Contemporary early postoperative cone repair outcomes for patients with Ebstein anomaly
Introduction: Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormalities of the tricuspid valve (TV) leaflets and right ventricular (RV) cardiomyopathy. The current treatment for most patients with EA is cone reconstruction (CR) surgery. However, timing of repair has been highly variable, ranging from infancy to adulthood. Historically, surgical intervention for EA in the presence of severe RV failure has been associated with a longer intensive care unit course, increased need for ECMO support and a longer duration of mechanical ventilation. We sought to evaluate contemporary early postoperative outcomes in all age groups of patients with Ebstein anomaly following CR.
Methods: Retrospective chart review was undertaken for patients >1 year of age who had a CR for EA at our institution between 6/1/2007 and 12/31/2018. Short term complications included ECMO, delayed sternal closure, dialysis, adrenal suppression, cardiac arrest, mechanical ventilation >2 days, readmission or reoperation within 30 days, and death. Need for bidirectional Glenn (BDG) was also noted. Outcomes were compared by age and disease severity. A subset of patients diagnosed before age 1 (Group A1) was used to better delineate age effect. The incidence of outcomes was compared across age groups using the Cochran-Armitage test for trend.
Results: A total of 284 patients were reviewed (mean (SD) age 20.9 (16.9) years, 47% males), with 13%, 44%, 26% and 16% in age groups 1<4, 4<19, 19<40, and 40+, respectively. Group A1 included 175 patients. Severe disease was seen in 43 (15%) patients (group A1: 23, 13%). Only one patient died of cardiac/surgical causes. Short-term complications were seen in 14% of patients overall and 17% in group A1, with 5% of patients in both groups having a reoperation within 30 days. In the overall cohort, short-term complication rates did not vary significantly across the age groups (Figure 1, p=0.18) or between those with mild/moderate vs. severe disease (12.9% vs. 18.6%, p=0.31). Decreasing rates of short-term complications were seen with increasing age in group A1 (Figure 1, p=0.13). Hospital length of stay decreased with age (Spearman rank correlation -0.41 overall, -0.35 group A1) and plateaued after age 40 in the overall cohort. The need for BDG was inversely associated with age (Figure 1, both p<0.01) and was more common among patients with severe disease (overall: 35% vs. 14%, p<0.001; group A1: 57% vs. 20%, p<0.001).
Conclusions: Overall, patients of all ages who undergo CR for EA have good early, post-operative outcomes with low reoperation rates and mortality. Bidirectional Glenn is more often required in younger patients and in those with severe disease. Younger adults tend to have the shortest hospital stays; however there is no difference in ICU complications or reoperation rates between all age groups.