Hypoplastic Left Heart Syndrome

Abstract

Children with HLHS present at 2–4 weeks of age when the ductus arteriosus closes. Survival of children with HLHS prior to repair relies on adequate shunting of blood through an ASD and a PDA. Presentation often includes evidence of poor cardiac output and, in extreme cases, cardiac shock. Poor peripheral pulses and perfusion are the dominant features on physical examination. Murmurs may not be appreciated by auscultation; however, the second heart sound is single. Surgical repair is performed in three stages termed Norwood procedure stages I, II, and III. The first stage is typically performed in the neonatal period, stage II at 3–6 months of age, and stage III at 18 months of age. Morbidity and mortality from this congenital heart disease continues to be significant when compared to other congenital heart diseases.