Outcomes of double switch procedure in patients with congenitally corrected transposition of the great arteries with or without associated VSD

Abstract

Anatomical repair by double switch procedure (DS) for patients with congenitally corrected transposition of the great arteries (ccTGA) remains under debate. The outcomes after DS for ccTGA without LVOTO, with specific focus on the left ventricle (LV) performance in patients with intact ventricular septum (IVS) were assessed. Since 1994, 39 patients underwent DS: 9 had an IVS, and 30 had an associated VSD. Palliative procedure consisted in pulmonary artery banding (PAB) for 31 patients (79%): 9 (twice in 5) for tricuspid regurgitation (TR) and 22 for hemodynamically significant associated VSD. Survival and LV performance in follow up were analyzed. PAB was efficient to diminish TR significantly in all 9. The median age at repair was 22 months (12 days–13 years). Associated VSD closure was performed in 76% of patients ( n = 30). Four patients required peri-operative pacemaker implantation. Hospital mortality occurred in 1 (2.5%: 70% CI 1–8%) neonate who underwent DS and aortic arch repair: he died because of low output syndrome. Two (5%) patients required early reoperation for pulmonary venous pathway stenosis release. Median follow up was 13.7 years (range: 3 months–26 years). Two late deaths occurred 16 and 84 months postoperatively because of refractory LV dysfunction: both patients had IVS and benefited from LV retraining. Six patients (16%) required 7 reoperations during follow up. At last visit, 5 patients (14%) presented with LV dysfunction (LV ejection fraction < 50%) requiring medical treatment while remaining 31 (84%) were asymptomatic. Actuarial survival curves are shown in Fig. 1 . Mortality was significantly higher in the IVS group compared to the VSD group (33% vs. 0%, P = 0.01). The DS appeared to be the procedure of choice for ccTGA associated with VSD. PAB was efficient for the management of TR in early life. Our Results suggest that the strategy of LV retraining by PAB to achieve DS at any price remains questionable.