Incidence of Type 1 diabetes mellitus and effect on mortality in young patients with congenital heart defect – A nationwide cohort study☆

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Highlights

  • •

    This is the first large retrospective cohort study focusing on adults with congenital heart disease who also develop T1DM.

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    The incidence of developing T1DM was 50% higher in patients with CHD.

  • •

    The combination of CHD and T1DM was associated with a 4-fold increase in mortality compared to controls with only T1DM.

  • •

    The growing numbers of adult with CHD will significantly affect cardiologist practise in the years to come.

Abstract

Background

1% of all live born children are born with a congenital heart defect (CHD) and currently 95% reach adulthood. Type 1 diabetes mellitus (T1DM) is an autoimmune disease that can develop due to i.e. heredity, exposure to infections and stress-strain. The incidence of T1DM in patients with CHD is unknown and we analysed the risk of developing T1DM for patients with CHD, and how this influences mortality.

Methods

By combining registries, the incidence of T1DM and the mortality was analysed in patients with CHD by birth cohort (1970–1993, 1970–1984 and 1984–1993) matched with population-based controls matched for sex, county and year of birth without CHD and followed from birth until a maximum of 42 years.

Results

221 patients with T1DM among 21,982 patients with CHD and 1553 patients with T1DM among 219,816 matched controls were identified. The hazard ratio (HR) for developing T1DM was 1.50 (95%, CI 1.31–1.73) in patients with CHD compared to the controls and the first birth cohort (1970–1984) had the highest risk for T1DM, HR 1.87 (95%, CI 1.56–2.24). After onset, mortality risk was 4.21 times higher (95%, CI 2.40–7.37) in patients with CHD and T1DM compared to controls with T1DM.

Conclusion

From a nationwide cohort of patients with CHD and controls, the incidence of developing T1DM was 50% higher in patients with CHD, showing a significant increase in risk among birth cohort 1970–1984. The combination of CHD and T1DM was associated with a 4-fold increase in mortality compared to controls with only T1DM.

Section snippets

Background

Congenital heart defect (CHD) is the most common congenital malformation among live born children as well as a major cause of death during infancy and young children [[1], [2], [3], [4], [5], [6]]. Globally about 1% of all live born children are diagnosed with CHD [3,4,[7], [8], [9]]. With improved clinical, medical and surgical care, survival rates have improved to the point that >95% of children with CHD now reach adulthood [1,3,4,6,7,[9], [10], [11], [12], [13], [14], [15], [16], [17], [18],

Study design

We performed a cohort study that described the incidence of T1DM and the mortality in adult patients with CHD before and after onset of T1DM divided by birth cohort, all (born 1970–1993), first birth cohort (born1970–1984), second birth cohort (born1985–1993), compared to population-based controls matched for sex, year of birth and county without CHD and followed from birth until a maximum of 42 years, 1970–2011.

Data sources

The computations for this study are based on individual data from the Swedish

Baseline characteristics

From the National Patient Registry 21,982 patients with CHD were identified, 51.5% men and 48.5% women. Mean age at the last follow-up in the first birth cohort (1970–1984) was 32.2 (SD 8.7) years in the patients with CHD and 34.4 (SD 4.5) years in the controls. In the second birth cohort (1985–1993) the mean age at follow up was 21.4 (SD 4.5) years for patients with CHD and 22.1 (SD 2.6) years for controls (Table 1, Appendix D, Appendix E).

The incidence of T1DM

Of patients with CHD, 221 (1%) adults were diagnosed

The incidence of T1DM

The incidence of T1DM in CHD patients compared to healthy controls has not been extensively studied before. In the present study, patients with CHD had an almost 50% higher incidence of T1DM compared to patients without CHD, in line with our previous data [33].This is the first time an increased incidence of T1DM has been reported for patients with CHD. However, a Danish registry study reported the relative risk of developing T2DM to be 1.4 for patients with CHD above 30 years of age [38]. The

Conclusion

From a nationwide cohort of patients with CHD and controls, the risk of developing T1DM was 50% higher in patients with CHD and the mortality after onset of T1DM was associated with a 4-fold increase in patients with CHD compared to controls without CHD. This study suggests the combination of CHD and T1DM to be more lethal than each diagnosis on its own. These findings are important in future medical care for patients with CHD.

The following are the supplementary data related to this article.

Declaration of competing interest

The authors declare no conflict of interest.

Acknowledgments

This work was supported by a grant from the Swedish Heart-Lung Foundation (20090724) and by grants from the Swedish state under the agreement between the Swedish government and the county councils, the ALF-agreement (grant number 236611).

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