Fetal Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a spectrum of congenital cardiac anomalies that result in the left ventricle being incapable of providing adequate systemic perfusion. This lesion is incompatible with life unless a series of palliative cardiac interventions and surgeries are performed. Prenatal diagnosis of HLHS is becoming increasingly common due to advances in fetal diagnostic imaging. Prenatal detection of HLHS allows the fetal and perinatal cardiac teams to make an accurate cardiac diagnosis, provide adequate parental counseling, and derive appropriate plans for delivery room management as well as neonatal interventions for these neonates born with HLHS. This chapter describes cardiac anatomy, fetal echocardiographic evaluation, parental counseling, delivery planning, and impact of prenatal detection on outcomes in fetuses suspected to have HLHS.