Perinatal outcome and five-year survival in children with prenatal diagnosis of hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) is a congenital heart disease of low prevalence and high lethality. Objective: to determine the perinatal outcome and survival at one and five years of fetuses with a prenatal diagnosis of HLHS. Patients and Method: Prospective cohort study of all the fetuses with HLHS from the Perinatal Reference Center (CERPO) born between January 2008 and Decem-ber 2017. Demographic and clinical perinatal data were obtained from the CERPO database. At one and five years of age, a telephone survey was conducted to determine the surgical treatment and survival. Results: 1,573 patients were admitted to the CERPO, 899 with congenital heart diseases (CHD), confirming the prenatal diagnosis of HLHS in 7% (110/1,573). The mean gestational age at diagnosis and the median at admission were 26+3 and 32+3 weeks, respectively. 89% were born alive, 90% at term, and 57% delivered by cesarean section. The median birth weight was 3,128 grams. 89% survive the prenatal period, 50% the early neonatal period, 33% the late neonatal period, 19% the first year, and 17% at 5 years. Conclusions: In this center, the one-year and five-year survival of fetuses with prenatal diagnosis of HLHS was 19% and 17%, respectively. It is important for prenatal counseling to consider publications based on local casuistry, that include patients with prenatal and postnatal diagnoses and those who underwent surgery, in order to provide more precise information to parents.