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Although considerable effort has been invested in the development of screening programmes to detect congenital heart disease before and after birth, a large proportion of infants with congenital heart defects remain undetected by these programmes and come to the attention of the medical profession only after they develop symptoms.1 These symptomatic infants can present in extremis and die rapidly. A study of 1590 babies with congenital heart disease who presented in the northern region of the United Kingdom was published in this journal in 1999: one in 10 presented with cardiac symptoms before the first neonatal screening examination and more than half were considered to have a normal cardiovascular system at this examination.1 Of these, almost 40% presented with symptoms or died before the routine 6 week check. These observations are consistent with data from abroad, including the large Baltimore-Washington Study, in which it was observed that, of all infants with cardiovascular malformations who died in the first week of life, one in four did not have the cardiovascular malformation identified before death.2 It is likely that, with the trend towards earlier discharge of apparently well neonates, these numbers may increase in the future, rather than decrease.
Advances in surgical technique have substantially reduced the perioperative mortality for neonates with congenital heart lesions. In the best centres, the in hospital mortality for patients who undergo the arterial switch operation for simple complete transposition of the great arteries now approaches 1%.3 As a result of these advances, death before surgery is becoming proportionally of greater significance: in a recent series reported from Toronto, 4% of neonates presenting with simple transposition died before surgery.4
It thus appears that for certain lesions, an era is approaching when the time of greatest hazard to the infant is not while …
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