Since reparative surgery without cardiac catheterization has been advocated for certain lesions such as total anomalous pulmonary venous connection (TAPVC), it is important to assess the accuracy of two-dimensional (2-D) echocardiography in the prospective diagnosis of this lesion. Abandoning traditional cardiac catheterization and angiography would be justified only if all surgically relevant anatomic features could be consistently displayed noninvasively. Consequently, we reviewed our experience with TAPVC to determine the suitability of subcostal 2-D echocardiography as the sole prospective diagnostic tool. Over a 5-year period, of 2444 infants under 2 years of age who underwent subcostal 2-D echocardiography, 38 were diagnosed as having TAPVC as the only major cardiac defect. There were no false-negative or false-positive cases during the time of the study. Drainage sites were diagnosed correctly in 36 (95%). Of the five cases of mixed-type TAPVC, the second drainage site was missed by 2-D echocardiography in two cases.