Projected growth of the adult congenital heart disease population in the United States to 2050: an integrative systems modeling approach

Abstract

Background: Mortality for children with congenital heart disease (CHD) has declined with improved surgical techniques and neonatal screening; however, as these patients live longer, accurate estimates of the prevalence of adults with CHD are lacking.

Methods: To determine the prevalence and mortality trends of adults with CHD, we combined National Vital Statistics System data and National Health Interview Survey data using an integrative systems model to determine the prevalence of recalled CHD as a function of age, sex, and year (by recalled CHD, we mean positive response to the question "has a doctor told you that (name) has congenital heart disease?", which is a conservative lower-bound estimate of CHD prevalence). We used Human Mortality Database estimates and US Census Department projections of the US population to calculate the CHD-prevalent population by age, sex, and year. The primary outcome was prevalence of recalled CHD in adults from 1970 to 2050; the secondary outcomes were birth prevalence and mortality rates by sex and women of childbearing age (15-49 years).

Results: The birth prevalence of recalled CHD in 2010 for males was 3.29 per 1,000 (95 % uncertainty interval (UI) 2.8-3.6), and for females was 3.23 per 1,000 (95 % UI 2.3-3.6). From 1968 to 2010, mortality among zero to 51-week-olds declined from 170 to 53 per 100,000 person years. The estimated number of adults (age 20-64 years) with recalled CHD in 1968 was 118,000 (95 % UI 72,000-150,000). By 2010, there was an increase by a factor of 2.3 (95 % UI 2.2-2.6), to 273,000 (95 % UI 190,000-330,000). There will be an estimated 510,000 (95 % UI: 400,000-580,000) in 2050. The prevalence of adults with recalled CHD will begin to plateau around the year 2050. In 2010, there were 134,000 (95 % UI 69,000-160,000) reproductive-age females (age 15-49 years) with recalled CHD in the United States.

Conclusion: Mortality rates have decreased in infants and the prevalence of adults with CHD has increased but will slow down around 2050. This population requires adult medical systems with providers experienced in the care of adult CHD patients, including those familiar with reproduction in women with CHD.

Keywords: Congenital; Heart defects; Mortality; Population; Survival; Vital statistics.

Fig. 1

Integrative systems model combines mortality and prevalence data in a non-linear regression framework, based on an age- and time-specific compartmental model of disease progression

Fig. 2

Congenital-heart-disease-specific mortality rates as a function of time from 1970 to 2010, stratified by age group. (Markers show National Vital Statistics System data and lines show model estimates.)

Fig. 3

a Recalled congenital heart disease prevalence per 1000 as a function of age, stratified by year of birth. b Recalled prevalence per 1000 at birth (solid red), for 1–4 year olds (dashed green), and for 5–19 year olds (dotted purple) as a function of time, based on data from the National Health Interview Survey (circle and square markers)

Fig. 4

Estimated number of adults (ages 20 to 64 years) with recalled congenital heart disease cases (blue solid line) and prevalence of recalled congenital heart disease in adults (per 1000) (green dotted line), with 95 % uncertainty intervals shaded in grey, as a function of time, from 1965 to 2060. Previous estimates of ACHD prevalence are marked with circles for comparison