Modelling Survival and Mortality Risk to 15 Years of Age for a National Cohort of Children with Serious Congenital Heart Defects Diagnosed in Infancy

Knowles, Rachel L; Bull, Catherine; Wren, Christopher; Wade, Angela; Dezateux, Carol

doi:10.1371/journal.pone.0106806

Cited by 28 publications

(41 citation statements)

References 52 publications

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“…With advances in medical, surgical, and intensive care interventions, an estimated 83% of babies with CHD now survive infancy in the United States. 5 Although 1-year survival estimates have been described, 3,[6][7][8][9][10][11] long-term survival estimates are not well researched, and survival may continue to decrease into adulthood.…”

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confidence: 99%

Long‐Term Survival of Individuals Born With Congenital Heart Disease: A Systematic Review and Meta‐Analysis

Best

Rankin

2016

JAHA

117

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BackgroundEstimates of long‐term survival are required to adequately assess the variety of health and social services required by those with congenital heart disease (CHD) throughout their lives.Methods and ResultsMedline, Embase, and Scopus were searched from inception to June 2015 using MeSH headings and keywords. Population‐based studies that ascertained all persons born with CHD within a predefined area and reported survival estimates at ≥5 years were included. Unadjusted survival estimates for each CHD subtype at ages 1 year, 5 years, 10 years, and so forth were extracted. Pooled survival estimates for each age were calculated using meta‐analyses. Metaregression was performed to examine the impact of study period on survival. Of 7840 identified articles, 16 met the inclusion criteria. Among those with CHD, pooled 1‐year survival was 87.0% (95% CI 82.1–91.2), pooled 5‐year survival was 85.4% (95% CI 79.4–90.5), and pooled 10‐year survival was 81.4% (95% CI 73.8–87.9). There was significant heterogeneity of survival estimates among articles (P<0.001 for 1‐, 5‐, and 10‐year survival). A more recent study period was significantly associated with greater survival at ages 1 year (P=0.047), 5 years (P=0.013), and 10 years (P=0.046). Survival varied by CHD subtype, with 5‐year survival being greatest for those with ventricular septal defect (96.3%, 95% CI 93.7–98.2) and lowest for those with hypoplastic left heart (12.5%, 95% CI 0.0–41.4).ConclusionsAmong persons with CHD, the mortality rate is greatest during the first year of life; however, this systematic review and meta‐analysis showed that survival decreases gradually after infancy and into adulthood.

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confidence: 99%

Long‐Term Survival of Individuals Born With Congenital Heart Disease: A Systematic Review and Meta‐Analysis

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Rankin

2016

JAHA

117

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“…Compared with the White ethnic group, the incidence of all CHDs in Asian and Black infants was around 50% higher and for severe and complex CHD types that have high infant mortality, such as univentricular heart (UVH), HLH, TGA and PA, the incidence in Asian and Black infants was double that for White infants. This IR approximates the birth prevalence for life-threatening structural CHDs as these defects all require an interventional procedure in the first year of life; however, around 5% of children with serious CHDs will die without undergoing any procedure 22. The proportion of infants who were diagnosed prenatally and age at first intervention do not vary significantly by ethnicity, thus there was no evidence of differential access to prenatal and postnatal diagnostic services nor clinical intervention.…”

Section: Discussionmentioning

confidence: 87%

Ethnic and socioeconomic variation in incidence of congenital heart defects

Knowles¹,

Ridout

Crowe

et al. 2016

Arch Dis Child

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IntroductionEthnic differences in the birth prevalence of congenital heart defects (CHDs) have been reported; however, studies of the contemporary UK population are lacking. We investigated ethnic variations in incidence of serious CHDs requiring cardiac intervention before 1 year of age.MethodsAll infants who had a cardiac intervention in England and Wales between 1 January 2005 and 31 December 2010 were identified in the national congenital heart disease surgical audit and matched with paediatric intensive care admission records to create linked individual child records. Agreement in reporting of ethnic group by each audit was evaluated. For infants born 1 January 2006 to 31 December 2009, we calculated incidence rate ratios (IRRs) for CHDs by ethnicity and investigated age at intervention, antenatal diagnosis and area deprivation.ResultsWe identified 5350 infants (2940 (55.0%) boys). Overall CHD incidence was significantly higher in Asian and Black ethnic groups compared with the White reference population (incidence rate ratios (IRR) (95% CIs): Asian 1.5 (1.4 to 1.7); Black 1.4 (1.3 to 1.6)); incidence of specific CHDs varied by ethnicity. No significant differences in age at intervention or antenatal diagnosis rates were identified but affected children from non-White ethnic groups were more likely to be living in deprived areas than White children.ConclusionsSignificant ethnic variations exist in the incidence of CHDs, including for specific defects with high infant mortality. It is essential that healthcare provision mitigates ethnic disparity, including through timely identification of CHDs at screening, supporting parental choice and effective interventions. Future research should explore the factors underlying ethnic variation and impact on longer-term outcomes.

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“…In particular, for cases of hypoplastic left heart, survival may be improved with palliative surgery, though many parents opt for comfort care . Moreover, younger age at surgical intervention appears to improve survival in children with CHD . The NorCAS does not hold information on morbidities such as sepsis or pulmonary hypertension, which are more prevalent in children with CHD and increase the risk of mortality …”

Section: Discussionmentioning

confidence: 99%

Survival, by Birth Weight and Gestational Age, in Individuals With Congenital Heart Disease: A Population‐Based Study

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Tennant

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2017

JAHA

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BackgroundCongenital heart disease (CHD) survival estimates are important to understand prognosis and evaluate health and social care needs. Few studies have reported CHD survival estimates according to maternal and fetal characteristics. This study aimed to identify predictors of CHD survival and report conditional survival estimates.Methods and ResultsCases of CHD (n=5070) born during 1985–2003 and notified to the Northern Congenital Abnormality Survey (NorCAS) were matched to national mortality information in 2008. Royston–Parmar regression was performed to identify predictors of survival. Five‐year survival estimates conditional on gestational age at delivery, birth weight, and year of birth were produced for isolated CHD (ie, CHD without extracardiac anomalies). Year of birth, gestational age, birth weight, and extracardiac anomalies were independently associated with mortality (all P≤0.001). Five‐year survival for children born at term (37–41 weeks) in 2003 with average birth weight (within 1 SD of the mean) was 96.3% (95% CI, 95.6–97.0). Survival was most optimistic for high‐birth‐weight children (>1 SD from the mean) born post‐term (≥42 weeks; 97.9%; 95% CI, 96.8–99.1%) and least optimistic for very preterm (<32 weeks) low‐birth‐weight (<1 SD from mean) children (78.8%; 95% CI, 72.8–99.1).ConclusionsFive‐year CHD survival is highly influenced by gestational age and birth weight. For prenatal counseling, conditional survival estimates provide best‐ and worst‐case scenarios, depending on final gestational age and birth weight. For postnatal diagnoses, they can provide parents with more‐accurate predictions based on their baby's birth weight and gestational age.

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Modelling Survival and Mortality Risk to 15 Years of Age for a National Cohort of Children with Serious Congenital Heart Defects Diagnosed in Infancy

Cited by 28 publications

References 52 publications

Long‐Term Survival of Individuals Born With Congenital Heart Disease: A Systematic Review and Meta‐Analysis

Long‐Term Survival of Individuals Born With Congenital Heart Disease: A Systematic Review and Meta‐Analysis

Ethnic and socioeconomic variation in incidence of congenital heart defects

Survival, by Birth Weight and Gestational Age, in Individuals With Congenital Heart Disease: A Population‐Based Study

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