Left Ventricular Outflow Tract: Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome includes various lesions that ultimately result in a small left ventricle along with the underdeveloped aortic and mitral valves, as such the left ventricle cannot handle systemic circulation. It is a severe form of congenital heart defect with 100% fatality without treatment in the neonatal period. Multidisciplinary team-based approaches along with technical advances in staged surgeries to achieve single ventricular circulation have improved survival for this otherwise lethal condition. Knowledge of unique hemodynamics associated with Fontan physiology is necessary for appropriate management of this population and to address potential complications. Cardiac CT is being increasingly used after single-ventricle palliation in patients with hypoplastic left heart syndrome.