Key result
Moderate-severe or severe atrioventricular valve regurgitation was associated with significantly worse 15-year survival compared with no or mild regurgitation (58.3% vs 89.2%; P=0.033).
Why the study?
Survival and the incidence of Fontan completion in patients with single-ventricle and concomitant unbalanced atrioventricular septal defect required long-term analysis.
Cohort (n=151)
Yes
Absolute Event Rate: 58.3% vs 89.2%
p-value: p=.033
OBJECTIVE: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. METHODS: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. RESULTS: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. CONCLUSIONS: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.
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Arrigoni et al. (2021) conducted a cohort in Single-ventricle and concomitant unbalanced atrioventricular septal defect (n=151). Atrioventricular valve procedure vs. No atrioventricular valve procedure was evaluated on 15-year survival (moderate-severe/severe vs no/mild regurgitation) (p=.033). Moderate-severe or severe atrioventricular valve regurgitation was associated with significantly worse 15-year survival compared with no or mild regurgitation (58.3% vs 89.2%; P=0.033).
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