Kate Best 1 , Judith Rankin - PowerPoint Presentation

1. Kate Best1, Judith Rankin1Long-term survival of children born with congenital heart disease: a systematic review1 Institute of Health & Society, Newcastle University, UK

2. BackgroundObjectives of PhDDescribe the prevalence of CHD, trends and risk factors using BINOCAR data.Conduct a systematic review on the long-term survival of children born with CHD.Analyse survival and predictors of survival of children born with CHD using BINOCAR data linked to death registrations.Predict the future prevalence/survival of CHDs.

3. BackgroundPrevalence of CHD: 80 per 10,000 total births

4. Infant survival has been frequently reportedBut after infancy, mortality remains higher than that of the general populationbackground

5. Individuals with CHD require highly specialised healthcareLong-term follow-up of children born with CHD is important in predicting life time healthcare requirements background

6. Previous researchIn 2008, a systematic review on the long-term prognosis of CHD was published BUTit included hospital-based studiesSurvival %97.299.698.195.496.995.793.598.587.497.590.296.892.9

7. AimTo conduct a systematic review of all population-based studies that report the long-term survival of individuals born with CHD.Compare survival estimatesExamine predictors of survival

8. methodsMEDLINE, EMBASE and Scopus from their inception to October 2013Inclusion criteria:Population-based studiesCases ascertained at birthSurvival estimates reported at age ≥5 Survival estimates correspond to all CHD combined or by subtype

9. methodsExclusion criteriaStudies not available from the British LibraryStudies not written in English

10. methodsSearch strategy:MeSH-terms (e.g. “exp Heart Defects, Congenital/ep, mo”) and key words (e.g. “congenital” and “heart” or “cardiac”)Core journals & reference lists were searchedTitles and abstracts were screened according to the inclusion criteria and irrelevant citations were excluded. Full articles were then retrieved.  

11. methodsData extractionTwo data extractorsStudy descriptions extractedKaplan-Meier survival estimates and 95% confidence intervals (CIs) were extracted from each included study Authors contacted for clarification

12. methodsData summary/ AnalysisStudies that included and excluded cases with extra-cardiac anomalies grouped separatelySummary estimates were estimated using meta-analyses.

13. Results6,269 citation identified from electronic database searching1,178 duplicates5,091 titles reviewed88 abstracts reviewed35 full papers reviewed25 studies excluded10 were not population-based studies5 reported infant survival only3 reported mortality rates by year of death2 reported survival of all congenital anomalies only2 did not report survival estimates at the specified ages2 reported survival categorised by another variable only1 excluded cases of certain ethnicities10 studies included

14. ResultsDastgiri, Gilmour et al. 2003Fixler, Nembhard et al. 2010Frid, Bjorkhem et al. 2004Garne 2004Miller, Siffel et al. 2010Moons, Sluysmans et al. 2009Olsen, Christensen et al. 2010Samanek and Voriskova 1999Tennant, Pearce et al. 2010Wang, Hu et al. 201119701980199020002010Study periods

15. ResultsDastgiri, Gilmour et al. 2003Fixler, Nembhard et al. 2010Frid, Bjorkhem et al. 2004Garne 2004Miller, Siffel et al. 2010Moons, Sluysmans et al. 2009Olsen, Christensen et al. 2010Samanek and Voriskova 1999Tennant, Pearce et al. 2010Wang, Hu et al. 201119701980199020002010Follow-up periods

16. ResultsDastgiri, Gilmour et al. 2003Fixler, Nembhard et al. 2010Frid, Bjorkhem et al. 2004Garne 2004Miller, Siffel et al. 2010Moons, Sluysmans et al. 2009Olsen, Christensen et al. 2010Samanek and Voriskova 1999Tennant, Pearce et al. 2010Wang, Hu et al. 201119701980199020002010Follow-up periods2520152551051555Max age

17. ResultsFixlerDastgiriFridGarneMillerMoonsOlsenSamanekTennantWang

18. resultsStudyCHD subtypesDastgiri et al. 2003All subtypes combinedFixler et al. 2010Single Ventricle, Hypoplastic left heart, Pulmonary atresia, Tricuspid atresiaFrid, et al. 2004AVSDGarne et al 2004All subtypes combined and individuallyMiller et al. 2010AVSDMoons et al. 2009All subtypes combined and individuallyOlsen et al. 2010All subtypes combined and individuallySamanek et al 1999All subtypes combined and individuallyTennant et al. 2010All subtypes combined and individuallyWang et al. 2011Hypoplastic left heart, TGV, ToF, Common Trunkus, CoA, AVSD, Aortic atresia/stenosis

