National mortality statistics
In 2001, 811 deaths due to congenital heart disease were recorded in the UK.
Just over a third of these deaths were in babies aged less than a year, one in seven were in children and adolescents (aged 1-19 years) and the remaining half were in adults (aged 20 years and over).
Data from England and Wales show that the proportion of deaths from congenital heart disease occurring in babies has declined markedly over the last two decades. In 1986 death was most common in infancy, with just under 60% of deaths from congenital heart disease occurring in babies aged less than a year. By the early 1990’s death from congenital heart disease was most common in adults aged 20 years and over.
Survival
Data from the Northern Regional Paediatric Cardiology Database show that 82% of babies diagnosed with congenital heart disease survive the first year of life.
However, first year (infant) survival rates vary widely depending on the nature of the congenital heart defect. In the Northern Regional Paediatric Cardiology Database the lowest first year survival rates were observed in babies with hypoplastic left heart, truncus arteriosus and more severe forms of pulmonary artresia, and the highest in babies with mild cases of aortic or pulmonary stenosis, ventricular septal defect, and congenitally corrected transposition of the great arteries.
It should be noted that these data are based on births going as far back as 1985. With major advances to treatment over the past decade, babies born today with the more serious heart defects are likely to have much higher one-year survival rates than these data suggest. For example, today around 50% of babies born with hypoplastic left heart survive infancy (Andrews R, Tulloh R, Sharland G, Simpson J, Rollings S, Baker E, Qureshi S, Rosenthal E, Austin C and Anderson D (2001) Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis. Archives of Disease in Childhood;85:474-77).
Combining data on observed infant survival with estimates of the likely childhood survival rates (from 1 year to 16 years), it is predicted that overall 78% of babies diagnosed with congenital heart disease will survive to adulthood1.
1. This prediction is based on published survival rates in patients who mostly had operations 15 or more years ago when they were in infancy. Marked improvements in care in the last 15 years mean that this prediction is likely to be an underestimate of the true survival rate into adulthood. Data from the USA suggest this is likely to be around 85% (Perloff JK and Warnes CA (2001) Challenges posed by adults with repaired congenital heart disease. Circulation;103:2637-43).
