ABSTRACT
Introduction Left heart malformations account for nearly 9% of all congenital heart defects.1 Proper development of the inflow and outflow structures of the left heart are crucial to ensuring a fully oxygenated cardiac output is delivered to the rest of the body. In the early stages of embryogenesis, a sequence of carefully orchestrated molecular events establishes the left atrium as the receiving chamber of pulmonary venous return, left ventricular (LV) inflow through a mitral apparatus, and connection of the LV outflow to the aorta. Perturbation of these events results in various forms of left-sided congenital heart disease. In this chapter, we review the development, pathology, and pathophysiology of left-sided heart malformations, as well as their implications in fetal and postnatal care.
