ABSTRACT
Introduction Hypoplastic left heart syndrome (HLHS), originally described by Lev in 1952 and named by Noonan and Nadas 6 years later, accounts for 1-4% of all congenital heart defects. If left untreated it is the commonest cardiac cause of death in the newborn period. Approximately 1000-1500 babies in the USA and 200 babies in the United Kingdom are born with HLHS each year. Two different treatment strategies are presently available, staged palliative reconstruction and neonatal cardiac allotransplantation. Advances in medical and surgical management over the last two decades have resulted in a significant improvement in outcome of this once uniformly fatal lesion. Five-year survival rates in excess of 70% are being consistently reported. The staged surgical management involves three separate procedures ultimately leading to a single ventricular repair with modified Fontan anatomy. The functional capacity and neurodevelopmental outcome of these children is reported to be no different to that of other children with complex congenital heart disease. This chapter will detail the case management of a neonate undergoing the first-stage reconstruction.
