LATE RESULTS OF SYSTEMIC ATRIOVENTRICULAR VALVE-REPLACEMENT IN CORRECTED TRANSPOSITION

From December 1964 to October 1993, 40 patients (aged 5 months to 70 y ears, mean 21.8 years, median 13.6 years) with corrected transposition and systemic atrioventricular valve insufficiency underwent replaceme nt (n = 39) or repair (n = 1) of the systemic atrioventricular valve, Thirty-nine patients had situs solitus and 1 had situs inversus, Assoc iated anomalies included Ebstein's malformation of the systemic atriov entricular valve (n = 22), ventricular septal defect (n = 19), and pul monary stenosis (n = 14), Preoperatively, 16 patients (40.0%) had comp lete heart block and 27 patients (67.5%) were in New York Heart Associ ation functional classes III and N. The early mortality was 10.0% (n = 4) and 8 patients died subsequently, The principal cause of death in all 12 patients was systemic ventricular failure, Overall survival inc luding early mortality was 78.0% at 5 years and 60.7% at 10 years; sur vival excluding early mortality was 86.7% at 5 years and 67.5% at 10 y ears, Survivorship correlated with preoperative systemic ventricular e jection fraction of 44% or more (p < 0.001) and later interval of oper ation (9 deaths in 15 patients before 1981 versus 3 deaths in 25 patie nts subsequently) (p = 0.06), There were no cases of surgically induce d complete heart block. Two patients underwent late reoperations relat ed to the systemic atrioventricular valve prosthesis, Follow-up extend ed to 26.0 years (median 4.7 years). At last follow-up, 18 of the 28 s urvivors were in New York Heart Association functional class I, 9 were in class II, and 1 was in class III, We conclude that the results of systemic atrioventricular valve replacement in corrected transposition have improved significantly during the past decade. To preserve syste mic ventricular function, we suggest operation be considered at the ea rliest sign of progressive ventricular dysfunction as assessed by seri al clinical evaluation and echocardiography.