The presentation of symptomatic heart disease in infancy based on 10 years' experience (1973-82). Implications for the provision of services.

The diagnoses and age at presentation of 1665 infants with symptomatic heart disease, who were admitted to the Brompton Hospital, London, during the period 1973-82 were reviewed. The frequency of certain conditions had changed during the period of the study. Complete transposition of the great arteries and critical aortic stenosis had become less common, whereas the frequency of right ventricular outflow tract obstruction, and of critical pulmonary stenosis in particular, had increased. Several conditions, including coarctation of the aorta, hearts with univentricular-atrioventricular connexion, and double outlet ventricle, formed a higher proportion of cases in the present series than in the New England Regional Infant Cardiac Program study, whereas the proportion of cases of atrial septal defect, hypoplastic left heart syndrome, and pulmonary atresia with intact septum was smaller. Most of the cyanotic infants presented during the first 14 days of life at the time of ductal closure. As expected this was also true of other duct dependent circulatory disorders such as aortic coarctation and interrupted aortic arch. Acyanotic infants with potentially large left to right shunts tended to present during the second month of life, when the pulmonary vascular resistance fell. This study emphasises that most symptomatic infants with heart disease present during the first two months of life. As a consequence the provision of expert neonatal and infant medical services is an integral part of any paediatric cardiac service.