Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study

Abstract

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 15year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P ¼ .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.

National outcomes of the Fontan operation with endocardial cushion defect

“…While there is no direct information of the type of ventricular hypoplasia in our study, an associated diagnosis of HLHS in the ECD group (17%, 62/360 patients) in our study cohort indirectly suggests that left ventricular hypoplasia was at-least present seen in 17% of these patients undergoing a FO and is in agreement with prior studies. [7][8][9] Second, ECD can occur with conotruncal anomalies such as transposition of great arteries (TGA) 3 and DORV. This combination of TGA and ECD is generally associated ventricular hypoplasia (95% of cases).…”

“…It is known that particularly a successful valve repair at the time of FO has positive prognostic impact on survival. 8,23 Whereas any AV valvular insufficiency more than moderate in degree is associated with diminished survival and it is recommended that such degree of valvar insufficiency be addressed at the time of FO. 8 This is line with our study with the observation that despite 2.6% (87/ 3380) of the patients having valve repair/replacement, all these patients were in the survivor group.…”

“…In previous studies, the discharge mortality in the ECD group is between 3.8% and 7%. 8,9 However, these studies also included historical patients undergoing FO in the 1960s and beyond. In multivariable analysis, ECD was an independent risk predictor for a mortality in our study.…”

“…7 However, once a patient can undergo a successful FO, the long-term prospects are reasonable in the absence of more than moderate valvular insufficiency and good ventricular function, particularly in the recent times. 8,9 Suboptimal results for ECD are likely due to association with Down syndrome, 6,13 HS, 6,22 AV valvar insufficiency, 8,23 and early propensity to develop pulmonary vascular disease particularly if the pulmonary vascular bed is unprotected. 19,26 Interestingly, despite the higher incidence of pulmonary hypertension in ECD patients, it was not an independent predictor for mortality for a FO.…”

“…The traditional outcomes of the FO in these patients have been suboptimal due to abnormalities in the atrioventricular valves, presence of associated syndromes such as Down syndrome and Heterotaxy syndrome (HS) 4–7 . Previous studies have been limited by the smaller number of patients with ECD who underwent a FO, longer duration of the study spanning more than 2 to 3 decades with an era effect, and have been longitudinal in nature without directly comparing short‐term outcomes of ECD patients with non‐ECD patients undergoing a FO 7–9 . This study aims to evaluate the outcomes of a large subset of ECD patients who underwent a FO, over a much shorter study period and makes a direct comparison with patients who underwent FO without ECD diagnosis during the same study period and specifically looks at short‐term outcomes of the FO.…”