Systemic Atrioventricular Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Vessels

BACKGROUND Limited data exist regarding the long-term outcomes of systemic atrioventricular valve (SAVV) inter-vention (morphologic tricuspid valve) in congenitally corrected transposition (ccTGA).OBJECTIVES The goal of this study was to evaluate the mid-and long-term outcomes of SAVV surgery in ccTGA.METHODS We performed a retrospective review of 108 ccTGA patients undergoing SAVV surgery from 1979 to 2022. The primary outcome was a composite endpoint of mortality, cardiac transplantation, or ventricular assist device implantation. The secondary outcome was long-term systemic right ventricular ejection fraction (SVEF). Cox proportional hazard and linear regression models were used to analyze survival and late SVEF data.RESULTS The median age at surgery was 39.5 years (Q1-Q3: 28.8-51.0 years), and the median preoperative SVEF was 39% (Q1-Q3: 33.2%-45.0%). Intrinsic valve abnormality was the most common mechanism of SAVV regurgitation (76.9%). There was 1 early postoperative mortality (0.9%). Postoperative complete heart block occurred in 20 patients (18.5%). The actuarial 5-, 10-, and 20-year freedom from death or transplantation was 92.4%, 79.1%, and 62.9%. The 10-and 20-year freedom from valve reoperation was 100% and 93% for mechanical prosthesis compared with 56.6% and 15.7% for bioprosthesis (P < 0.0001). Predictors of postoperative mortality were age at operation (P = 0.01) and preoperative SVEF (P = 0.04). Preoperative SVEF (P < 0.001), complex ccTGA (P = 0.02), severe SAVV regurgitation (P = 0.04), and preoperative creatinine (P = 0.003) were predictors of late postoperative SVEF.CONCLUSIONS SAVV surgery remains a valuable option for the treatment of patients with ccTGA, with low early mortality and satisfactory long-term outcomes, particularly in those with SVEF $40%. Timely referral and accurate patient selection are the keys to better long-term outcomes.