Congenital cardiovascular defects account for significant morbidity and mortality in the pediatric population. Complications of congenital heart disease are lesion-dependent and may range from mild heart failure with no cyanosis to severe cyanosis and shock. Pharmacotherapy of congenital heart disease is also lesion-dependent and usage may range from palliative agents (e.g., prostaglandin E1 for relaxation of aortic stricture) to corrective agents (e.g., indomethacin for closure of the ductus arteriosus). This review will discuss the aberrant pathophysiology and complications associated with specific congenital heart defects, as well as the selection of pharmacological agents used in the management of these defects.

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