Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: A 19-year experience

Objective: The surgical indication, timing, strategy, and surgical technique for anatomic correction of congenitally corrected transposition of the great arteries are challenging. We evaluated the long-term results at The Royal Children's Hospital Melbourne. Methods: Review of 32 successive anatomic corrections between 1996 and 2015. Results: Twenty-one double-switch (66%), 6 Senning/Bex-Nikaidoh (19%), and 5 Senning/ Rastelli (16%) procedures were performed (median age, 1.9 years). Median follow-up was 5.4 years with 4 deaths and 1 heart transplant. Cumulative incidence of late reoperation was 8%, 29%, and 59% at 1, 5, and 10 years, respectively. Twenty-six patients had full follow-up with native hearts. Nineteen had normal left ventricle (LV) function. Late LV dysfunction, mostly mild, was not related to needing a pacemaker (P = .4) or a pulmonary artery band (PAB) (P = .08). Previous PAB was linked to the need for aortic valve surgery or neoaortic regurgitation moderate or greater (P = .03). Six required Senning revision. The introduction of the Shumacker modification of the Senning has generated stable pulmonary venous pathways. Six patients developed postoperative iatrogenic atrioventricular block dependent on a permanent pacemaker. Conclusions: Anatomic correction is a surgical challenge. It provides excellent functional outcomes in survivors with a significant need for reoperation and a definite risk of death or transplantation. Normal LV function should be expected in most patients. LV dysfunction was not linked to PAB or pacemaker requirement but surgery without LV training had better long-term LV function. The Shumacker modification provided stable venous pathways. Iatrogenic atrioventricular block remains a challenge.