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

^a The Labatt Family Heart Centre, Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
^b King Faisal Heart Institute, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

Received 7 September 2007; received in revised form 14 November 2007; accepted 20 November 2007.

^_* Corresponding author. Address: King Faisal Heart Institute (MBC 16), King Faisal Specialist Hospital and Research Centre, P.O. Box 3354, Riyadh 11211, Saudi Arabia. Tel.: +966 1 464 7272x39455; fax: +966 1 442 7791. (Email: balsoufi{at}hotmail.com; glen.vanarsdell{at}sickkids.on.ca).

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.

Key Words: Congenital heart disease • Great vessels anomalies • Reoperation • Arterial switch