Survival with congenital heart disease and need for follow up in adult life

Abstract

Objective: To predict the growth in demand for long term follow up of adults with congenital heart disease.

Design: Observed diagnoses of congenital heart disease in infancy and childhood were adjusted for observed infant survival, predicted further survival to age 16 years, underascertainment in older childhood, and predicted need for long term follow up.

Setting: The resident population of one health region in the UK.

Patients: All confirmed cardiovascular malformations diagnosed in 1985 to 1999 in children born in 1985 to 1994.

Results: 1942 cases of congenital heart disease were diagnosed in infancy in a population of 377 310 live births (5.2/1000). 1588 (82%) survived to 1 year and 1514 were predicted to survive to age 16. 605 further diagnoses were made in childhood-678 when adjusted for underascertainment. Thus, 2192 children were predicted to reach age 16, of whom 784 would require long term follow up in adult life. The adult population would comprise 28% complex, 54% significant, and 18% minor congenital heart disease. These figures predict the need for adult follow up of congenital heart disease of over 200 extra cases per 100 000 live births each year or over 1600 extra cases a year every year in the UK.

Conclusions: The need for follow up of congenital heart disease in adult life is likely to grow linearly, with increasing complexity and increasing need for reinvestigation and reintervention with time. Appropriate provision should be made for adequate manpower, resources, and facilities for care of these patients.

Figure 1

Data available on all diagnoses in babies and children born in 1985 to 1994. Ascertainment varies from 13 years for births in 1985 to four years for births in 1994. Data range from 10 years for 0-4 year olds to one year for 13 year olds. The figure shows data available (hatched) and data extrapolated (grey). The effect of the adjustment is shown in fig 2.

Figure 2

The effect of adjustment for underascertainment. All diagnoses at 1-13 years are shown (grey bars) with the effect of extrapolation to compensate for underascertainment at ages 5-13 years (solid bars). The difference between raw and adjusted figures is very small and is shown for individual diagnoses in table 2.

Figure 3

The spectrum of complexity of congenital heart disease at different stages of follow up. Complexity decreases during childhood but increases in adult life if children with minor malformations that do not require long term follow up are excluded.