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).