Atrioventricular Septal Defects and Atrioventricular Valve Anomalies

  • Mark K. FriedbergEmail author
  • Norman H. Silverman


Transesophageal echocardiography plays a major role during the surgical intervention of congenital pathologies that affect the atrioventricular junction and atrioventricular valves. The prebypass examination can refine the planned surgical procedure, while the postbypass assessment can detect clinically significant residual findings that otherwise would not have been identified, thereby prompting a return to cardiopulmonary bypass and subsequent reintervention. This chapter focuses on the morphologic features and corresponding echocardiographic findings, as displayed by transesophageal imaging, of the most common congenital anomalies that involve the atrioventricular junction. Particular emphasis is placed on atrioventricular septal defects and malformations affecting the atrioventricular valves.


Transesophageal echocardiography Atrioventricular septal defects Atrioventricular canal defects Endocardial cushion defects Atrioventricular valves Atrioventricular valve anomalies Congenital mitral valve anomalies Congenital tricuspid valve anomalies Ebstein anomaly Prosthetic mitral valve Mitral valve prolapse Infectious endocarditis 

Supplementary material

Video 8.1

Video displays a complete atrioventricular septal defect in the mid esophageal four chamber view. The atrial (short arrow) and ventricular (long arrow) communications are seen. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 1726 kb)

Video 8.2

Deep transgastric view demonstrating a common atrioventricular valve ‘en face’. All the components of the common atrioventricular valve are seen in this view including the superiorbridging leaflet with attachments to the crest of the septum (Rastelli type A) (MOV 545 kb)

Video 8.3

Deep transgastric images at 55° display ‘en face’ view of common atrioventricular valve in a complete atrioventricular septal defect (MOV 3426 kb) (387 kb)
Video 8.4 Transthoracic parasternal short axis imagedemonstrating a cleft in left atrioventricular valve pointing toward the inlet septum in an atrioventricular septal defect (MOV 387 kb)
Video 8.5

Transgastric basal short axis view demonstrating a cleft (arrow) in left atrioventricular valve. LV left ventricle, RV right ventricle (MOV 49255 kb)

Video 8.6

The cleft in the left-sided component of the atrioventricular valve (arrow) is seen in this mid esophageal long axis view in a patient with an atrioventricular septal defect. In this defect the cleft points towards the septum. Ao aorta, LA left atrium, LV left ventricle, RV right ventricle (MOV 915 kb)

Video 8.7

Deep transgastric sagittal view in a child with a complete atrioventricular septal defect and double-orifice of the left component of the atrioventricular valve. In diastole, the common atrioventricular valve orifice is seen ‘en face’ with its left (arrows) and right components. A separate valvar area of double orifice is seen over the left ventricle. LV left ventricle, PA pulmonary artery, RV right ventricle (MOV 1204 kb)

Video 8.8

Mid esophageal four chamber view of a complete atrioventricular septal defect. Color Doppler depicts atrial shunting across two atrial communications (ostium primum and ostium secundum defects). Shunting across a small ventricular septal defect is also seen. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 473 kb)

Video 8.9

A large secundum atrial septal defect is seen (arrow) in association with an atrioventricular septal defect. Color interrogation confirms atrial level left to right shunting. A turbulent color flow signal is identified in the left atrium corresponding to a jet of left atrioventricular valve regurgitation (not fully displayed in the video). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 2103 kb)

Video 8.10

Video displays large ventricular component (arrow) of a complete atrioventricular septal defect. Note the right ventricular wall thickness reflecting the elevated pulmonary artery pressure that characterizes this type of lesion. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 1343 kb)

Video 8.11

Deep transgastric long axis view depicting shunting across the ventricular component of an atrioventricular septal defect. Ao aorta, LV left ventricle, RV right ventricle (MOV 570 kb)

Video 8.12

Mid esophageal four chamber view depicting a ventricular septal defect of the ‘atrioventricular canal-type’. In this defect separate atrioventricular valve orifices were present and the atrial communication was of the secundumtype. Several chordal attachments (arrows) are seen from the atrioventricular valves to the crest of the ventricular septum. Note the lack of normal offsetting of the atrioventricular valves. LA left atrium, LV, left ventricle, RA right atrium, RV right ventricle (MOV 1439 kb)

Video 8.13

The ‘goose neck’ deformity of the left ventricular outflow tract that characterizes atrioventricular septal defects is shown in this deep transgastric sagittal view. Ao aorta, LA left atrium, LV left ventricle (MOV 1366 kb)

Video 8.14

Modified mid esophageal four chamberview with anterior transducer angulation displaying chordal attachments of the left atrioventricular valve across the left ventricular outflow tract onto the interventricular septum (arrow). In some cases this can result in outflow tract obstruction. Ao aorta, LA left atrium, LV left ventricle (MOV 1723 kb)

Video 8.15

The video displays unbalanced atrioventricular septal defects (right and left ventricular dominanttypes) as noted (MOV 3903 kb)

Video 8.16

Mid esophageal four chamber view displays a tricuspid pouch lesion (arrow) in a patient with a partial atrioventricular septal defect. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 942 kb)

Video 8.17

The video displays a complete atrioventricular septal defect in different transesophageal echocardiographic cross-sections. Two-dimensional imaging depicts the anatomical features of the defect. Color flow mapping demonstrates interatrial and interventricular shunting in addition to a significant regurgitant jet across the left component of the common atrioventricular valve. The characteristics of the regurgitant jet are further defined by multiplane imaging, highlighting the importance of color Doppler interrogation in multiple views (MOV 3416 kb)

Video 8.18

Preoperative mid esophageal four chamber view displaying a partial atrioventricular septal defect. The atrial ostium primum defect is well seen and tissue tags are noted on the right ventricular aspect of the septum (no evidence of ventricular level shunting). Color Doppler interrogation demonstrates two jets originating from the left ventricle. One jet is diverted by atrial septal tissue into both atria and a second one is directed from the left ventricle into the right atrium (left ventricular to left atrial shunt) (MOV 1035 kb) (396 kb)
Video 8.19 The image shows preoperative color flow information obtained in a mid esophageal four chamber view using a pediatric biplane transesophageal probe in a small infant with a complete atrioventricular septal defect. Significant regurgitation is demonstrated from both the left and right atrioventricular valvar components, during systole (MOV 396 kb)
Video 8.20

Deep transgastric long axis view in infant with Down syndrome and ‘tet-canal defect’. Note the free-floating superior bridging leaflet of the common atrioventricular valve (Rastelli type C). The characteristic aortic override in tetralogy of Fallot is well seen (MOV 4104 kb)

Video 8.21

Postoperative transesophageal echocardiogram following repair of complete atrioventricular septal defect. Color Doppler interrogation demonstrates two small jets of left atrioventricular valve regurgitation. The jet closest to the interatrial septum represents regurgitation across a residual cleft (MOV 759 kb)

Video 8.22

Mid esophageal long axis view displaying mitral valve stenosis resulting from cleft closure following repair of an atrioventricular septal defect. The left atrium appears dilated. Narrowing of the left ventricular outflow tract is also noted in association with multiple chordal attachments across the subaortic region onto the ventricular septum. Color flow information superimposed on the morphologic data shows a turbulent jet across the left ventricular inflow corresponding to mitral stenosis. The disturbed systolic color flow signal across the left ventricular outflow tract also confirms the subaortic obstruction. A small jet of aortic regurgitation is noted. The coronary sinus appears mildly dilated (MOV 1292 kb)

Video 8.23

Preoperative transesophageal echocardiogram inan infant requiring reoperation to address a residual inlet ventricular septal defect as well as significant regurgitation across a large left atrioventricular valve cleft related to suture dehiscence Both lesions are well characterized by color flow imaging in the mid esophageal four chamber view (MOV 3455 kb)

Video 8.24

Deep transgastric long axis view obtained following repair of an atrioventricular septal defect. The image displays interrogation of the left ventricular outflow tract using a combination of Doppler modalities (color flow mapping and spectral Doppler). No evidence of obstruction was demonstrated. Note the favorable angle for pulsedwave Doppler interrogation of the outflow tract in this view. Ao aorta, LV left ventricle, RV right ventricle (MOV 2884 kb)

Video 8.25

Postbypass transesophageal echocardiogram obtained in the mid esophageal four chamber view demonstrates flow by color Doppler from the left atrium (LA) into the right ventricle (RV; blue jet). This communication, representing a left to right shunt, was inadvertently created during repair of the defect. The echocardiographic findings led to revision of the repair. LV left ventricle, RA right atrium (MOV 2179 kb)

Video 8.26

The initial portion of the video displays a mid esophageal four chamber view obtained immediately after separation from bypass following repair of a complete atrioventricular septal defect. Both atrial and ventricular septal patches are well seen. A contrast study was performed to evaluate a small color Doppler signal suggestive of residual ventricular level shunting (not shown). Agitated saline injected through a femoral venous catheter demonstrated opacification of the right atrium with unexpected almost immediate appearance of contrast in the superior aspect of the left atrium. The atrial septal defect patch was well outlined in the contrast injection and the shunting did not appear to be related to a residual leak at this level. The findings were thought to be consistent with mistaken partial incorporation of inferior vena cava flow into the left atrium (right to left shunt). This was confirmed upon return to bypass and after surgical revision (postbypass #2) the same contrast injection demonstrated an expected normal flow pattern and no residual shunts. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 3058 kb)

Video 8.27

Three-dimensional image obtained by transesophageal echocardiography in an older child displaying ‘en face’ view of a complete atrioventricular septal defect (Video provided by David A. Roberson, M.D.) (MOV 1049 kb)

Video 8.28

Mid esophageal four chamber view displaying an abnormal mitral valve. The mitral valve exhibits grossly abnormal leaflets with reduced diastolic excursion. Small ridges on the leaflets could be construed as a supravalvar mitral ring. Color Doppler imaging demonstrates a turbulent color jet across the mitral valve. The degree of flow velocity acceleration is significant, reaching the left ventricular apex before it turns round, while maintaining its dimensions until distorted by the apical wall. This finding indicates the significant severity of the obstruction. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 2152 kb) (533 kb)
Video 8.29 Video displays a mid esophageal four chamber view in a child with a mitral stenosis. The distorted funnel-shaped mitral valve inflow is seen. A tiny ridge at the posterior annulus is suggestive of a supravalvar mitral ring. The left atrium appears substantially enlarged (MOV 533 kb)
Video 8.30

Transgastric imaging in the mid left ventricular short axis and two chamber views demonstrates a single posteromedial papillary muscle in a child with a parachute mitral valve deformity (MOV 2195 kb)

Video 8.31

Transesophageal echocardiogram depicts a supravalvar mitral ring (arrow). The pathology is well seen in orthogonal views that display the mitral inflow (mid esophageal four chamber and two chamber views). Color Doppler demonstrates flow acceleration at the level of the mitral valve annulus and a small jet of mitral regurgitation. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 4135 kb)

Video 8.32

The images display a supravalvar mitral ring as viewed from the mid esophageal mitral valve commissural view obtained at 60°. The supravalvar ridges (arrows) that characterize this lesion are shown (arrows). Note the dilated left atrium (LA). LV left ventricle (Video provided by Louis I. Bezold, M.D.) (MOV 1706 kb)

Video 8.33

Mid esophageal long axis view recorded from the same infant depicted in Video 8.32 demonstrate a supravalvar ring and reduced diastolic excursion of the mitral valve leaflets. Aliasing of the color flow signal at the mitral inflow confirms the obstruction. Associated lesions included aortic valve stenosis, a subaortic ridge and a perimembranous ventricular septal defect. Ao aorta, LA left atrium, LV left ventricle, RV right ventricle (Video provided by Louis I. Bezold, M.D.) (MOV 1841 kb)

Video 8.34

Mid esophageal four chamber view displaying flow convergence between the left atrium and left ventricle due to a supravalvar mitral ring. Additionally, two jets of mitral inflow in diastole are seen corresponding to two areas of stenosis in this abnormal mitral valve. A regurgitant mitral jet is also evident. A small amount of aortic regurgitation is seen (MOV 2027 kb)

Video 8.35

Transthoracic left ventricular short axis image depicting a double-orifice mitral valve (MOV 7001 kb)

