What question did this study set out to answer?

To characterize temporal trends in all-cause mortality among individuals diagnosed with congenital heart disease.

April 23, 2026Journal of the American College of Cardiology

Changing Mortality in Congenital Heart Disease

Q: What is the clinical evidence from this study?

Study design: Cohort. Population: congenital heart disease (n=71686). Intervention: Later time period (2002 to 2005) vs. Earlier time period (1987 to 1990). Primary outcome: all-cause mortality (mortality rate ratio 0.69, 95% CI 0.61-0.79).

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Key result

Congenital heart disease mortality drops ~31% in recent era compared to 1987-1990.

mortality rate ratio 0.69
95% CI 0.61-0.79
n=71,686

Why the study?

Temporal trends in all-cause mortality in patients with congenital heart disease have not been fully characterized despite advances in care.

Key Points

To characterize temporal trends in all-cause mortality among individuals diagnosed with congenital heart disease.
Conducted a population-based cohort study in Quebec, Canada, examining 71,686 patients with congenital heart disease from July 1987 to June 2005.
Followed participants for 982,363 patient-years and recorded 8,561 all-cause deaths across distinct calendar periods and age strata.
Overall mortality decreased by 31% (mortality rate ratio [MRR]: 0.69, 95% CI: 0.61 to 0.79) comparing 2002–2005 to 1987–1990, shifting the age-at-death distribution from bimodal to unimodal.
Mortality rates decreased across all age groups under 65 years, led by infants (MRR: 0.23, 95% CI: 0.12 to 0.47) and adults aged 18 to 64 years (MRR: 0.84, 95% CI: 0.73 to 0.97).
Survival gains were predominantly driven by mortality reductions in severe forms of congenital heart disease, particularly in children (MRR: 0.33, 95% CI: 0.19 to 0.60).

Study Design

Type

Cohort (n=71,686)

Structured PICO

Population

71,686 patients with congenital heart disease in Quebec, Canada, followed for 982,363 patient-years between 1987 and 2005.

Outcome

Temporal trends in all-cause mortalityhard clinical

Main Result

Relative Risk: 0.69 (95% CI 0.61–0.79)

Mortality in congenital heart disease has significantly decreased over time, particularly in infants and those with severe forms, shifting the burden of death towards adulthood.

Discussion

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Implication

Survival gains in severe CHD shift mortality burden to adulthood; leaves open optimal adult management strategies in this growing population.

Cite This Study

Khairy et al. (2010) conducted a cohort in congenital heart disease (n=71,686). Later time period (2002 to 2005) vs. Earlier time period (1987 to 1990) was evaluated on all-cause mortality (mortality rate ratio 0.69, 95% CI 0.61-0.79). Overall mortality in patients with congenital heart disease decreased by 31% in 2002-2005 compared to 1987-1990 (mortality rate ratio 0.69; 95% CI 0.61-0.79).

synapsesocial.com/papers/69ea3867c2ceeb8fbfae7e7d https://doi.org/10.1016/j.jacc.2010.03.085

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Implication

Survival gains in severe CHD shift mortality burden to adulthood; leaves open optimal adult management strategies in this growing population.

Changing Mortality in Congenital Heart Disease

Key Points

Study Design

Structured PICO

Main Result

Discussion

Implication

Cite This Study

Changing Mortality in Congenital Heart Disease

Key Points

Study Design

Structured PICO

Main Result

Discussion

Implication

Cite This Study

Also Consider

Also Consider