Congenitalheartmalformation:Prevalence,severity,survival,andqualityoflife先天性心脏病流行病学Thelowestvalues/1000livebirthsAuthor‰RegionFyler2.10NewEngland,USAGardiner2.17Toronto,CanadaAkman3.20Bogalusa,USAMacMahon3.20Birmingham,UKMckeown4.20Birmingham,UKBosi4.60ItalyBierman4.70HawaiiPrevalenceofcongenitalheartmalformationatbirththehighestvalues/1000birthsauthor‰RegionFischer8.20Tyrol,AustriaKerrebijn8.26Leiden,NetherlandGrech8.80MaltaHoffmann8.82SanFrancisco,USAMeberg9.02NorwayNakazawa10.00JapanManetti12.30Florence,ItalyYerushalmi11.70California,USAPrevalenceofcongenitalheartmalformationatbirthTheoldestsurveys/1000livebirthsAuthorDatePrevalence(‰)Macmahon1940—493.23Carlgren1941–506.35Harris1944—505.90Pleydell1944—573.20Landtman1945–638.00Richards1946—535.50Gardiner1948—492.17Mustachi1949—515.88PrevalenceofcongenitalheartmalformationatbirthRecentdata/1000livebirthsAuthorDatePrevalence(‰)Grech1990-948.80Robida1984-9412.23Bosi1992-934.60Anderson1987-9010.20Cerboni1988-906.08Ferencz1981-894.23Pexieder1986-876.04Meberg1982-889.02PrevalenceofcongenitalheartmalformationatbirthPrevalenceofcongenitalheartmalformationatbirthRegionaldifferences/1000livebirthsRegionMeanRangedifferenceplaceEurope6.673.20Birmingham,UK12.30Florence,ItalyUSA5.763.20Bogalusa8.56SanFranciscoCanada3.972.17Toronto5.54AlbertaOthercountries8.976.08GuadeloupeYearlyfluctuationinBohemiandatafrom1980through1989YearNo.ofmalformationPrevalence/100019806467.0419815476.3919825686.7719835486.7319845666.9719854956.1619864365.5319874555.8219884025.1119893674.80PrevalenceofcongenitalheartmalformationatbirthTheprevalencewashighestinOctober,andlowestinDecember,JuneandJuly.Thepeakoftheprevalencecoincidedwiththeepidemicsofinfluenzainearlypregnancy8and9monthspreviously.TherewasadefiniteseasonalvariationRatioofboystogirlsBoystogirlswithCHD:51.9%to48.1%Ahigherproportionofboyswasfoundwith:DORV(2.68:1);HLHS(2.25:1)cTGA(2.11:1);Ahigherproportionofgirlswasfoundwith:PDA(1:1.66);Ebstein’smalformation(1:1.57)Commonarterialtrunk(1:1.22)DistributionofcongenitalheartmalformationVSD41.59%ASD8.67%Aorticstenosis7.77%Pulmonarystenosis5.81%cTGA5.39%Aorticcoarctation5.29%PDA5.07%Greaterthan5%oftheoverallnumberofmalformationDistributionofcongenitalheartmalformationAVSD4.00%HLHS3.42%TOF3.36%DORV,commonarterialtrunkandpulmonaryatresia1.05----1.37%TAPVC0.80%Tricuspid0.78%Ebstein’malformation0.44%Interruptedaorticarch0.44%Spontaneousclosurein576of2092recognizedventricularSeptaldefects,accountingfor27.5%ofthecasesCriticalheartmalformationinneonates,10mostfrequentNumberproportionoftotalMalformationsnumberseen(%)cTGA10284Pulatresia3974Coarctation3449HLHS3494Tricuspidatresia1377DORV2050VSD88CommonAT850TOF728Criticalheartdisease:whichthreatenlife,whichmanifestedbyseverveCongestiveheartfailure,deepcyanosis,orbothsymptoms0%5%10%15%20%25%一个月以内一岁以内五岁以内15岁以内死亡率DeathrateofchildrenbornwithCHDSurvivalinmostfrequentcardiacmalformationCHDNumbersurvival(%)atageof1515yrsVSD2097919089ASD436949393Aorticstenosis391908988PS292969696cTGA271625654Coarctation266686565PDA255919191AVSD201625554HLHS172000TOF169847877Questionnaires,1564例8-13岁先心病患儿生存质量调查•83.2%未用任何药•3.2%体能差•59.5%可参加娱乐消遣性活动•6.7%可参加竞争性的运动•35.5%上学延迟•6.9%学习差•身高及体重与同龄人无区别•先心病儿给父母造成心理紧张生活质量
