Congenital Heart Disease
Changing Mortality in Congenital Heart Disease

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Objectives

This study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD).

Background

Historically, most deaths in patients with CHD occurred in early childhood. Notable advances have since been achieved that may impact on mortality trends.

Methods

We conducted a population-based cohort study of patients with CHD in Quebec, Canada, from July 1987 to June 2005. A total of 8,561 deaths occurred in 71,686 patients with CHD followed for 982,363 patient-years.

Results

The proportion of infant and childhood deaths markedly declined from 1987 to 2005, with a reduction in mortality that exceeded that of the general population. Distribution of age at death transitioned from a bimodal to unimodal, albeit skewed, pattern, more closely approximating the general population. Overall, mortality decreased by 31% (mortality rate ratio: 0.69, 95% confidence interval [CI]: 0.61 to 0.79) in the last (2002 to 2005) relative to the first (1987 to 1990) period of observation. Mortality rates decreased in all age groups below 65 years, with the largest reduction in infants (mortality rate ratio: 0.23, 95% CI: 0.12 to 0.47). In adults 18 to 64 years, the mortality reduction (mortality rate ratio: 0.84, 95% CI: 0.73 to 0.97) paralleled the general population. Gains in survival were mostly driven by reduced mortality in severe forms of CHD, particularly in children (mortality rate ratio: 0.33, 95% CI: 0.19 to 0.60), and were consistent across most subtypes.

Conclusions

Deaths in CHD have shifted away from infants and towards adults, with a steady increase in age at death and decreasing mortality.

Key Words

congenital heart disease
epidemiology
mortality
population

Abbreviations and Acronyms

CHD
congenital heart disease
CI
confidence interval
ICD-9
International Classification of Disease-9th Revision
IQR
interquartile range

Cited by (0)

The study was supported by the Alliance for Quebec Interinstitutional Research(ACQUIRE), Fonds de la Recherche en Santé du Québec, a nonprofit provincial government grant-funding organization, with no role in data collection, data analysis, data interpretation, or writing of the report. Dr. Khairy is supported by a Canada Research Chair, Electrophysiology and Adult Congenital Heart Disease, University of Montreal, and the Canadian Institutes of Health Research. Dr. Mackie was supported by the Fonds de la Recherche en Santé du Québec. Drs. Pilote and Abrahamowicz are supported by the James McGill Research Chairand Canadian Institutes of Health Research. Dr. Abrahamowicz is also supported by the Natural Sciences and Engineering Council of Canada. Dr. Pilote is also supported by the Fonds de la Recherche en Santé du Québec. Dr. Marelli is supported by the Heart and Stroke Foundation of Canada. All other authors report that they have no relationships to disclose.