One hundred consecutive patients with situs solitus of the atria and tricuspid atresia have undergone surgical repair since 1968. In patients with ventriculoarterial concordance, a nonvalved Dacron conduit or an aortic valve homograft was interposed between the right atrium and right ventricular outlet chamber. In patients with ventriculoarterial discordance, an aortic valve homograft established continuity between the right atrium and pulmonary artery. Among 73 patients with ventriculoarterial concordance, the hospital mortality rate was 11% (eight deaths), and in 27 patients with ventriculoarterial discordance, it was 15% (four deaths). Before 1974, the year of operation was a significant determinant of hospital mortality (p less than 0.001). Thereafter, the hospital mortality declined and is currently 3.7%. In 82 patients with ages ranging from 4 to 16 years, there were six deaths (7.3%) whereas there were six deaths (33.3%) in 18 patients less than 4 or more than 16 years (p less than 0.001). Mode of ventriculoarterial connection and type of repair did not influence significantly the hospital mortality. There were six late deaths due to infection (two), reoperation (two), heart failure (one), and sudden death (one). Regardless of the mode of ventriculoarterial connection, use of a homograft valve produced better results, i.e., more asymptomatic patients (NYHA Class I) (p = 0.0168) and higher postoperative exercise capacity. Postoperative catheterization data and angiocardiographic measurements in patients with ventriculoarterial concordance demonstrated significant advantages with the interposition of a homograft valve between the right atrium and outlet chamber. Of 82 surviving patients, 94% are in NYHA Class I or II.