Hypoplastic left heart syndrome: current modalities of treatment and outcomes

Abstract

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.

Keywords: Congenital heart defects; Hypoplastic left heart syndrome; Interventions; Modifications; Norwood procedure; Treatment outcomes.

Fig. 1

Illustration of Norwood procedure with a modified Blalock-Taussig Shunt

Fig. 2

Illustration of Norwood procedure with a right ventricle-pulmonary artery shunt using a ring-enforced conduit

Fig. 3

Staged palliative approach based on risk stratification and HLHS morphology. MTP, medical termination of pregnancy; PAB, pulmonary artery banding; PGE1, prostaglandin E1; SpO2, oxygen saturation; the asterisk indicates that cardiac transplantation may also be used as an option in case of patient’s clinical deterioration in between stages