Management of Hypoplastic Left Heart Syndrome in PCICU Before and After Cardiac Surgery

Hypoplastic left heart syndrome (HLHS) is the most common cardiac malformation causing death from congenital disease in the first month of life. The syndrome is a spectrum of left-sided cardiac malformations, including of aortic atresia or stenosis, hypoplasia of ascending aortic arch, and hypoplasia or absence of the left ventricle. The long-term outlook for babies with this condition was until recently very poor, but two treatment options now offer hope, heart transplantation and staged reconstructive surgery pioneered by Norwood, to reconstruct a viable circulation.