Two-dimensional echocardiography was used in the prospective evaluation of 40 patients with the clinical diagnosis of dextrocardia. A segmental analysis of the situs, connections, ventricular anatomy, and chamber positions was utilized for a complete diagnostic assessment. An adequate examination was possible in 33 of these patients; the findings were confirmed by cardiac catheterization and angiography in 31 patients and at operation in 26. Use of the location of the liver and the drainage of the hepatic veins and inferior vena cava allowed atrial visceral situs to be defined in 33 patients (solitus 21, inversus 9, and ambiguous 3). Pulmonary venous connections were correctly identified in 27. In 33 patients, atrioventricular (AV) and ventriculoarterial connections and ventricular anatomy were correctly predicted. Twenty patients had 2 separate well-developed ventricles. Ventriculoarterial connections were determined correctly in all 20 patients: concordant in 5, discordant in 6, double-outlet right ventricle in 5, and single-outlet right ventricle (pulmonary atresia) in 4. In 16 patients a ventricular septal defect was correctly identified. In the remainder the ventricular septum was intact. Thirteen patients had univentricular heart: 8 had 2 AV valves (double-inlet ventricle) 3 had common AV inlet, and 2 had atresia of 1 AV connection. Two-dimensional echocardiography allowed the accurate assessment of complex congenital heart defects associated with dextrocardia. Utilizing a segmental approach, one can correctly predict atrial-visceral situs, ventricular morphology and situs, and AV and ventriculoarterial connections.