Hypoplastic Left Heart Syndrome: A Review

Abstract

Hypoplastic left heart syndrome is a rare and poorly understood congenital disorder featuring a univentricular myocardium, invariably resulting in early childhood death if left untreated. The process to palliate this congenital cardiomyopathy is of high complexity and may include invasive interventions in the first week of life. The preferred treatment strategy involves a staged correction with 3 surgical procedures at different points in time. The Norwood procedure is usually performed within the first weeks of life and aims to increase systemic circulation and relieve pulmonary vascular pressure. This procedure is followed by the bidirectional Glenn and the Fontan procedures in later life, which offer to decrease stress in the ventricular chamber. The prognosis of children with this disease has greatly improved in the past decades; however, it is still largely driven by multiple modifiable and nonmodifiable variables. Novel and clever alternatives have been proposed to improve the survival and neurodevelopment of these patients, although they are not used as standard of care in all centers. The neurodevelopmental outcomes among these patients have received particular attention in the last decade in light to improve this very limiting associated comorbidity that compromises quality of life.