Background: The excellent results for repair of truncus arteriosus reported in some centers have not applied to patients with associated interrupted aortic arch. This work aims at understanding the discrepancy of results in our own experience.
Patients and methods: Ten patients among 83 consecutive children with truncus arteriosus repaired from 1987 to September 2004 who had aortic arch interruption were analyzed, with particular emphasis on clinical presentation and outcome. The comprehensive Aristotle complexity score was calculated for each patient. The Kaplan-Meier method was used to estimate survivals.
Results: Preoperative mechanical ventilation was necessary in 5 of the 10 patients; 2 of them were moribund. Associated major lesions were as follows: severe (n = 2) and moderate (n = 4) truncal valve regurgitation, coronary artery anomalies (n = 3) and Di-George's syndrome (n = 4). The comprehensive Aristotle score was at least 20 in 6 patients. There were 5 operative deaths (5 of 10); early mortality was 50% (95% confidence limits: 19% to 81%). These deaths occurred in patients with Aristotle score of 20 or greater (5 of 6 = 83%). All 4 patients who had no moderate or severe truncal valve regurgitation survived the intervention. Survival was a low 37.5% +/- 16.1% from 1 year on compared with a high 95.5% +/- 2.5% for the 73 patients without aortic arch interruption.
Conclusions: This study confirms the predictive value of the Aristotle score, hospital mortality being significantly correlated with the highest Aristotle score (p = 0.024). To improve outcome in these high-risk patients, preoperative management should be optimized, repair should not be delayed, and regurgitant truncal valve should be repaired or replaced.