ABSTRACT
Congenital heart disease associated with only one effective pumping chamber, or single ventricle, comprises about 2% of all congenital heart disease (1). However, there are a wide diversity of structural heart defects where there is only one effective single ventricle. Variations are defined by the morphology of the ventricle, number and type of AV valves, orientation of the great vessels, associated lesions leading to obstruction of flow into or out of the single ventricle, and dependence of pulmonary or systemic flow upon a patent ductus arteriosus. Heart failure is a common complication of single ventricle lesions and may occur from the newborn period into adulthood.
