Current mid-term outcome with an integrated surgical strategy for correction of d-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction

Abstract

Objectives: The optimal surgical treatment strategy for transposition of the great arteries (TGA) associated with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) leading to the best long-term outcome has not been established yet. The wide range of anatomical variability has led to the development of different surgical treatment options. In a retrospective single-centre review, we aimed to report the long-term outcome in patients who underwent biventricular repair.

Methods: Between 1992 and 2015, 49 patients underwent biventricular repair for TGA with VSD and LVOTO. On the basis of anatomical substrate, the type of repair was chosen. According to the type of repair, the patients were divided into two groups. Group A (n = 30 patients) includes all patients who underwent intraventricular rerouting (Rastelli, n = 22; réparation à l'étage ventriculaire, n = 8); Group B patients (n = 14) had repair on an arterial level (arterial switch operation + VSD closure + LVOTO resection, n = 10; Bex-Nikaidoh, n = 4).

Results: The follow-up was completed in 90% with a median follow-up of 12.3 years (17.1-17.7 years). There was no difference in survival (Group A: 97% at 5, 10 and 15 years; Group B: 92% at 5, 10 and 15 years) and functional outcome between the groups. All patients were in New York Heart Association class I, having sinus rhythm and normal left ventricular (LV) function. Neurological and educational development was adequate for age in all but one who suffered from attention-deficit hyperactivity disorder. Group A exhibited significantly less freedom from right ventricular outflow tract (RVOT) reoperations/reintervention (79, 48 and 21% at 5, 10 and 15 years) (P = 0.008) due to RVOT dysfunction and endocarditis. Event-free LVOTO survival in Group A at 5, 10 and 15 years amounted to 96, 91 and 91% and was similar to Group B with event-free LVOTO survival at 5, 10 and 15 years of 100, 88 and 88%, respectively.

Conclusions: LV function is well preserved irrespective of the type of procedure. Patients who had undergone intraventricular rerouting (Group A) suffered from recurrent RVOT interventions due to RVOT dysfunction and endocarditis. The neurological outcome and the educational level were adequate for age in both groups. Executing the appropriate type of surgery for the individual patient's anatomy provides excellent functional and event-free outcome.

Keywords: Bex–Nikaidoh procedure; Left ventricular outflow tract obstruction; Rastelli procedure; Réparation à l’étage ventriculaire procedure; Transposition of the great arteries; Ventricular septal defect.