Surgical outcomes of anatomic repair in congenitally corrected transposition of the great arteries: a 28-year, multicentre Dutch experience

Abstract

Objectives: Anatomic repair is the contemporary preferred management strategy for congenitally corrected transposition of the great arteries (ccTGA). Most outcome data derive from small single-centre series. The goal of this study was to review long-term outcomes of surgical treatment in a multicentre cohort of ccTGA patients in the Netherlands over a 28-year period.

Methods: All patients with ccTGA undergoing anatomic repair (double switch repair, arterial switch operation with hemi-Mustard/bidirectional Glenn, or Rastelli/Nikaidoh with Mustard or hemi-Mustard/bidirectional Glenn) between 1997 and 2025 were analysed retrospectively.

Results: Sixty-two patients underwent anatomic repair at a median age of 2.5 (IQR: 1.4-4.0) years. Overall hospital mortality was 6.5%, decreasing to 1.9% after 2006. At a median follow-up of 11.9 years, the 20-year survival exceeded 80%. Reoperation was required in 31%, most often for conduit replacement after the Rastelli/Nikaidoh procedure, baffle obstruction or neoaortic valve/root disease after a double switch procedure, and least often after the arterial switch with hemi-Mustard/bidirectional Glenn (11.7%). Percutaneous interventions were performed in 16%. Complete atrioventricular block requiring a pacemaker implant occurred in one-third, whereas late atrial arrhythmias were infrequent (<10%). At the latest follow-up, systemic left ventricular function was preserved in most patients, with only 2 patients showing moderate dysfunction. Conclusions: After an initial learning curve, anatomic ccTGA repair exhibited low operative mortality, good long-term survival and preserved systemic left ventricular function in the majority of patients. Reintervention rates are considerable.