From August 1982 to October 1985, 8 consecutive patients, aged 4 months to 8 years (mean 2.6), with criss-cross or superoinferior ventricles, or both, were studied. Seven patients had usual atrial arrangement and 1 had mirror-image arrangement. In 5 patients there was atrioventricular (AV) concordance with crossed AV streams, the ventricular septum was horizontal in 4 and sagittal in 1, and the ventriculoarterial connections were discordant in all. The 3 patients with AV discordance had superoinferior ventricles, but a criss-cross AV relation was not present; the ventriculoarterial connections were concordant in 2 and double-outlet right ventricle in 1. The sequential connections and spatial relations of the cardiac chambers, as well as the associated anomalies, were accurately assessed by echocardiography. The diagnosis was confirmed by angiography in all patients, by surgery in 3 and by autopsy in 2.