
Becausetherearenoorganizedsystemsfortransportingasicknewbornor
infantwithheartdiseaseinLMICsettings,manyneonateswithCHDdevelop
sepsisandend-organinjurypriortoreachingapediatriccardiacprogram.26This
significantlyimpactsmortalityandmorbidity.Thevastdistancesthatneedtobe
coveredtoreachapediatricheartprogramfurthercompoundthesechallenges.In
addition,ambulanceservices(roadorair)fornewborntransportareexpensive
andgenerallybeyondthereachofmostfamiliesinLMICs.Fornewbornswith
criticalCHD,reachingatertiarycarecenterwhileincirculatorycollapseposes
notonlymedicalchallengesbutalsoethicalchallengeswhenthereisevidenceof
majorneurologicinsult.
ThepresentcombinedcapacityofallpediatricheartprogramsinLMICsfalls
woefullyshortoftheactualrequirements.17,19,21Mostprogramsare
overwhelmed.Programsthatprovidecareatsubsidizedcostsoftenhavelong

waitinglistsofseveralthousandpatients.Programsinprivatehospitalsare
expensiveandunaffordablefortheaveragefamily.Nevertheless,eventhese
unitsarefacingthepressureofincreasingnumbersofpatientswithfinancial
means,aswellasdemandfromoverseaspatientsseekingbetterpediatriccardiac
care.


AcquiredHeartDiseases
ChildrenwithacquiredheartdiseasehavesimilarneedsfortheircareasCHDin
termsofinfrastructureandresources.
RheumaticHeartDisease(SeeAlsoChapters54and55).
RHDislargelyadiseaseofthepoor,underprivileged,andmarginalized
populationswhooftenescapethegambitofhealthservices,particularlywhenit
isprivatized.18AdetaileddiscussionontheburdenofRHDisprovidedin
Chapter89.Chapter55describestheepidemiologyofRHD.Treatmentof
establishedRHDcanbeeffectivelyundertakenonlyincomprehensivepediatric
cardiacfacilitiesandashortfallinprogramsandpersonnelislikelytoaffectthe
qualityofcareandoutcomes.
KawasakiDisease(SeeAlsoChapter53).
KawasakidiseaseislikelytobeunderreportedinLMICsettingsbecauseitis
likelytobeunrecognized.27Giventheneedforcloseclinicalmonitoring,
advancedimaging,andexpensivemedications,healthsystemdeficienciesare
likelytoadverselyimpactchildrenwithKawasakidiseaseinLMICs.Delaysin
administrationofintravenousimmunoglobulinarelikelytotranslateintoa
higherincidenceofcoronaryaneurysmsandrelatedcomplications.
MyocarditisandCardiomyopathy(SeeAlsoChapters61and63).
Thefundamentalrequirementofpediatricintensivecareservicesforcareof
patientswithadvancedheartfailureisinveryshortsupplyinLMICs.28
Advancedlifesupportsystemssuchasextracorporealmembraneoxygenation
andventricularassistdevicesareextremelyscarceandlargelyunaffordable.

PediatrichearttransplantisnotaviableoptioninmostLMICsbecauseofvirtual
absenceoforgandonationnetworksandlimitationsininfrastructureand
expertise.Furthermore,thecumulativecostsofmanagingapatientwithcardiac
transplantissimplytooprohibitiveforthefragilehealthsystems.

PatientProfileofCongenitalHeartDiseasein
Low-andMiddle-IncomeCountries
ThepatientprofileofCHDthatistypicallyseeninLMICsisuniquebecauseof
thepreviouslylistedfactorsthatcontributetolatepresentation.Inaddition,


commonchildhoodconditionsthatprominentlyincludeundernutritionand
respiratoryinfectionsfrequentlycomplicatetheclinicalpresentationofCHD.
Thissectionwilldiscussthesespecificchallengesandsuggestwaystoapproach
them.

LatePresentation
Delaysinpresentationresultinvariedmanifestationsdependingontheageand
specificcondition.
CriticalHeartDiseasesinNeonates.
Neonateswithcriticalheartdiseaseareespeciallyvulnerabletodelaysin
diagnosisandreferral.Inaddition,theyarealsoparticularlyvulnerableto
clinicaldeteriorationduringtransport.Inabsenceofroutinescreeningand
becauseoflimitationsinsupervision,CHDisoftennotdiagnosedwhilethe
newbornisinhospitalforthe24to48hoursfollowingdelivery.22CriticalCHD
isoftenidentifiedafterdischarge,whenphysiologicperturbationsareclinically
manifest.Thenewbornistypicallybroughtbacktoahospitalfollowing
discharge,withvaryingdegreesofinsultintheformofhypoxia,end-organ
injury,and/ormetabolicderangement.Inaddition,theclinicalpictureisoften
furthercomplicatedbythecommonoccurrenceofneonatalsepsiswithan

increasingandalarmingtrendtowardmultidrugresistantbacterialandfungal
infections.29Althoughitislikelythatasubstantialnumberofnewbornswith
CHDdonotsurvive,theactualproportionofnewbornswhodiebecauseof
untreatedCHDhasnotbeendeterminedintheLMICenvironment.Among
survivors,neurologicinsultislikelyandawidespectrumofpossibilitiesranging
fromsubclinicalinsulttoovertmanifestationsofhypoxicischemic
encephalopathycanoccur.GiventhefactthatlatediagnosisofCHDisnot
uncommoneveninadvancednationswithmatureandwell-resourcedhealth
systems.22InLMICsmanymorenewbornswithCHDpresentarelatewhencare
iscomplicatedandfrequentlynolongerpossible.Theexactconsequencesthat
wouldresultfromlatepresentationvarydependingonthespecificCHD.Table
88.2liststheseconsequencesinthecommonCHDcategoriesinthenewborn.
Table88.2
ConsequencesofDelayinIndividualNeonatalHeartDiseaseCategories