Late mortality among Danish patients with congenital heart defect

To examine long-term mortality in Danish patients with congenital heart defect (CHD), we performed a population-based follow-up study using nationwide registries. We identified all children born in Denmark from January 1, 1977 to January 1, 2006 from the Danish Civil Registration System. Children with a primary diagnosis of CHD, diagnosed before 1 year of age, were then identified in the National Registry of Patients. We computed cumulative mortality of patients and the background population according to birth period (1977 to 1986, 1987 to 1996, and 1997 to 2005). We identified 6,646 patients with CHDs. Overall cumulative mortality estimates in patients with CHDs at 1 year and 10 and 25 years of age were 20% (95% confidence interval [CI] 19 to 21), 25% (95% CI 24 to 26), and 28% (95% CI 27 to 30). In Danes born in the same period equivalent mortality estimates were 0.6% (95% CI 0.6 to 0.6), 0.8% (95% CI 0.7 to 0.8), and 1.3% (95% CI 1.3 to 1.3). Mortality differed substantially according to heart defect type and mortality at 10 years of age ranged from 9% (95% CI 6 to 12) in patients with atrial septal defects (n = 361) to 55% (95% CI 45 to 66) in patients with common arterial trunk (n = 78). Mortality decreased during the study period; 1-year mortality was 28% (95% CI 26 to 31) for patients born from 1977 to 1986 (n = 2,907) compared to 13% (95% CI 12 to 15) for patients born from 1997 to 2005 (n = 2,741). Mortality decreased in all heart defect type categories. In conclusion, mortality in patients with CHD was high compared to the general population, especially in infancy, but also after 10 years of age, emphasizing the need for long-term medical follow up. Mortality at 1 year of age has decreased substantially during recent decades.