Anatomic Repair of Congenitally Corrected Transposition: Reappraisal of Eligibility Criteria

Viktoria H M Weixler,Mi-Young Cho,Peter Kramer,Stanislav Ovroutski,Felix Berger,Joachim Photiadis,Peter Murin,Olga Romanchenko,Michael Hübler

doi:10.1007/s00246-022-02841-z

Viktoria H M Weixler, Mi-Young Cho + Show 7 more

Open Access

https://doi.org/10.1007/s00246-022-02841-z

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Abstract

Several criteria to identify suitable candidates for anatomic repair in congenitally corrected transposition (cc-TGA) have been proposed. The purpose of this study was to critically re-evaluate adequacy of these recommendations in our patient cohort. All cc-TGA patients undergoing anatomic repair between 2010 and 2019 were reviewed. Evaluated eligibility criteria for repair included age ≤ 15 years, LV mass index ≥ 45–50 g/m2, LV mass/volume ratio > 0.9–1.5 and systolic LV to right ventricle pressure ratio > 70–90% among others. Repair failure was defined as postoperative early mortality or LV dysfunction requiring mechanical circulatory support. Twenty-five patients were included (median [interquartile range] age at surgery 1.8 years [0.7;6.6]; median postoperative follow-up 3.2 years [0.7;6.3]). Median preoperative LV ejection fraction was 60% [56;64], indexed LV mass 48.5 g/m2 [43.7;58.1] and LV mass/volume ratio 1.5 [1.1;1.6], respectively. A total of 12 patients (48%) did not meet at least one of the previously recommended criteria, however, all but two patients (92%) experienced favorable early outcome. Of 7 patients (28%) with indexed LV mass < 45 g/m2, 6 were successfully operated. There were two early repair failures (8%) with LV dysfunction: one patient died and one required mechanical circulatory support but recovered well. Surgery was performed successfully in patients with LV mass and volume Z-scores as low as − 2 and − 2.5, respectively. Anatomic correction for cc-TGA can be performed with excellent early outcome and is feasible even in patients with LV mass below previously recommended cut-offs. The use of LV mass and volume Z-scores might help to refine eligibility criteria.

Highlights

Corrected transposition of the great arteries is a rare and complex congenital malformation, accounting for approximately 0.05% of all congenital heart defects [1]
Different types of anatomic repair aiming for correction of the double discordance have been proposed, combining an atrial stage switch (Senning or Mustard procedure) together with either arterial switch operation (ASO) as double switch operation or, in case of relevant left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD), as intra-ventricular rerouting by means of a Rastelli procedure [3]
pulmonary artery banding (PAB) was performed at a median age of 2.8 months [1.4;34.9] and median time interval between PAB and anatomic correction was 9.9 months [4.9;10.1]

Summary

Introduction

Corrected transposition of the great arteries (cc-TGA) is a rare and complex congenital malformation, accounting for approximately 0.05% of all congenital heart defects [1]. Suggested criteria mostly focus on LV pressure load and muscle mass, their appropriateness has not been broadly validated [11–13]. It remains unclear, to what extent such criteria apply to cc-TGA patients with an unrestrictive VSD. In general these patients might be perceived as suitable for anatomical correction since the morphological LV is supposedly “well-trained” due to continuous systemic pressure load and volume load, it is not self-evident that this will naturally translate into sufficient ventricular size or muscle mass development [14]

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