Andersons pediatric cardiology 292

heartdisease.JAmCollCardiol.2002;39:1890–1900.)

FIG.13.4 Associationofthereportedprevalenceofventricularseptal
defects(VSD)tototal(left),totalexclusiveofventricularseptaldefects
(middle),andcyanoticcardiacdisease(right),asassessedfromreported
studies.(FromHoffmanJI,KaplanS.Theincidenceofcongenitalheart
disease.JAmCollCardiol.2002;39:1890–1900.)

Table13.9
MeanandMedianPrevalenceofSpecificCongenitalCardiac
LesionsforEachOneMillionLiveBirthsFromMultipleReports,the
NewEnglandRegionalInfantCardiacProgram,andtheBaltimoreWashingtonInfantStudy
Lesion
Ventricularseptaldefect
Patentarterialduct
Atrialseptaldefect
Atrioventricularseptaldefect
Pulmonarystenosis
Aorticstenosis
Coarctationoftheaorta
TetralogyofFallot
Transposition
Hypoplasticrightheart
Tricuspidatresia
Ebsteinmalformation
Pulmonaryatresiawithintact
ventricularseptum
Hypoplasticleftheart
Commonarterialtrunk
Double-outletrightventricle
Double-inletventricle

Numberof
Studies
43
40
43
40
39
37
39
41
41
32
11
5
11
36
30
16
23

3570
799
941
348
729
401
409
421
315

222
79
114
132

Lower
Quartile
1757
324
372
242
355
161
289
291
231
105
24
38
76

266
107
157
106

154
61
82
54

Mean

2829
567
564
340
532
256
356
356
303
160
92
40
83

Upper
Quartile
4482
782
1059
396
836
388
492
577
388
224
118

161
147

NERICP
1975–77
345
135
65
110
73
41
165
196
218
–
56
12
69

BWIS
1981–89
1557
115
375
360
436
141
224
328
229

81
35
47
81

226
94
127
85

279
136
245
136

163
30
32
54

184
56
95
54

Median


Totallyanomalouspulmonary
venousconnection

Allcyanotic
Allcongenitalcardiacdiseasea
Bifoliateaorticvalve

25

94

60

37
43
10

1391 1078
9596 6020
13,556 5336

91

120

58

66

1270
7669
9244

1533
10,567
13,817

888
2,033
–

–
–
93

aExcludingbifoliatenonstenoticaorticvalves,isolatedpartiallyanomalouspulmonaryvenous

connection,andsilentarterialducts.
BWIS,BaltimoreWashingtonInfantStudy;NERICP,NewEnglandRegionalInfantCardiac
Program.
ModifiedfromHoffmanJI,KaplanS.Theincidenceofcongenitalheartdisease.JAmCollCardiol.
2002;39:1890–1900;ReportoftheNewEnglandRegionalInfantCardiacProgram.Pediatrics
1980;65:375–461;andFerenczC,RubinJD,LoffredoCA,Magee,CA.Epidemiologyof
CongenitalHeartDisease:TheBaltimore-WashingtonInfantStudy:1981–1989.Vol4.Mount
Kisco,NY:Futura;1993.

Anotherrecentsystematicreviewincluded114reports,comprisingatotal
studypopulationof24,091,867livebirths.Atotalof164,396casesofcongenital
heartdiseasewereidentified.Thereportedprevalenceincreasedsubstantially
overtime,from0.6per1000livebirthsinthe1930sto9.1per1000livebirths
after1995.Interestingly,overthepast15years,stabilizationoccurred.
Significantgeographicdifferenceswerefound.Asiareportedthehighest
prevalence,with9.3per1000livebirths,withrelativelymorepulmonary

outflowobstructionsandfewerleftventricularoutflowtractobstructions.The
prevalenceinEuropewassignificantlyhigherthaninNorthAmerica(8.2per
1000livebirthsvs.6.9per1000livebirths).Therewasalowerprevalencein
low-incomecountries.Accesstohealthcareisstilllimitedinmanypartsofthe
world,asarediagnosticfacilities,probablyaccountingfordifferencesin
reportedbirthprevalencebetweenhigh-andlow-incomecountries.43


AdultsWithCongenitallyMalformed
Hearts
Itispredictedthat83%ofpatientsbornwithcongenitallymalformedheartswill
nowsurvivetoadulthood.Projectionssuggestthatthiswillresultintheneedfor
follow-upformorethan200extracasesper100,000livebirthseachyear.44,45In
arepresentativepopulationin2000,therewereequalnumbersofchildrenand
adultsalivewithseverecongenitalcardiacdisease.44TheCongenitalCardiac
CentreforAdultsinToronto,Canada,reportedasignificantandsteadyfallinthe
meanageoftheirpopulationovertheperiodfrom1987to1997,reflectiveofthe
numberofyoungadultsreferredtotheircenterwhohadundergoneprevious
repairasopposedtopalliation.46Estimatesindicatethat,ofthepatients,25%
wouldhavecomplexlesions,morethanhalfwouldhavesignificantlesions,and
lessthan20%wouldhaveminorlesions.47Morbidityisstillrelativelyhighin
thispopulation,especiallyforthosewithcomplexdefects.OverallKaplan-Meier
estimatesofmortalityafter5yearsforpatientswithacongenitalcardiacdefect
aftertheageof17havebeenreportedat3.0%toashighas12.6%forpatients
withcyanoticdefects,and8.2%forpatientswiththeFontancirculation.48
AnotherstudyusedtheCanadianprevalencedatatostatisticallymodelthe
prevalenceofcongenitallymalformedheartsintheUnitedStatesafteradjusting
forraceandethnicity.Thestudyestimatedthatinyear2010,therewere2.4
millionpeopleintheUnitedStateswithcongenitalheartdisease;ofthem,1.4
millionareadultsand300,000hadseveredisease.49Thereforetheoverallpoint

prevalenceofcongenitalcardiacdiseasehasandwillcontinuetoincrease,with
thespectrumshiftingtowardadultswithrepairedorpalliatedlesions.Thiswill
haveanimportantimpactonincreasingtheneedforspecializedcare,with
servicesneedingtoexpandtomeetthisgrowingneed.
Withtheincreaseinprevalence,tertiarycarecenterswithadultcongenital
cardiologyclinicshaveseenincreasesofuptothreefoldinworkloadbetween
1992and1997.46Annualratesofhospitalizationforpatientswithallother
congenitalcardiacdefectswasalsonotinconsequential.50Expenditureshave
beenreportedtobehigherforadultswithcongenitallymalformedheartsthanfor
thegeneralpopulation.51Withthegreaterpotentialneedforreoperationandcare
duringfollow-up,aneconomicandlogisticalburdencanbeexpectedinthe