Outcomes of left atrioventricular valve operation following atrioventricular septal defect repair

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BackgroundA left atrioventricular valve (LAVV) operation following repair of an atrioventricular septal defect (AVSD) can be challenging. We sought to describe characteristics and outcomes of patients requiring LAVV operation. MethodsThis was a retrospective review of AVSDs requiring LAVV operation between 2000 and 2020. Patients who experienced adverse events (AEs), defined as the need for a LAVV reoperation (repair or replacement) or death, were compared to patients without AEs. Freedom from AEs was determined using the Kaplan-Meier method. Reoperation and death were characterized in terms of cumulative incidence function, estimated using competing risk models. ResultsOf 843 patients with an AVSD repair, 59 (7.3%) required an LAVV operation and 7 (9%) needed valve replacement. A simple repair (cleft closure and/or annuloplasty) was performed in 26 patients (48.1%) and complex repair using multiple techniques was performed in 28 patients (51.8%). Eleven patients (20%) required further LAVV reoperation, including replacement of mechanical valve in 3, new valve replacement in 6 (2 Melody, 4 mechanical) and re-repair in 2. The cumulative incidence of freedom from AE was 84.1% (95% confidence interval [CI], 75.0%-94.2%) at 1 year, 78.3% (95% CI, 68.2%-90.0%) at 5 years, 73.4% (95% CI, 62.2%-86.7%) at 10 years, and 69.7% (95% CI, 57.5%-84.7%) at 15 years. Cox univariable regression identified lighter weight (P = .027) and early need for LAVV operation (P = .02) as associated with AEs and cleft closure (P = .003) as protective against AEs. The estimated cumulative incidence of reoperation was higher in complex repairs (17.3% [95% CI, 7.8%-38.7%] vs 0.5% [NA] at 1 year (P = .02, Gray's test)). A comparison of eras—2000-2009 and 2010-2020—showed no difference in AEs (P = .96, Grays test). ConclusionsAdverse outcomes remain common following LAVV operation. Smaller infants and infants requiring earlier operation and complex type repairs are at greatest risk. Future studies should focus on which high-risk LAVVs are more suited to early LAVV replacement.