The goals of this study are to understand the mechanisms behind the development of heart failure in children born with complicated heart defects consisting of a functional single pumping chamber, improve the ability to objectively measure cardiac function in these patients and determine the relationship of these pathophysiologic mechanisms to outcomes.
In recent decades, the intense interest in the contribution of diastolic dysfunction to adult heart failure has resulted in a number of echocardiographic methods to evaluate diastolic function. To evaluate their accuracy, echocardiographic measurements of diastolic function have been validated with gold-standard measures derived from micromanometer pressure analysis in the adult heart failure population. However, no studies have assessed the accuracy of echocardiographic measures of diastolic function in the single ventricle population. Also, the relationship between echocardiographic measures of diastolic function and outcomes in the single ventricle population ? an exercise necessary to determine their clinical usefulness - has not been investigated. The specific aims of this study seek to validate the accuracy and clinical utility of these non-invasive indices in the single ventricle population