Introduction:
Truncus arteriosus is an infrequent cyanotic congenital heart disease that occurs at a rate of 0.56–1.3 per 10,000 live births (1). Surgical mortality in patients with truncus arteriosus is high relative to other congenital heart disease and is reported to be between 6.9–11% (2, 3). Few studies had evaluated outcomes and risk factors for mortality in truncus arteriosus; these studies were limited by either small sample size or single center studies (4–6). The main objective of this study was to identify patient and hospitalization characteristics associated with mortality in infants with truncus arteriosus who had surgical repair. We evaluated 3,009 infants who underwent truncus arteriosus surgical repair. This is the largest cohort of patients with repaired truncus arteriosus . We hypothesized that using data from a large multicenter national database with both a large number of patients and a wide range of practice variations would allow us to evaluate the surgical outcomes better and to identify the factors associated with mortality in infants who had truncus arteriosus repair. We also sought to evaluate the resource utilization in the subgroup of subjects with truncus arteriosus and 22q11.2 deletion syndrome. Syndrome of 22q11.2 deletion occurs in 25% of patients with truncus arteriosus. We hypothesize that having 22q11.2 deletion syndrome would influence the resource utilization in the postoperative period, as these subjects would have a prolonged and more complicated postoperative course given the presence of immunodeficiency and multiple non-cardiac anomalies that are associated with 22q11.2 deletion syndrome (7).