Congenital Heart Disease in the General Population

Abstract

Background— Empirical data on the changing epidemiology of congenital heart disease (CHD) are scant. We determined the prevalence, age distribution, and proportion of adults and children with severe and other forms of CHD in the general population from 1985 to 2000. Methods and Results— Where healthcare access is universal, we used administrative databases that systematically recorded all diagnoses and claims. Diagnostic codes conformed to the International Classification of Disease , ninth revision. Severe CHD was defined as tetralogy of Fallot, truncus arteriosus, transposition complexes, endocardial cushion defects, and univentricular heart. Prevalence of severe and other CHD lesions was determined in l985, 1990, 1995, and 2000 using population numbers in Quebec. Children were subjects <18 years of age. The prevalence was 4.09 per 1000 adults in the year 2000 for all CHD and 0.38 per 1000 (9%) for those with severe lesions. Female subjects accounted for 57% of the adult CHD population. The median age of all patients with severe CHD was 11 years (interquartile range, 4 to 22 years) in 1985 and 17 years (interquartile range, 10 to 28 years) in 2000 ( P <0.0001). The prevalence of severe CHD increased from 1985 to 2000, but the increase in adults was significantly higher than that observed in children. In the year 2000, 49% of those alive with severe CHD were adults. Conclusions— The prevalence in adults and median age of patients with severe CHD increased in the general population from 1985 to 2000. In 2000, there were nearly equal numbers of adults and children with severe CHD.

Adult Congenital Heart Disease in a Nationwide Population 2000–2014: Epidemiological Trends, Arrhythmia, and Standardized Mortality Ratio

“…Such an increase might continue until the prevalence is close to the incidence of CHD at birth adjusted by optimized survival. In 2014, the prevalence of ACHD in our study was 2.17 per 1000 adults, which is within the previous reported estimated range (1.7–6.12 per 1000) 1, 3, 4, 5, 6. In most high‐income countries in which care for ACHD is increasingly structured, prevalence of severe ACHD would increase over time 3, 4, 11.…”

“…The CHD population was further grouped as having simple CHD and severe CHD. Severe CHD included tetralogy of Fallot (TOF), transposition of great arteries, double outlet of right ventricle, endocardial cushion defect, total anomalous pulmonary venous return, tricuspid atresia, congenitally corrected transposition of the great arteries, common truncus, and common ventricles 4, 9. Simple CHD includes the other CHD diagnoses.…”

“…Open heart surgery for CHD was first performed in the 1940s in North America. According to administrative database, prevalence of ACHD in Quebec was 4.09 per 1000 adults in 2000 and 6.12 per 1000 adults in 2011 3, 4. Data of ACHD prevalence may also be estimated from CHD incidence at birth and survival estimate for each type of CHD.…”

Genetics and Genetic Testing in Congenital Heart Disease

“…Contemporary advancements in medical care, surgical interventions, and diagnostics have contributed to a well-characterized decrease in patient mortality and concomitant increase in CHD prevalence among patients of reproductive age [38][39][40] . Recent analyses indicate that adults now constitute roughly two-thirds of the CHD population, representing a nearly 60% increase in CHD prevalence among adult patients since the year 2000 38 .…”

Occurrence and pattern of congenital heart diseases in a rural area of sub-Saharan Africa

“…In a previous study by Ariane et al ., late presentation to practitioners was observed in 79.3% of cases 12. Both access to healthcare and the high cost of treatment also explain the very high incidence of late presentations (68.2%) recorded in Bannerman’s study in Zimbabwe 9…”