Improved results with single-stage total correction of Taussig-Bing anomaly

Abstract

Objective: The arterial-switch operation (ASO) for management of Taussig-Bing anomaly is associated with important morbidity, mainly related to multiple associated cardiac anomalies. Our surgical management has evolved to suggest a single-stage total repair strategy tailored to address all abnormalities on an individual basis. We examine the efficacy of this treatment approach.

Methods: Thirty-three children, (infants n=29), with Taussig-Bing underwent ASO (1979-2005). In our earlier experience (group 1, n=17), initial palliation was performed as needed, including pulmonary-artery banding (n=9), coarctation repair (n=6), and atrial septostomy (n=3) followed by ASO at a later age. In our later experience (group 2, n=16), single-stage total repair was performed; ASO with ventricular septal defect closure and baffling of left ventricle to neo-aorta performed in neonates with arch obstruction (n=8), or at age 6 weeks in those with no arch obstruction (n=8). Concomitant relief of right-ventricle outflow-tract obstruction (RVOTO) was performed in 14 patients. Demographics and operative variables affecting outcomes were analyzed.

Results: Mean age at operation for group 1 and 2 patients was 312+/-477 and 42+/-31 days (p<0.0001). Aortic arch obstruction (52%), sub-aortic RVOTO (61%) and unusual coronary patterns (52%) were similar for both groups. One-year survival for group 1 and 2 patients was 47+/-5% and 100% (p=0.001). Associated anomalies such as great vessels position, arch obstruction, and unusual coronaries were not significant risk factors for mortality on multivariable analysis. Ten-year freedom from RVOT and arch re-operation was 55+/-5% and 96+/-4%. Five-year event-free survival for groups 1 and 2 was 35+/-6% and 87+/-1% (p=0.0016). Significant factors affecting event-free survival were group 1 (HR 108, p=0.0005), and larger weight at surgery (HR 1.3, p=0.02).

Conclusions: The Taussig-Bing anomaly is complex and often associated with other cardiac anomalies (arch obstruction, RVOTO, unusual coronary pattern). Advances in perioperative care have significantly mitigated mortality. In our experience with single-stage total repair, event-free survival, especially freedom from RVOT re-operation, has significantly improved.