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1、Congenital heart malformation: Prevalence, severity, survival, and quality of life先天性心脏病流行病学先天性心脏病流行病学(li xn bn xu)第一页,共十七页。The lowest values/1000 live birthsAuthor Region Fyler 2.10 New England, USAGardiner 2.17 Toronto, CanadaAkman 3.20 Bogalusa, USAMacMahon 3.20 Birmingham, UKMckeown 4.20 Birming

2、ham, UKBosi 4.60 ItalyBierman 4.70 HawaiiPrevalence of congenital heart malformation at birth第二页,共十七页。the highest values/ 1000 births author Region Fischer 8.20 Tyrol,AustriaKerrebijn 8.26 Leiden , NetherlandGrech 8.80 MaltaHoffmann 8.82 San Francisco, USAMeberg 9.02 NorwayNakazawa 10.00 JapanManett

3、i 12.30 Florence, ItalyYerushalmi 11.70 California,USAPrevalence of congenital heart malformation at birth第三页,共十七页。The oldest surveys/ 1000 live birthsAuthor Date Prevalence () Macmahon 194049 3.23Carlgren 1941 50 6.35Harris 194450 5.90Pleydell 194457 3.20Landtman 1945 63 8.00Richards 194653 5.50Gar

4、diner 194849 2.17Mustachi 194951 5.88Prevalence of congenital heart malformation at birth第四页,共十七页。Recent data / 1000 live birthsAuthor Date Prevalence() Grech 1990-94 8.80Robida 1984-94 12.23Bosi 1992-93 4.60Anderson 1987-90 10.20Cerboni 1988-90 6.08Ferencz 1981-89 4.23Pexieder 1986-87 6.04Meberg 19

5、82-88 9.02Prevalence of congenital heart malformation at birth第五页,共十七页。Prevalence of congenital heart malformation at birthRegional differences/ 1000 live birthsRegion Mean Range difference place Europe 6.67 3.20 Birmingham, UK 12.30 Florence, ItalyUSA 5.76 3.20 Bogalusa 8.56 San FranciscoCanada 3.9

6、7 2.17 Toronto 5.54 AlbertaOther countries 8.97 6.08 Guadeloupe第六页,共十七页。 Yearly fluctuation in Bohemian data from 1980 through 1989Year No. of malformation Prevalence/ 1000 646 7.04 547 6.39 568 6.771983 548 6.73 566 6.97 495 6.16 436 5.53 455 5.82 402 5.111980 367 4.80 Prevalence of congenital hear

7、t malformation at birth第七页,共十七页。 The prevalence was highest in October, and lowest in December ,June and July. The peak of the prevalence coincided with the epidemics of influenza in early pregnancy 8 and 9 months previously.There was a definite seasonal variation第八页,共十七页。Ratio of boys to girlsBoys

8、to girls with CHD: 51.9% to 48.1%A higher proportion of boys was found with : DORV (2.68: 1); HLHS (2.25 :1) cTGA ( 2.11 :1); A higher proportion of girls was found with:PDA (1 :1.66); Ebsteins malformation (1 :1.57)Common arterial trunk (1:1.22)第九页,共十七页。Distribution of congenital heart malformation

9、VSD 41.59%ASD 8.67%Aortic stenosis 7.77%Pulmonary stenosis 5.81%cTGA 5.39%Aortic coarctation 5.29%PDA 5.07% Greater than 5% of the overall number of malformation第十页,共十七页。Distribution of congenital heart malformation AVSD 4.00% HLHS 3.42% TOF 3.36%DORV, common arterial trunk and pulmonary atresia 1.0

10、5-1.37%TAPVC 0.80%Tricuspid 0.78%Ebstein malformation 0.44%Interrupted aortic arch 0.44%第十一页,共十七页。Spontaneous closure in 576 of 2092 recognized ventricular Septal defects, accounting for 27.5% of the cases第十二页,共十七页。Critical heart malformation in neonates,10 most frequent Number proportion of total M

11、alformations number seen(%)cTGA 102 84Pul atresia 39 74Coarctation 34 49HLHS 34 94Tricuspid atresia 13 77DORV 20 50VSD 8 8Common AT 8 50TOF 7 28Critical heart disease: which threaten life,which manifested by severve Congestive heart failure,deep cyanosis, or both symptoms第十三页,共十七页。Death rate of chil

12、dren born with CHD第十四页,共十七页。Survival in most frequent cardiac malformation CHD Number survival (%) at age of 1 5 15yrsVSD 2097 91 90 89ASD 436 94 93 93Aortic stenosis 391 90 89 88PS 292 96 96 96cTGA 271 62 56 54Coarctation 266 68 65 65PDA 255 91 91 91AVSD 201 62 55 54HLHS 172 0 0 0TOF 169 84 78 77第十五页,共十七页。Questionnaires, 1564例例8-13岁岁先心病患儿生存先心病患儿生存质量质量(zhling)调查调查 83.2% 未用任何药未用任何药 3.2% 体能差体能差 59.5% 可参加娱乐消遣性活动可参加娱乐消遣性活动 6.7%可参加竞争性的运动可参加竞争性的运动 35.5% 上学延迟上学延迟 6.9% 学习

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