Abstract
Hypoplastic left heart syndrome (HLHS) represents a variety of cardiac malformations that may result from errors in the early stages of cardiac development. HLHS includes a wide spectrum of cardiac malformations including hypoplasia of the left ventricle, ascending aorta, hypoplasia, or atresia of the aortic and mitral valves. Over the recent years, the improved resolution of advanced equipment with awareness and increased performance of second-trimester ultrasound examinations for the assessment of fetal anomalies have helped in understanding the spectrum and have expanded our knowledge of HLHS. They are one of the causes which constitute for neonatal morbidity and mortality and hence the rapid need for prenatal evaluation with ultrasound to detect cardiac anomalies. Prenatal recognition of disease also allows families to prepare for a child with a life-threatening defect by consultation with the multidisciplinary team that will care for their newborn and discussing the short- and long-term prognosis.