Andersons pediatric cardiology 2254
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Abstract
Theoverwhelmingmajorityoftheworld'schildrenliveinlow-andmiddleincomecountries(LMICs).Thecombinedburdenofcongenitaland
acquiredheartdiseaseinLMICsisconsiderable.Significantchallengesare
presentedbydysfunctionalhealthsystems,shortfallofhumanandmaterial
resources,andsignificantcomorbiditiesthatincludeundernutritionand
infections.Thesechallengesrequireapproachesthatareoftenquite
differentfromthestandardpracticesofpediatriccardiaccareinhighincomenations.Thischapteridentifiescommonchallengesinpediatric
cardiaccareinLMICsandexaminessomeofthesolutionsthathave
emergedfromprogramsthathavebeenestablishedinLMICs.
Keywords
Developingcountries;Lowresourceenvironments;Cost-effective
practices
Anestimated15millionchildrendieorbecomedisabledannuallybytreatableor
preventableheartdiseaseinlow-andmiddle-incomecountries(LMICs).Sadly,
for90%ofchildrenwithheartdisease,treatmentiseitherunavailable,
unaffordable,orofsuboptimalquality.Globaleffortstoreducemortalityin
childrenyoungerthan5yearshavefocusedonreducingdeathfrom
communicablediseasesinLMICs,withlittletonoattentionfocusingon
pediatriccongenitalheartdisease(CHD)andacquiredheartdisease.Lackof
awarenessofCHDandacquiredheartdisease,accesstocare,poorhealthcare
infrastructure,competinghealthpriorities,andacriticalshortageofspecialists
areimportantreasonswhypediatricheartdiseasehasnotbeenaddressedinlowresourcedsettings.1
GlobalBurdenofPediatricHeartDisease
Heartdiseaseinchildrenhasnotmadeanyimpressionontheglobalhealth
agenda;henceitislittlewonderthat90%ofthechildrenwithheartdisease
neverreceivethecaretheyrequire.Amidanarrativedominatedbytheacquired
heartdiseasesofadults,predominantlylifestylerelated,itisthereforenow
importanttoillustratethefullextentoftheproblemsofheartdiseaseinchildren,
definedasapersonyoungerthan18years.2
Thereare,broadlyspeaking,twogroupsofheartdiseaseinchildren,either
congenitaloracquiredheartdiseases.TheCHDgroupembracesallmannerof
heartdefectspresentatembryologicdevelopmentoftheheart,throughfetallife
andarepresentatbirth.Ofcourse,theymayalsobepresentinthecaseofa
spontaneousabortionorstillbirth,andweknowthatCHDisanimportantcause
ofboth.However,includedintheCHDdefinitionarealsostructuresnormalin
fetallife,whichoughttocloseafterbirthbutinsomecaseshavepersistedinto
earlychildhood.Thuspersistenceofthearterialductoraninteratrialdefectis
includedinourdefinition.TherearemanyadultslivingwithCHD,andinwellresourcedcountriesthereisevidencetoshowthatthroughinterventionsand
surgerywithimpressivesurvivalrates,therearenowmoreadultswithCHDthan
therearechildren.3Arrhythmogenicdiseasemaynotalwaysfitperfectlyinto
eithergroup.CertainlythegeneticsoftheprolongedQTsyndrome,havingbeen
welldescribed,shouldbeincludedasaformofCHD.ThisislikewiseforWolffParkinson-Whitesyndrome,eventhoughpatientsinvariablybecome
symptomaticyearsafterbirth.Acquiredheartdiseaseinchildrenincludes
rheumaticheartdisease(RHD),Kawasakidisease,Chagasdisease,
cardiomyopathy,myocarditis,pericarditis,infectiveendocarditis,andother
infectionsoftheheart.Thissectionfocusesondescribingtheglobalburdenof
congenitalandRHDbecausetheycontributetotheoverwhelmingmajorityof
burdenofpediatricheartdisease.Theremainingconditionshavenotbeen
adequatelystudiedfromtheperspectiveofdefiningtheglobalburden.
GlobalBurdenofCongenitalHeartDisease
Whatismeantbytheterm“burdenofdisease”?Tosomeitintuitivelymeansthe
prevalenceorincidenceofadisease.Thatbeingthecase,whenconsidering
CHDitwouldseemtobeasimplematterofreviewingallthestudiesof
prevalence,includingbirthprevalence,showingthatCHDoccursin
approximately8per1000live-bornbabies,usingestimatesoftheglobal
population(9billion)andcalculating72millionpersonswithheartdiseaseor1
millionbornwithCHDperyear.
Tothebestofourknowledge,however,forseveralreasonsrelatedto
ascertainmentandattrition,thissimplemethodologyislikelyan
oversimplification.Astudyof20,307newbornsinIndiafoundanoverallCHD
birthprevalenceratesimilartothatreportedworldwide,at8.07per1000live
births(95%confidenceinterval[CI],6.94to9.4).4AnotherstudyfromChina
showedsimilarresults,with686havingCHDinacohortof84,062births,
resultinginanoverallincidenceof8.2per1000totalbirths.5
OurtaskismadesomewhateasierbytherecentGlobalBurdenofDisease
(GBD)StudyundertakenbytheInstituteforHealthMetricsandEvaluation.This
isthemostcomprehensiveworldwideobservationalepidemiologicstudyto
date.6Itdescribesmortalityandmorbidityfrommajordiseases,injuries,andrisk
factorstohealthatglobal,national,andregionallevels.Theprevalenceof
diseaseatbirthandatotheragesisakeydescriptortobedrawnfromthiswork.
TheGBDStudyisthemostimportantstatisticalandepidemiologicresource
availableandincludesCHDandacquiredheartdiseases.
TheInstituteforHealthMetricsandEvaluationhassystematicallyreviewed
allavailabledatasourcesonmorbidityandmortalityofCHDfrom195countries
andterritoriesforinclusioninepidemiologicmodels.Usingthepublicly
availableGBD-Comparevisualizationtool,thetopcausesofinfantdeathsfrom
1990to2016havebeenidentifiedforvariousregionsoftheworldandclassified
onbasisoftheirsociodemographicindex(SDI).7
AnnualglobalinfantCHDmortalityisestimatedtobe142,917(95%CI,
126,267to164,297).Communicablediseasesaretheleadingcauseofdeathin
infantsinlowandlow-middleSDIcountries.InhighSDIregions,congenital
anomaliesandsuddeninfantdeathsyndromearetheleadingcausesofdeath.
From1990to2016thedeathrateduetocommunicablediseasesdecreasedby
50%inlowSDIregionsand75%inmiddleSDIregions.Thedeathratefrom
CHDdecreasedby60%inhighSDI,40%inmiddleSDI,and20%inlowSDI
regions.ThedeathrateinlowSDI(62per100,000)andmiddleSDI(87per
100,000)regionsremainselevatedcomparedwithhighSDIregions(20per
100,000).CHDisnowthefifthleadingcauseofdeathininfantsglobally.
Despitealowerabsolutedeathrate,CHDaccountsforahigherproportionof
deathsinmiddle(13%)andhigh-middle(16%)SDIregions.7Ifthistrend
