Hypoplastic left heart syndrome: current modalities of treatment and outcomes

Hypoplastic left heart syndrome is a constellation of malformations which result from the severe underdevelopment of any left-sided cardiac structures. Once considered to be universally fatal, the prognosis for this condition has tremendously improved over the past four decades since the work of William Norwood in the early 1980s. Today, a staged surgical approach is applied for palliating this distinctive cohort of patients, in which they undergo three operative procedures in the first 10 years of their life. Advancements in medical technologies, surgical techniques, and our growing experience in the management of HLHS have made survival into adulthood a possibility. Through this review, we present the different phases of the staged approach with primary focus on stage 1-its modifications, current technique, alternatives, and latest outcomes.