Double-orifice left atrioventricular valve in patients with atrioventricular septal defects: Surgical strategies and outcome

Background: The surgical management of double orifice left atrioventricular valve (DOLAVV) in atrioventricular septal defects (AVSD) can be challenging and has important surgical implications. Methods: Between 1961 and 2009, 44 patients with DOLAVV associated with AVSD were reviewed; this constituted 6.7% of cases of AVSD. The median age at operation was 6.6 years (range 2 months-70 years). DOLAVV was associated with partial, intermediate, and complete AVSD in 28 patients (64%), 1 patient (2%), and 15 patients (34%), respectively. Results: Forty-one patients (93%) had partial or complete closure of the zone of apposition (ZOA), and in 3 patients (7%), the ZOA was left open. The accessory orifice was found to be regurgitant in 4 patients, and in all 4 patients it was closed. Four patients had partial annuloplasties. Early mortality consisted of 1 death (2.2%). This was a patient in whom the tissue bridge was divided and severe regurgitation resulted. Median follow-up was 10.3 years (maximum, 36 years). Three adult patients required mitral valve replacement for severe mitral regurgitation (MR) at 3, 11, and 20 years, respectively. Two of these patients experienced progression of MR resulting from an unsutured ZOA. There were 3 late deaths (6%). One death occurred after mitral valve replacement and the others died of noncardiac causes. No patient had hemodynamically significant mitral stenosis. Conclusions: Repair of DOLAVV in AVSD can be performed with low risk, excellent late survival, and freedom from reoperation. The ZOA is typically closed unless the valve area appears small. In the majority of patients, the accessory orifice is competent and can be left alone. Late repeated repair may be difficult because of leaflet dysplasia.