The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation

doi:10.1016/j.ahj.2012.07.023

American Heart Journal

Volume 164, Issue 4, October 2012, Pages 568-575

https://doi.org/10.1016/j.ahj.2012.07.023 Get rights and content

Purpose

The prevalence of adult patients with congenital heart disease (CHD) has been reported with a high degree of variability. Prevalence estimates have been calculated using birth rate, birth prevalence, and assumed survival and derived from large administrative databases. To report more robust prevalence estimate, we performed a systematic review for studies concerning CHD prevalence in adults. Moreover, to diminish bias of calculated estimates, we conducted an evidence-based calculation for the Netherlands.

Methods

A systematic database search was performed to identify reports on the prevalence of adult CHD. Bicuspid aortic valve, mitral valve prolapse, Marfan syndrome, cardiomyopathy, congenital arrhythmia, and spontaneously closed defects were excluded. In addition, CHD prevalence was calculated using birth rate, birth prevalence, and survival estimates.

Results

Our search yielded 10 publications on the prevalence of CHD in adults. Four reported results from population wide cross-sectional data, whereas in 6, prevalence was calculated. Mean prevalence reported by empirical studies was 3,562 per million when unspecified lesions were included and 2,297 per million when these were excluded. Mean prevalence derived from calculation was 3,536. Our calculated estimate was 3,228 per million adults. Taking these estimates as well as the limitations inherent to their derivation into consideration, the prevalence of CHD in the adult population is approximately 3,000 per million adults.

Conclusion

Section snippets

Systematic search

This systematic review is in adherence with the preferred reporting items for systematic reviews and meta-analysis (PRISMA).

Eligibility

Studies were eligible when they stated the prevalence of CHD or the number of patients within a predefined geographically and temporally defined adult population. Congenital heart disease was defined according to the criteria of Mitchell et al,²⁰ in accordance with previous epidemiological studies.6, 21 This definition excludes patients with arrhythmias, cardiomyopathies,

Study selection

Literature searched identified 1,354 potentially eligible studies, including duplicates (Figure 1). After review of title and abstract, 20 articles were retrieved for detailed full-text examination. Most studies were ineligible because they did not pertain to CHD or to the prevalence of CHD. Many studies only reported prevalence at birth, in children, or in selected populations as Down syndrome. Eight studies met eligibility and were included in the systematic review. Reference checks rendered

Main findings

Best available evidence suggests that the prevalence of CHD in adults is approximately 3,000 per million. Mean overall prevalence obtained by pooling estimates provided by 3 population wide cross-sectional studies was 3,562 per million including unspecific cases and 2,297 excluding unspecific cases. Severe lesions comprise approximately 3%, and moderate lesions, 15%. Mean of calculated estimates was 3,546 per million. Prevalence in the Netherlands according to our current calculation was 3,228

Conclusions

This systematic review presents a comprehensive overview of publications on the prevalence of CHD in adults. The best available evidence suggests that overall prevalence of CHD in the adult population is approximately 3,000 per million. However, there is large heterogeneity between studies, which complicates interpretation of current estimates.

Disclosures

Conflicts of interest: None.

Financial disclosures: None.

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Patients with a history of cardiac surgery were not more likely to have any hypertensive disorder diagnosed than healthy controls. Patients with a history of cardiac surgery were more likely to have an operative delivery (P<.0001) but equally likely to have a cesarean delivery (P=.528) compared with healthy controls. Birthweight was not statistically different of 2655±808 g in neonates born to patients with a history of cardiac surgery vs 2844±830 g born to healthy controls (P=.092).
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Clinical InvestigationValvular and Congenital Heart DiseaseThe prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation

Purpose

Methods

Results

Conclusion

Section snippets

Systematic search

Eligibility

Study selection

Main findings

Conclusions

Disclosures

Arch Cardiovasc Dis

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu

Am Heart J

Int J Cardiol

Int J Cardiol

J Thorac Cardiovasc Surg

Am J Cardiol

Am J Cardiol

Ann Thorac Surg

Am Heart J

J Pediatr

Am J Cardiol

J Am Coll Cardiol

Ann Thorac Surg

Ann Thorac Surg

Am J Cardiol

Survival with congenital heart disease and need for follow up in adult life

Heart

Congenital heart disease in the general population: changing prevalence and age distribution

Circulation

Atrial and ventricular septal defects—epidemiology and spontaneous closure

J Matern Fetal Neonatal Med

25-year mortality after surgical repair of congenital heart defect in childhood. A population-based cohort study

JAMA.

Late results of pediatric cardiac surgery in Finland: a population-based study with 96% follow-up

Circulation

Reflections on the past, present and future of pediatric cardiology

Cardiol Young

The natural history of congenital isolated pulmonic and aortic stenosis

Annu Rev Med

Clinical Investigation
Valvular and Congenital Heart Disease
The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation