The Angle of the Components of the Common Atrioventricular Valve Predicts the Outcome of Surgical Correction in Patients With Atrioventricular Septal Defect and Common Atrioventricular Junction

Increased common atrioventricular valve tenting is a risk factor for progression to severe regurgitation in patients with a single ventricle with unbalanced atrioventricular septal defect

“…[9][10][11] Leaflet prolapse and tethering of the mitral valve, tricuspid valve (TV), and left atrioventricular valve post-AVSD repair have been previously identified as important mechanisms of valve failure. [12][13][14][15] In a more recent study, 16 we noted that prolapse as a mechanism of TV regurgitation in HLH is a later phenomenon, with TV tethering being the predominant feature in neonates with HLH who subsequently developed severe tricuspid regurgitation. With this background, we hypothesized that the presence of leaflet tethering in the common atrioventricular valve (AVV) may be an important precursor to subsequent severe regurgitation in patients with an uAVSD.…”

“…Before 1990, overall mortality of uAVSD surgery was high, ranging from 57% to 100%. 21 With improvement in operative and medical strategies over the past 2 decades, 5,6,12 hospital survival of BCPA procedure has improved (91%). 22 However long-term survival remained modest at 50% in a cohort of 35 patients with a median follow-up of 25 months 23 and 66% at 30 months in second cohort of 22 patients.…”

“…Most of the information on effects of leaflet tethering on valve function arises from the adult literature where mitral valve leaflet tethering (ie, increased tenting height, tenting area, and annular to leaflet angle) is an independent risk factor for progressive AVVR after myocardial infarction. 18,19,25,26 In congenital heart disease, Bharucha and colleagues 12 found in a population of patients with complete AVSD that a greater angle of the opening components of the common valve was a marker of moderate to severe AVVR after total correction. This is similar to our finding of greater annular plane to leaflet angle as a risk factor.…”

Congenital heart disease in children

“…[28]. Detailed analysis of the angle of the atrio ventricular valves to the ventricular septum has also been observed to correlate with outcome in terms of valvular function [29]. For unbalanced atrioventricular septal defects, the relative size of the left and right atrioventricular valves will also influence the approach including the feasibility of a biventricular repair (.…”

Three dimensional echocardiography in congenital heart disease

“…Recently, 3DE has given much insight into the understanding of Ebstein's malformation,5 10 leading to better clinical and surgical management (figure 2). Another important contribution of 3D is in the understanding of complex heart defects, especially the atrioventricular septal defects (AVSDs) 11 12. The application of 3D MPR to dissect cardiac anomalies in anatomically appropriate planes has led to biventricular repair of some complex single ventricles 4.…”