PRESENTATION OF OBSTRUCTIVE LEFT-HEART MALFORMATIONS IN INFANCY

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.