Late results of repair of truncus arteriosus.

Over the last three decades, management of truncus arteriosus has evolved with improving outcomes. Surgical repair is currently performed primarily during the neonatal period. This has prevented the severe morbidity and mortality resulting from congestive heart failure and pulmonary vascular obstructive disease. Although it has been 30 years since successful surgical repair of this lesion, the long-term follow-up studies have been limited to children who underwent truncus repair beyond the infancy period. In this article, we review the literature and also summarize the long-term results of truncus arteriosus at the University of California, San Francisco, where a repair in early infancy has been routine since 1975. A retrospective review was performed to assess long-term outcomes among 165 patients (81% of patients were < 1 year of age) who survived the initial hospital stay following complete repair of truncus arteriosus since 1975. There have been 23 late deaths, eight of which occurred within 6 months of repair and 13 of which occurred within 1 year. Ten of the late deaths were related to reoperations. The actuarial survival rate among all hospital survivors was 90% at 5 years, 85% at 10 years, and 83% at 15 years, and was essentially identical for infants alone. Significant independent risk factors for poorer long-term survival were truncus with moderate to severe truncal valve insufficiency before repair. During the follow-up period, 107 patients underwent 133 conduit reoperations at a median of 5.5 years after the initial repair. In addition, 26 patients underwent 30 truncal valve replacements. Actuarial freedom from truncal valve replacement was 63% at 10 years among patients with truncal insufficiency before initial repair. Ten- to 20-year survival and functional status were excellent among infants undergoing complete repair of the truncus arteriosus. Copyright 1998 by W.B. Saunders Company