Truncus Arteriosus repair: The implications of truncal valve abnormalities and interrupted arch on surgical mortality

Truncus Arteriosus (TA) is a heterogeneous disease that shares a common entity: failed septation of the embryonic truncus and conus. Other structural cardiovascular anomalies can coexist that may contribute to the overall mortality associated with complete surgical repair. We reviewed retrospectively the outcome of patients who underwent complete repair of TA between 1990 and 1998 at our institution (n=54). Overall early mortality for these patients was 29.6% (16/54). Further analysis of the results revealed that the pre-operative presence of two specific coexisting cardiovascular defects was associated with high mortality in patients with TA. First, the pre-surgical state of the truncal valve was an important indicator for survival. Mortality was 100% for patients with grade 3 truncal valve stenosis (3/3) or grade 3 truncal valve insufficiency (4/4) as demonstrated on preoperative echocardiography. Second, the presence of an interrupted aortic arch (IAA) was also associated with 80% early mortality (4/5; 1/5 had both IAA and grade 3 truncal insufficiency). Thus, for patients without severe truncal valve abnormalities or the presence of an interrupted aortic arch, overall early mortality was 14% (6/43). These data suggest that in the current era of paediatric cardiac surgery, complete repair of TA can be carried out with a reasonable early mortality in the absence of severe truncal valve stenosis or insufficiency and in the absence of an interrupted aortic arch. Either one must ask whether infants with severe truncal valve abnormalities or IAA should undergo complete repair or, new strategies to deal with truncal regurgitation, truncal stenosis and IAA must be developed.