Outcome of univentricular repair in patients with Down syndrome

Total cavopulmonary connection (TCPC) is rarely performed for a functional single ventricle in children with Down syndrome; therefore, the postsurgical outcomes are not well known. We evaluated mortality and related factors after TCPC in children with Down syndrome.

Between January 2004 and March 2010 we identified 8 patients with Down syndrome among 235 patients who had undergone TCPC. The preoperative clinical course, preoperative data, and postoperative clinical course were evaluated. In addition, clinical parameters and postoperative clinical course were compared between children with Down syndrome (n = 8) and a non-Down syndrome group (n = 227).

The median age at the time of TCPC was 4.1 years (range, 3.4-5.5 years), and the preoperative mean pulmonary artery pressure was 13.9 ± 1.81 mm Hg. We observed respiratory complications in 2 patients, surgical site infection in 3 patients, and chylothorax in 2 patients. No significant difference was observed in preoperative data and mortality rate (1 of 8 patients [12.5%] in the Down syndrome group; 5 of 227 patients [2.2%] in the non-Down syndrome group) between the groups of children with and without Down syndrome. However, when the postoperative clinical course was examined, the durations of intensive care unit stay (P = .009) and hospital stay (P = .007) were found to be significantly prolonged in the Down syndrome group.

Patients with Down syndrome tend to show prolonged recovery after TCPC. However, as opposed to previous reports, the mortality rate of patients undergoing TCPC is lower with no significant difference from that of children without Down syndrome.