Atrioventricular Defects Flashcards
AV Canal
AVSD results from?
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
the failure of the endocardial cushions to form and migrate during embryology
resulting in defiency of atria and ventricle septa at the crux
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
ASD
VSD
Mitral and tricuspid valve malformations
What syndrome/Trisomy is often Associated with AVSD?
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
Down Syndrome/ Trisomy 21
HIGHLY associated with AV canal
AVSD is usually Classified into two types, what are they?
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
1.Partial AV canal
2.Complete AV Canal
Incomplete AV Canal
“Incomplete AV canal” is a term often used when there is
* Two separate AV valves
* Primum ASD
* VSD outlet/infundibular VSD
Partial AV canal typically dosent have a VSD
Transitional AV Canal
- ostium primum ASD
- mitral valve cleft
- little to no inlet VSD (VSD gets covered by the chordal tissue)
functions like a partial AVSD
The term unbalanced AVSD refers to
one ventricle can be dominate while the other one is hypoplastic
-complete AV canal due to large VSD
*often associated with hypoplastic LV and AO Outflow obstruction or CoA
With AVSD what type of ASD is present
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
Primum atrial septal defect
(because of the involvement with the crux)
In AVSD the MV is commonly
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
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
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
**ostium primum ASD with cleft MV **
incomplete AV canal has a “Outlet” VSD
-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
What type of VSD is associated with Partial AV canal?
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
Usually no VSD but can have a “outlet” or infundibulum VSD considered an incomplete AV canal at that point.
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
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
What type of VSD is associated with Complete AV canal?
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
Large Inlet VSD located in the posterior portion of the IVS
AVSD may occur in combination with what other congenital defect?
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
Tetrology of Fallot
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
-CoA
-Pulmonary stenosis
-Tetralogy of Fallot
-Down Syndrome (Trisomy 21 is highly associated with AV Canal)
AVSD surgical repair is usually performed within ____ of life
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
usually within 1st year of life
Complete AVSD in 3-6 months
Partial AVSD 1-3 years
With AVSD the papillary muscles are rotated
when in a PSAX view the papillary muscles by 2D are rotated counterclockwise
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
Pulmonary artery banding when pulmonary over circulation is present and timing is elective for surgery.
AVSD surgical repair involves?
Endocardial cushion defect, AV canal defect, atrioventricular septal defect (AVSD), Common AV canal defect
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
AVSD post operative exam should look for?
-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
what is a Gerbode defect
shunt from the LV to the RA. usually result of a AV Canal repair
With the rupture of a sinus of Valsalva aneurysm, which heart chamber, most likely receives the left to right shunt through the aneurysm
Right ventricle;
usually the right coronary sinuses is most commonly involved resulting in the shunt going to the right ventricle
What type of arrhythmia does AVSD repair cause
Surgically created heart block
What is the rastelli classification of AVSDs based on
based on the morphology and attachments of the superior bridging leaflet of the common atrioventricular valve to the septum
Complete AVSD canal
What are the three types of rastelli classification?
Type A:
The superior bridging leaflet is divided and attached to the crest of the interventricular septum.
This is the most common type.
Type B:
The superior bridging leaflet is partly divided and attaches to a papillary muscle on the right side of the ventricular septum. This is the least common type
Type C:
The superior bridging leaflet is undivided or “free-floating” and has no attachments to the ventricular septum
