Management strategy and long-term outcome for truncus arteriosus.

OBJECTIVE: Evaluation of a consistent policy of elective repair of truncus arteriosus at 2-3 months of age for the patients who are independent of hospital ward care, and long-term outcome.
METHODS: Retrospective study of 82 patients with truncus arteriosus who underwent total repair at the Victorian Paediatric Cardiac Surgical Unit between 1979 and December 1995. The timing was based on a consistent policy of elective repair at 2-3 months of age for patients who were independent of hospital ward care. Earlier repair was performed when the patients were in uncontrolled congestive heart failure.
RESULTS: Follow-up was complete for all patients with a mean of 76 months (1-183). There were 11 hospital deaths (13.4% CL 9-18.5), and five late deaths, actuarial survival at 80 months was 81% (CL 70-88%) with 39 patients uncensored at that point. For the purpose of this presentation, patients have been grouped according to their age at repair; 1, neonates n = 17 (hospital mortality = 5); 2, infants 1-6 months of age n = 48 (hospital mortality = 4); 3, patients beyond 6 months n = 17 (hospital mortality = 2). This series includes 10 patients with interrupted aortic arch with no mortality, and 10 patients with discontinuous pulmonary artery (hospital mortality = 2). Thirty-seven patients have had 54 conduits replaced to date. Using multiple regression, body weight < 3 kg was the only significant independent risk factor for hospital mortality.
CONCLUSIONS: Our management policy tended to gather patients with risk factors described elsewhere into presentation group 1 and low risk truncus patients into presentation group 2. Deferral of surgery to 2-3 months of age is possible and lowers the surgical risk.