Focus Issue: Structural Heart Disease
Congenital Heart Disease: State-of-the-Art Paper
Birth Prevalence of Congenital Heart Disease Worldwide: A Systematic Review and Meta-Analysis

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Congenital heart disease (CHD) accounts for nearly one-third of all major congenital anomalies. CHD birth prevalence worldwide and over time is suggested to vary; however, a complete overview is missing. This systematic review included 114 papers, comprising a total study population of 24,091,867 live births with CHD identified in 164,396 individuals. Birth prevalence of total CHD and the 8 most common subtypes were pooled in 5-year time periods since 1930 and in continent and income groups since 1970 using the inverse variance method. Reported total CHD birth prevalence increased substantially over time, from 0.6 per 1,000 live births (95% confidence interval [CI]: 0.4 to 0.8) in 1930 to 1934 to 9.1 per 1,000 live births (95% CI: 9.0 to 9.2) after 1995. Over the last 15 years, stabilization occurred, corresponding to 1.35 million newborns with CHD every year. Significant geographical differences were found. Asia reported the highest CHD birth prevalence, with 9.3 per 1,000 live births (95% CI: 8.9 to 9.7), with relatively more pulmonary outflow obstructions and fewer left ventricular outflow tract obstructions. Reported total CHD birth prevalence in Europe was significantly higher than in North America (8.2 per 1,000 live births [95% CI: 8.1 to 8.3] vs. 6.9 per 1,000 live births [95% CI: 6.7 to 7.1]; p < 0.001). Access to health care is still limited in many parts of the world, as are diagnostic facilities, probably accounting for differences in reported birth prevalence between high- and low-income countries. Observed differences may also be of genetic, environmental, socioeconomical, or ethnic origin, and there needs to be further investigation to tailor the management of this global health problem.

Key Words

congenital
epidemiology
heart defects
incidence
prevalence

Abbreviations and Acronyms

AoS
aortic stenosis
ASD
atrial septal defect
CHD
congenital heart disease
CI
confidence interval
Coarc
coarctation of the aorta
GUCH
grown-up congenital heart disease
PDA
patent ductus arteriosus
PS
pulmonary stenosis
TGA
transposition of the great arteries
TOF
tetralogy of Fallot
VSD
ventricular septal defect

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All authors have reported that they have no relationships relevant to the contents of this paper to disclose.