M. Abuharb et al., PRESENTATION OF OBSTRUCTIVE LEFT-HEART MALFORMATIONS IN INFANCY, Archives of Disease in Childhood, 71(3), 1994, pp. 60000179-60000183
This study documents the presentation of acutely life threatening cong
enital heart disease in infancy in order to assess the performance of
screening examinations soon after birth and at 6 weeks of age. Ah case
s of obstructive left heart malformations presenting in infancy in one
health region from 1987-91 were analysed retrospectively. Altogether,
120 infants presented with either hypoplastic left heart syndrome, in
terruption of the aortic arch, coarctation of the aorta, or aortic val
ve stenosis. Twelve became symptomatic or died within 24 hours. Thirty
four had an abnormal neonatal examination, eight of whom were referre
d. Six more became symptomatic before discharge. Ninety four babies we
nt home, 51 developed heart failure before 6 weeks, and another seven
died without diagnosis. Twenty five of 36 (69%) reaching 6 weeks witho
ut diagnosis had a second examination which was abnormal in 17. Two ba
bies died undiagnosed after 6 weeks and the other 18 presented at up t
o 11 months of age. The neonatal and 6 week examinations perform poorl
y as screening tests for congenital heart disease. A normal neonatal e
xamination does not exclude life threatening congenital heart disease.