Optimizing Operative Decision Making for Complex Transposition with Left Ventricular Outflow Tract Obstruction in the Modern Era

Purpose: Studies that have assessed the Rastelli and Nikaidoh operations for complex transposition with left ventricular outflow tract obstruction (LVOTO) have failed to evaluate the anatomical drivers that may contribute to surgical selection and successful outcomes. We present our procedural selection process for optimizing outcomes of the Nikaidoh and Rastelli operations.

Methods: This is a retrospective assessment of an anatomical decision-making algorithm for selecting patients to undergo a Nikaidoh or Rastelli operation for complex transposition of the great arteries (TGA) with LVOTO. The aim of this study was to evaluate the effectiveness of the phenotypically-driven decision analysis approach at mitigating the risks and complications that arise from the Nikaidoh and Rastelli operations. Procedural selection was based on the following patho-anatomical features: right ventricular hypoplasia, restrictive or inlet ventricular septal defect (VSD) by echocardiography or surgeon visualization, straddling atrioventricular valve (AVV) or abnormal valvular attachments, anomalous coronary anatomy, pulmonary annular dimension < 5mm, and great vessel orientation. This study included pediatric patients from a single center who underwent surgical repair of TGA-LVOTO, congenitally corrected-TGA, or DORV-TGA type-LVOTO with a Nikaidoh or Rastelli operation from January 2004 to January 2021. Patients who underwent a Yasui operation or had a concomitant arch reconstruction were excluded.

Results: 34 patients underwent repair with 50% (n=17) Nikaidoh and 50% (n=17) Rastelli operations. A Nikaidoh was chosen if patient's had straddling AVV (27% vs 0%), right ventricular hypoplasia (64% vs 0%), and restrictive or inlet VSD (84% vs 6%), all p< 0.05. Rastelli was more commonly pursued with side-by-side great vessel anatomy (71% vs 6%, p=0.03). There were no differences in procedural selection based on anomalous coronary anatomy or re-operative status, and the pulmonary annular dimensions were similar between the groups, all p>0.05. We found that by applying an anatomical selection approach, the composite risks of reoperation, catheter-based readmission, readmission, and death were statistically similar between the groups (p=0.08). Early outcomes were similar between the Nikaidoh and Rastelli groups in-terms of 1-year mortality (6% (n=1) vs 0%), post-operative ECMO support (18% vs 18%), and median length of stay 13 days [6-25] vs 11 days [6-16], all p>0.05. At 10-year follow-up, there were similar rates of reoperation (35% vs 41%, p=0.37) between the Nikaidoh and Rastelli groups but higher rates of catheter-based interventions (6% vs 29%, p=0.04) in the Rastelli group. When structured as a competing risk analysis, rates of death and reoperation were similar at 10-years (Figure).

Conclusion: To optimize surgical success and mitigate the risks of both the Nikaidoh and Rastelli operations for repair of complex transposition with LVOTO in the modern era, preoperative anatomical features should guide procedural selection. Using this approach enhances outcomes and allows for similar rates of early complications and late reinterventions.

Identify the source of the funding for this research project: None