In patients with hypoplastic left heart syndrome, the right ventricle is positioned as the systemic ventricle. Monitoring its function is important but difficult because of its unusual position and geometry. The purposes of this study were to determine the feasibility of applying automatic echocardiographic measurements to the evaluation of right ventricular function in patients with hypoplastic left heart syndrome and to compare their function with that of normal subjects. Eleven patients with hypoplastic left heart syndrome (mean age 1.6 years) were evaluated with automatic border detection. Images were adequate if greater than 85% of the endocardium was being tracked. Systolic indexes were fractional area change and peak emptying rate; diastolic indexes were peak filling rate and the proportion of filling that occurred during rapid filling, diastasis, and atrial contraction. These data were compared with those in 18 normal control subjects. Three patients had completed stage 1 (aortic reconstruction, surgical shunt, and atrial septectomy), three patients had completed stage 2 (hemi-Fontan), and five patients had completed stage 3 (Fontan). Images were adequate in all cases. Fractional area change and peak emptying rate were significantly lower in the patients with hypoplastic left heart syndrome compared with control subjects (38% +/- 6% versus 61% +/- 10% and 3.5 +/- 0.9 versus 5.3 +/- 1.3 sec-1, respectively). Peak filling rate was significantly lower (4.1 +/- 1.3 versus 6.6 +/- 2.3 sec-1) and atrial contribution was significantly higher (18% +/- 7% versus 12% +/- 4%) in the patients. Automated echocardiographic analysis is feasible in assessing systemic ventricular function in children with hypoplastic left heart syndrome. These patients have abnormal systolic and diastolic function. Automatic border detection may be useful in detecting and monitoring systemic ventricular dysfunction as these patients progress through the surgical stages of repair.