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
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.
- adult congenital heart disease
- patient survival
Statistics from Altmetric.com
Improvements in diagnosis, medical treatment, and surgical repair are changing the pattern of survival of congenital heart disease. In recent years the survival of more complex types of congenital heart disease has improved, leading to an increasing work load for follow up, reinvestigation, and reoperation. Over the same time, the care of adults with congenital heart disease has begun to develop as a separate subspecialty.1 It seems likely that all but those with the simplest forms of congenital heart disease will require specialist follow up, and this will be the main growth area in care of patients with congenital heart disease.2 So far there has been inadequate planning for the future care of such patients and there are no population based data to assess the future growth of this subspecialty.2 3 The aim of this study was, therefore, to predict the likely future growth of the population of survivors of congenital heart disease requiring follow up beyond the age of 16 years.
The former Northern Health Region comprises the counties of Cumbria, Northumberland, Tyne & Wear, Durham, and Cleveland and has a resident population of just over three million. All infants and children with suspected heart disease from 15 of the 16 health districts are referred to a single centralised paediatric cardiology centre.4 Children from the health district of South Cumbria are referred elsewhere for geographical reasons and so were not included in this study.
The paediatric cardiology database contains details of all infants and children with structural congenital heart disease born since 1 January 1985.4 Ascertainment was prospective for all diagnoses made from 1990 onwards and retrospective for diagnoses made between 1985 and 1989. Ascertainment is thought to be complete for all significant and complex heart disease with some underascertainment of minor malformations in infancy in the early part of the study.5
Diagnoses made in infancy were limited to babies born to mothers resident in the region at the time of birth. There was no significant inflow or outflow of population within the first 12 months.6 Diagnoses made after the first birthday were limited to those made in children resident in the region at the time of diagnosis. Infants and children moving into the region and referred for follow up of congenital heart disease that had already been recognised were not included. This allows us to compensate for immigration and emigration to other regions. National statistics show no significant net inflow or outflow of population during the period of the study.6 The Office of National Statistics provided data on the regional birth rate.
Cardiovascular malformations were defined according to the generally accepted definition of “a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional importance”.7 In keeping with previous population studies, children with isolated cardiac arrhythmias, cardiomyopathy, acquired heart disease, isolated bicuspid aortic valve, mitral valve prolapse without regurgitation, isolated dextrocardia, cardiac tumours, patent ductus associated with prematurity, and atrial septal defect undergoing spontaneous closure in infancy were excluded. All cases were classified according to the main anatomical diagnosis. Children with more than one 