Abstract
Patients born with a single cardiac ventricle are one of the most complex and challenging subgroups of congenital heart disease to manage, from their initial diagnosis to their long-term post-surgical sequelae. Advances in antenatal detection, operative techniques, and post-operative strategies have led to improved outcomes over the past two decades, yet morbidity and mortality remain high relative to other congenital heart lesions. Optimal management and outcome depend in part on a thorough understanding of the anatomy and physiology unique to these infants by all caregivers that may be involved including neonatologists, primary care pediatricians, emergency medicine physicians, and pediatric intensivists. This review will discuss in detail the course of these infants, from their birth through to their three stage surgical palliations and beyond. This review will also highlight many of the most recent medical and surgical innovations available to these infants.
Keywords: Congenital heart disease, hypoplastic left heart syndrome, Fontan procedure, Norwood procedure, single ventricle, superior cavopulmonary anastomosis
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