Video 8.36

Mid esophageal two chamber view depicting a double-orifice mitral valve. Two-dimensional imaging shows two distinct valvar orifices during diastole. Color flow appears more prominent across the more posterior valvar opening, which also displays a small jet of regurgitation. No evidence of obstruction is detected across the valve by color Doppler (confirmed by pulsed wave Doppler interrogation in the mid esophageal four chamber view) (MOV 6204 kb)

Video 8.37

Transgastric basal short axis view displays the mitral valve in short axis. The cleft (arrow) is associated with mitral regurgitation as depicted by color Doppler imaging. LV, left ventricle; RV, right ventricle (MOV 4417 kb)

Video 8.38

Multiplane imaging of the mitral valve demonstrating restricted diastolic valvar motion and marked thickening leaflets and support apparatus. The leaflets appear to insert directly into bulky subvalvar structures without definitive chordae, suggesting the presence of a mitral arcade (confirmed at surgery). Color Doppler demonstrates aliased flow across through an eccentric opening and a mitral regurgitant jet (MOV 6534 kb)

Video 8.39

Transesophageal echocardiogram depicting a straddling mitral valve in a child with double outlet right ventricle. The sweep starts at the mid esophageal four chamber view. As the transducer is anteflexed from a posterior position a ventricular septal defect comes into view. At this level, chordal attachments from the mitral valve are seen to straddle the ventricular septum and insert on the right ventricular aspect near the crest of the ventricular septum. The abnormal, side-by-side spatial orientation of the great arteries is depicted. A secundum atrial septal defect is seen. The deep transgastric long axis view demonstrates abnormal mitral valve chordal structures along the pathway from the left ventricle to adjacent great artery (aortic root) (MOV 187567 kb)

Video 8.40

Multiplane examination in the mid esophageal and transgastric views in infant with congenital mitral regurgitation. Two-dimensional imaging demonstrates the dysplastic and thickened valve leaflets with rolled edges that coapt poorly. A broad mitral regurgitant jet is seen coursing along the posterolateral aspect of the left atrium. Severe left atrial dilation is seen with shifting of the interatrial septum in the left to right direction consistent with left atrial hypertension (MOV 214452 kb)

Video 8.41

The characteristic features of Ebstein anomaly are displayed in this mid esophageal four chamber view including: apical septal leaflet displacement, redundancy of the anterior leaflet, and atrialization of the right ventricle. The anterior, ‘sail-like’ leaflet prolapses mildly into the right atrium. Although the left ventricle is not well seen, the right ventricle appears to be the dominant ventricular chamber in this patient (MOV 7350 kb)

Video 8.42

Mid esophageal four chamber view of severe form of Ebstein anomaly displaying adherence of the tricuspid septal leaflet to the underlying myocardium and tethering of chordal structures. The marked redundancy of the anterior leaflet is seen. The valvar coaptation point is displaced well into the apex of the right ventricle. The deep transgastric sagittal view depicts the degree of atrialization of the right ventricle. Note the small size of the ‘true’ or ‘functional’ right ventricle. aRV atrialized right ventricle, LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 1079 kb)

Video 8.43

Mid esophageal four chamber view demonstrating bulging of the interventricular septum towards the left ventricle (septal shift), resulting in narrowing of the left ventricular outflow tract (LVOT) and a “pancaked” appearance of the left ventricle (LV). Ao aorta, LA left atrium, LV left ventricle, RV right ventricle (MOV 859 kb)

Video 8.44

Mid esophageal four chamber view displaying an abnormal tricuspid valve (thickened and dysplastic leaflets) with associated severe regurgitation. The images show an exaggerated degree of normal offset of the atrioventricular valves onto the septum suggesting a more likely diagnosis of Ebstein anomaly over that of tricuspid valvar dysplasia, where normally hinged leaflets are present. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MOV 5206 kb)


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Copyright information

© Springer-Verlag London 2014

Authors and Affiliations

  1. 1.Division of Cardiology, Department of PediatricsThe Labatt Family Heart Center, The Hospital for Sick Children, University of TorontoTorontoCanada
  2. 2.Division of Pediatric Cardiology, Department of PediatricsLucile Packard Children’s Hospital, Stanford University School of MedicinePalo AltoUSA

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