19. resultsStudyCHD subtypesDastgiri et al. 2003All subtypes combinedFixler et al. 2010Single Ventricle, Hypoplastic left heart, Pulmonary atresia, Tricuspid atresiaFrid, et al. 2004AVSDGarne et al 2004All subtypes combined and individuallyMiller et al. 2010AVSDMoons et al. 2009All subtypes combined and individually, including VSDOlsen et al. 2010All subtypes combined and individuallySamanek et al 1999All subtypes combined and individuallyTennant et al. 2010All subtypes combined and individuallyWang et al. 2011Hypoplastic left heart, TGV, ToF, Common Trunkus, CoA, AVSD, Aortic atresia/stenosis

20. Results: vsdTennantTennantTennantTennantGarneMoonsOlsenSamanekOlsen859095100510152025510152025Excluding extra-cardiac anomaliesIncluding extra-cardiac anomalies95% CISurvival estimatePercentage SurvivedAge

21. Results: AVSDFridMillerTennantFridMillerTennantFridTennantTennantFridGarneMoonsSamanekWangFridOlsenSamanekFridSamanekWangOlsenWang20406080100510152025510152025Excluding extra-cardiac anomaliesIncluding extra-cardiac anomalies95% CISurvival estimatePercentage SurvivedAge

22. Results AVSD20406080100510152025510152025Excluding extra-cardiac anomaliesIncluding extra-cardiac anomalies95% CISurvival estimatePercentage SurvivedAge69 (55-81)64 (48-79)68(41-89)65 (58-71)61(56-67)60(56-64)51(41-61)

23. Results: HLHFixlerTennantFixlerGarneMoonsSamanekWangSamanekSamanekWangWang020406080510152025510152025Excluding extra-cardiac anomaliesIncluding extra-cardiac anomalies95% CISurvival estimatePercentage SurvivedAge

24. Results: HLH020406080510152025510152025Excluding extra-cardiac anomaliesIncluding extra-cardiac anomalies95% CISurvival estimatePercentage SurvivedAge18(1-66)18 (3.5-39)9(8-59)

25. Results: all CHD subtypesDastgiriGarneTennantOlsenTennantTennantTennantOlsenGarneMoonsSamanekOlsenSamanekSamanekOlsen708090100510152025510152025Excluding extra-cardiac anomaliesIncluding extra-cardiac anomalies95% CISurvival estimatePercentage SurvivedAge

26. Results: all CHD subtypes708090100510152025510152025Excluding extra-cardiac anomaliesIncluding extra-cardiac anomalies95% CISurvival estimatePercentage SurvivedAge87 (86-88)84 (67-96)86 (72-96)76(74-79)

27. resultsSources of heterogeneityStudy periodAscertainment of milder formsAscertainment of cases with extra-cardiac anomaliesCoding of cases with multiple CHD

28. resultsPredictors of survivalYear of birth (5 studies)All 5 studies reported improved survival over timeSex (2 studies)No association (AVSD only) (Frid)Females increased risk of death (All CHD) (Wang)

29. resultsMaternal age at delivery (2 studies)No association (SV physiology) Increased survival with increasing maternal age (All CHD).811.21.4RR<2020-2425-2930-34 35+ Maternal age95% CIRR

30. Strengths & LimitationsStrengths:Restricted to population-based studiesSeparated studies including/excluding extra-cardiac anomaliesSystematic search strategyAuthors contacted2 data extractors

31. Strengths & LimitationsLimitations:Survival up to age 25 only4 studies up to age 5 onlyMost studies included cases with extra-cardiac anomaliesNo studies from low income populationsSmall sample sizes for individual subtypesDoesn’t account for morbidityLittle information on surgeries

32. Further researchFurther research is required into the long-term survival:Subtypes separatelyOf isolated cases of CHD in particular Predictors of survival (in particular socioeconomic position)Survival in low income populationsSurvival associated with surgeries

33. conclusionSurvival varies substantially by CHD subtypeFurther research into long-term survival and predictors is requiredThis information would inform health service planning and for informing parents when a CHD is detected antenatally or in early childhood

34. AcknowledgementsThank-you to the British Heart Foundation for funding this studyThank-you for listening!