Pregnancy outcome of fetuses with a diagnosis of hypoplastic left ventricle on prenatal sonography

Objective. The goal of our study was to assess the pregnancy outcome of fetuses with a diagnosis of hypoplastic left heart syndrome (HLHS) on prenatal sonography to determine the frequency of intrauterine fetal demise (IUFD) and any factors associated with IUFD. Methods. We reviewed all cases with the diagnosis of HLHS on prenatal sonography at our institution from 1992 through 2003. Data collected included gestational age at diagnosis, sonographic findings, karyotype testing, and pregnancy outcome. Results. Our study included 176 fetuses with HLHS. One hundred thirty-four fetuses were liveborn; 32 pregnancies were terminated; 3 IUFDs occurred; and outcome was unknown in 7. Of the 134 liveborn fetuses, 2 had abnormal karyotypes and 30 had other anomalies. Two of the 3 fetuses with IUFD had abnormal karyotypes, 1 with trisomy 13 and 1 with trisomy 18, and both had other anomalies on sonography. The third fetus with IUFD had no other anomalies but was 1 of triplets, and the karyotype was unknown. Conclusions. Fetuses with HLHS diagnosed prenatally who have normal chromosomes are unlikely to die in utero.