Late left ventricular dysfunction after anatomic repair of congenitally corrected transposition of the great arteries

Objective Early results for anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) are excellent. However, the development of left ventricular dysfunction late after repair remains a concern. In this study we sought to determine factors leading to late left ventricular dysfunction and the impact of cardiac resynchronization as a primary and secondary (upgrade) mode of pacing. Methods From 1992 to 2012, 106 patients (median age at surgery, 1.2 years; range, 2 months to 43 years) with ccTGA had anatomic repair. A retrospective review of preoperative variables, surgical procedures, and postoperative outcomes was performed. Results In-hospital deaths occurred in 5.7% (n = 6), and there were 3 postdischarge deaths during a mean follow-up period of 5.2 years (range, 7 days to 18.2 years). Twelve patients (12%) developed moderate or severe left ventricular dysfunction. Thirty-eight patients (38%) were being paced at latest follow-up evaluation. Seventeen patients had resynchronization therapy, 9 as an upgrade from a prior dual-chamber system (8.5%) and 8 as a primary pacemaker (7.5%). Factors associated with left ventricular dysfunction were age at repair older than 10 years, weight greater than 20 kg, pacemaker implantation, and severe neo-aortic regurgitation. Eight of 9 patients undergoing secondary cardiac resynchronization therapy (upgrade) improved left ventricular function. None of the 8 patients undergoing primary resynchronization developed left ventricular dysfunction. Conclusions Late left ventricular dysfunction after anatomic repair of ccTGA is not uncommon, occurring most often in older patients and in those requiring pacing. Early anatomic repair and cardiac resynchronization therapy in patients requiring a pacemaker could preclude the development of left ventricular dysfunction.