Survival With Congenital Heart Disease in Sweden

Study Questions:

What are the survival trends in children and young adults with congenital heart disease (CHD) as compared with matched controls?

Methods:

A registry-based, prospective, matched-cohort study was conducted in Sweden. Data from two national registries (the national patient and cause of death registers) were linked to identify individuals with CHD born between January 1, 1970 and December 31, 1993. Follow-up data were collected until December 31, 2011. Children serving as controls (10 for each patient) were matched for birth year, sex, and county, and randomly selected from the general population.

Results:

A total of 21,982 patients were identified, of which 48.4% were female. Survivorship for children <5 years was higher for patients born later in the study period, with 98% survival for those born in 1990-1993 as compared with 96% for those born in 1970-1979. During the same time periods, hazard ratios (HRs) of death for patients as compared with controls decreased from 225.8 (95% confidence interval [CI], 136.84-372.70) to 33.47 (95% CI, 22.54-49.70). Patients with the most severe defects, including truncus arteriosus, hypoplastic left heart syndrome, tetralogy of Fallot, and atrioventricular septal defect, had the highest risk for death (HR, 64.07; 95% CI, 53.39-76.89). Patients with less complex disease, atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), coarctation of the aorta (CoA), and Ebstein anomaly also showed increased risk as compared with the general population (HR, 10.9; 95% CI, 9.65-12.32).

Conclusions:

Although survivorship has improved significantly over time, the mortality risk for children and young adults with CHD remains high as compared with matched controls.

Perspective:

This study reinforces previous research demonstrating increased mortality for patients with CHD as compared with the general population, as well as improved outcomes for patients repaired in the most recent surgical era. The strength of the study is that it encompasses the entirety of a national population, with patients well-matched with a large number of controls. There has been some disagreement between studies regarding the impact of simple congenital heart lesions on mortality. This study increased risk of death in all groups of CHD. A contributing factor to the elevated risk in this study may be a slightly different classification scheme. In this study, the simple lesion group (group 2) included patients with ASD, VSD, and PDA, but also those with CoA and Ebstein anomaly, which have been shown to be higher risk lesions in previous studies.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Aortic Surgery, Cardiac Surgery and CHD & Pediatrics, Congenital Heart Disease

Keywords: Heart Defects, Congenital, Aortic Coarctation, Ductus Arteriosus, Patent, Ebstein Anomaly, Heart Defects, Congenital, Heart Septal Defects, Atrial, Heart Septal Defects, Ventricular, Hypoplastic Left Heart Syndrome, Tetralogy of Fallot, Truncus Arteriosus, Young Adult


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