Atrioventricular Defects

Question 1

AVSD results from?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 1

the failure of the endocardial cushions to form and migrate during embryology

resulting in defiency of atria and ventricle septa at the crux

Question 2

AVSD is characterized by the combination of what abnormalities?

what makes a AV canal a AV canal?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 2

ASD
VSD
Mitral and tricuspid valve malformations

Question 3

What syndrome/Trisomy is often Associated with AVSD?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 3

Down Syndrome/ Trisomy 21

HIGHLY associated with AV canal

Question 4

AVSD is usually Classified into two types, what are they?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 4

1.Partial AV canal
2.Complete AV Canal

Question 5

Incomplete AV Canal

Answer 5

“Incomplete AV canal” is a term often used when there is a partial AV Canal (two seperate AV valves) that has a outlet/infundibular VSD

Question 6

Transitional AV Canal

Answer 6

• ostium primum ASD
• mitral valve cleft
• little to no VSD (VSD gets covered by the chordal tissue)

functions like a partial AVSD

Question 7

The term unbalanced AVSD reffers to

Answer 7

one ventricle can be dominate while the other one is hypoplastic

*often associated with hypoplastic LV and AO Outfow obstruction or CoA

Question 8

With AVSD what type of ASD is present

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 8

Primum atrial septal defect
(because of the involvement with the crux)

Can also invole the ostium secundum

Question 9

In AVSD the MV is commonly

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 9

A cleft mitral valve with MR

A cleft or split valve refers to a valve with an extra leaflet resulting in MR
-MR jet will usually be directed towards the crux

Question 10

What characteristic findings on echo present with a partial AV canal?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 10

**ostium primum ASD with cleft MV **

*(incomplete AV canal has a “Outlet” VSD where partial AVSD do not)

-Typically, only the right and left atria are involved (not all 4 chambers)
-usually RV/ RA volume overload (due to ASD)
-LA/LV volume overload depending on the degree of MR
-occasionally a LV to RA shunt
-Elongated LVOT
*very mild cases can persist into adulthood; with little/no MR and small ASD

Question 11

What type of VSD is associated with Partial AV canal?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 11

“outlet” or infundibulum VSD

Question 12

What characteristic findings on echo are present in a Complete AV canal?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 12

Ostium primum ASD with common AV valve (five leaflets), and inlet VSD

-Involves all 4 chambers of the heart and the AV valves
-Mitral and tricuspid valves are merged forming a single multi-leaflet AV valve with posterior and anterior bridging leaflets
-ASD/VSD shunts are mostly L-R but bidirectional shunting is possible and indicates pulmonary hypertension
-Common AV valve regurgitation is common
-Elongated LVOT
-SaO2 levels on the left and right side of the heart maybe equal due to mixing of blood

Question 13

What type of VSD is associated with Complete AV canal?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 13

Large Inlet VSD located in the posterior portion of the IVS

Question 14

AVSD may occure in combination with what other congenital defect?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 14

Tetrology of Fallot

Question 15

What other cardiac abnormalities and syndrome may be associated with AV canal?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 15

-CoA
-Pulmonary stenosis
-Tetralogy of Fallot
-Down Syndrome (Trisomy 21 is highly associated with AV Canal)

Question 16

AVSD surgical repair is usually performed within ____ of life

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 16

usually 1st year of life

Question 17

AVSD the papillary muscles are

Answer 17

when in a PSAX view the papillary muscles by 2D are roatated counterclockwise

Question 18

What palliative procedure is often used to prolong time prior to major surgery in AVSD patients?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 18

Pulmonary artery banding when pulmonary overcirculation is present and timing is elective for surgery.

Question 19

AVSD surgical repair involves?

Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect

Answer 19

Goal is to close all shunts and make two functioning Atrioventricular valves

-1 or 2 synthetic patches are used to close ASD and VSD separating the Left and right atria and ventricles and AV valves
-mitral valve cleft is repaired

Additionally if present: PDA ligation, PA debanding, repair PS, repair CoA

Question 20

AVSD post operative exam should look for?

Answer 20

-Residual shunting
-Atrioventricular valve regurgitation or stenosis
-LVOT obstruction (associated with partial AVSD repairs)

*common to have moderate to severe MR and need additional surgery or valve replacement