TAUSSIG-BING ANOMALY WITH AND WITHOUT AORTIC ARCH PATHOLOGY, SINGLE CENTRE EXPERIENCE WITH ANATOMICAL CORRECTION

Background: Taussig-Bing anomaly (TBA) is a rare congenital cardiac anomaly and frequently associated with aortic arch pathology (more than 50% of the patients) such as interrupted aortic arch (IAA), coarctation and arch hypoplasia. The aim of the present report is to describe our results of TBA correction. Methods: Between 12/2007 to 08/2023, 12 patients with TBA underwent surgical correction at our Institution. Ten patients (83%) underwent single stage neonatal anatomical correction with ASO, one had staged repair with anatomical correction with ASO performed at 4 months of age and the other one had Kawashima operation. Results: There were 7 male and 5 female patients, with a median age of 23 days (7 dys to 12 Yrs) and median body weight of 3.5 kg (2.8-35 kg). Lecompte manoeuvre was performed in 8 patients of 11 who underwent ASO. There were no early deaths. Only 1 late death (8.3%) due to pulmonary hypertension. Median follow-up was 6.3 years (7.8mon-16,4yrs). Overall survival was 100% at 1 year, 91.7% at 2, 5, 10, 16yrs. All patients were in NYHA class I at last FU. Freedom from reintervention was 91.7% (95% CI) and 66.7% (95% CI) at 1 and 5yrs, respectively. The most common reintervention was RVOT patch enlargement a median time of 10 months after ASO. Conclusions: Patients with Taussig-Bing anomaly and aortic arch obstruction can undergo neonatal single stage ASO with excellent survival and very low morbidity. In complex coronary anomalies staged repair may represent a safe strategy to manage TBA.