RESULTS OF A POLICY OF PRIMARY REPAIR OF TRUNCUS ARTERIOSUS IN THE NEONATE

Although the early mortality for repair of truncus arteriosus has decr eased in the modem era, routine correction in the neonate has not been widely adopted. To assess the results of our protocol of early repair , we reviewed 46 neonates and infants undergoing repair of truncus art eriosus at the University of Michigan Medical Center from January 1986 to January 1992. Their ages ranged from 1 day to 7 months (median 13 days) and weights from 1.8 kg to 5.4 kg (mean 3.1 kg). Repair was perf ormed beyond the first month of life in only 8 patients, because of la te referral in 7 and severe noncardiac problems in 1. Associated cardi ac anomalies were frequently encountered, the most common being interr upted aortic arch (n = 5), nonconfluent pulmonary arteries (n = 4), hy poplastic pulmonary arteries (n = 4), and major coronary artery anomal ies (n = 3). Truncal valve replacement was performed in 5 patients wit h severe regurgitation, 3 of whom also had truncal valve systolic pres sure gradients of 30 mm Hg or more. The truncal valve was replaced wit h a mechanical prosthesis in 2 patients and with a cryopreserved homog raft in 3 patients. Right ventricle-pulmonary artery continuity was es tablished with a homograft in 41 patients (range 8 mm to 15 mm), a val ved heterograft conduit in 4 (range 12 mm to 14 mm), and a nonvalved p olytetrafluoroethylene tube in the remaining patient (8 mm). There wer e 5 hospital deaths (11 %, 70% confidence limits 7% to 17%). Multivari ate and univariate analyses failed to demonstrate a relationship betwe en hospital mortality and age, weight, or associated cardiac anomalies . Only 1 death occurred among 9 patients with interrupted aortic arch or nonconfluent pulmonary arteries. Hospital survivors were followed-u p from 3 months to 6.3 years (mean 3 +/- 0.4 years). Late noncardiac d eaths occurred in 3 patients, all within 4 months after the operation. Actuarial survival was 81% +/- 6% at 90 days and beyond. Despite the prevalence of major associated conditions, early repair has resulted i n excellent survival. We continue to recommend repair promptly after p resentation, optimally within the first month of life.