ABSTRACT

Improved surgical results have decreased the mortality associated with congenital heart defects in the United States from 2.5 to 1.5 per 100,000 population over the past 20 years (1). Despite these advances, chronic heart failure remains an important factor contributing to the long-term morbidity and mortality of patients with congenital heart disease (2-4). Chronic heart failure occurs most commonly in these patients as a result of excessive ventricular volume or pressure load in the setting of normal myocardial function. Primary

myocardial dysfunction also occurs in patients with palliated congenital heart disease in the late postoperative period. The presence of a hemodynamically significant congenital heart defect in conjunction with myocardial dysfunction is associated with significant morbidity and mortality (5,6).