Hypoplasia of the systemic ventricle in congenitally corrected transposition of the great arteries: preoperative documentation and possible implications of operation.

This article describes the determination of small systemic ventricular volume by quantitative cineangiocardiography in two patients with congenitally corrected transposition of great arteries, Ebstein's malformation of the systemic atrioventricular valve, and ventricular septal defect. A subjectively small systemic ventricle and an Ebstein-like abnormality of the systemic atrioventricular valve have been described previously in association with congenitally corrected transposition, but in vivo quantification of ventricular size and pump function has not been reported. An initial palliative rather than a totally corrective procedure is probably desirable in patients with this combination of findings. Ventricular volume calculations may prove useful as an aid in selection of operation in this setting.