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Congenital Defects & Pediatrics IA > AVC, ECD, AVSD- Topic 10 > Flashcards

AVC, ECD, AVSD- Topic 10 Flashcards

1
Q

AVC

A

Atrio-ventricular canal

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2
Q

ECD

A

endocardial cushion defect

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3
Q

AVSD

A

atrio-ventricular septal defects

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4
Q

AVSD/ECD/AVD Defects Definition

A

A deficiency or absence of septal tissue immediately above and/or below the normal plane of A-V Valves. The valves are abnormal in shape and/or function

*Incomplete fusion of the endocardial cushions which form primum atrial septum, A-V valves, and inlet ventricular septum

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5
Q

AVSD/ECD/AVD Defects Definition

A

A deficiency or absence of septal tissue immediately above and/or below the normal plane of A-V Valves. The valves are abnormal in shape and/or function

*Incomplete fusion of the endocardial cushions which form primum atrial septum, A-V valves, and inlet ventricular septum

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6
Q

A-V Canal Incidence of Congenital Heart Disease

A

8 per 1000 live births

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7
Q

AVSD is the _____ most common occurring CHD.

A

5th

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8
Q

AVSD is commonly associated with what conditions?

A

Down’s Syndrome and cardiac malformations such as TOF, DORV, SAS (subaortic stenosis)

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9
Q

AVSD is present in what percent of patients with heterotaxy syndrome?

A

60%

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10
Q

Heterotaxy Syndrome

A

Certain organs forming on the opposite side of the body; flipped

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11
Q

Heterotaxy Syndrome

A

Certain organs forming on the opposite side of the body; flipped

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12
Q

A-V Canal Morbidity/ Mortality

A
  • Children with a complete AV canal fail to thrive in the first few months of life
  • Pts may survive the first few years of life if PVR is high
  • If AV canal is repaired between 4-6 months of life, survival is >80%
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13
Q

Why may patients survive the first few years of life with AV Canal if the PVR is high?

A

High PVR decreases left to right shunting, increases LVEF

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14
Q

ECD

A

consists of defects in lower atrial and upper ventricular septa and deficiencies in the mitral/tricuspid valves

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15
Q

ECD is also called what?

A

AVD, or AVSD

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16
Q

Types of AVSD

A

Complete (CAVSD)
Partial (PAVSD)
Transitional (TAVSD)

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17
Q

How else can AVSD be classified?

A

Balanced

Unbalanced

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18
Q

Balanced AVSD: Ventricle Size

A

Ventricles are equal in size

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19
Q

Balanced AVSD: Size

A

Size is relatively normal

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20
Q

Balanced AVSD: AV Valves

A

Both left and right AV valves may equally share the common AV valve orifice. This arrangement is termed balanced defect

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21
Q

Unbalanced AVSD: Ventricles

A

One of the ventricles may be hypoplastic (not formed completely); size will be different

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22
Q

Unbalanced AVSD: Ventricles

A

One of the ventricles may be hypoplastic (not formed completely); size will be different

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23
Q

When in embryology is the AVSD developed?

A

Day 27-37 Atrial and ventricular septation and development

Day 29: Outflow tract septation

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24
Q

AV septal defects occur at the embryonic age of what? When what happens?

A

34-36 days
Fusion of the endocardial cushions fail
*This occurs when the endocardial cushion fibroblasts fail to migrate normally to form the septum of the AVC

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25
After fusing with the endocardial cushion, if there is a small residual opening at the ECC it is called what?
Ostium primum ASD (AVSD)
26
Embryology of AVSD
* Deficiency of the primum atrial septum, the ventricular septum, the septal leaflet of the tricuspid valve, and the anterior leaflet of the mitral valve occurs * AV valve becomes offset * Anterior leaflet of the AV valve extends across septum * if the leaflet opens preferentially toward a ventricle (limiting flow to the other ventricle), hypoplasia occurs and creates an unbalanced AVSD
27
Complete AVSD
Defect is one in which there are defects in all structures formed by the endocardial cushions. Therefore, there are hole (defects) in the atrial and ventricular septal, and the AV valve remains undivided or "common"
28
Complete AVSD
Defect is one in which there are defects in all structures formed by the endocardial cushions. Therefore, there are hole (defects) in the atrial and ventricular septal, and the AV valve remains undivided or "common"
29
Partial AVSD
A partial atrioventricular septal defect is one in which the part of the ventricular septum formed by the endocardial cushions has filled in (no VSD)
30
What fills in in a partial AVSD?
By tissue from the AV valves or directly from the endocardial cushion tissue causing tricuspid and mitral valves dividing into two distinct valves Note: valvular geometry might be affected
31
Partial AVSD defect is primarily in the __________ and _______.
Atrial septum; mitral valve
32
Partial AVSD is referred to as a _______ defect, and is usually associated with a _____________ that causes the valve to leak (MR).
Ostium primum atrial septal defect | cleft in the mitral valve
33
Partial AVSD Considerations
* Conduction system disruption (AV node displaced inferiorly between coronary sinus and ventricular crest) * Coronary sinus ostium often displaced * Associated anomalies: PDA, persistent LSVC
34
What anomalies are partial AVSD usually associated with?
PDA | persistant LSVC
35
Transitional AVSD
Looks similar to the complete form of atrioventricular septal defect, but the leaflets of the common AV valve are struck to the ventricular septum, thereby effectively dividing the valve into two valves and closing most of the hole between the ventricles (Behaves more like a partial AVSD even though it looks like a complete)
36
Surgical Repair of AVSD
Palliation Complete AVSD Repair *It's all about pulmonary flow
37
Surgical Repair of AVSD: Palliation
``` For excessive pulmonary flow PA Band: -Increases PVR -Decreases pulmonary flow -decreases pulmonary over-circulation ```
38
Palliation for insufficent pulmonary flow (AVSD)
IN patients with inadequate pulmonary flow/hypoxemia, a Blalock-Taussig-Thomas shunt or central shunt will be used
39
Surgical Repair of AVSD: Palliation (overview)
PA Band B-T Shunt Central shunt
40
Complete Repair of AVSD
The treatment of choice for an AVSD
41
Two types of Complete Repair :AVSD
Bi-ventricular Repair | Univentricular REpair
42
Bi-Ventricular Repair (AVSD)
VSD often closed with a synthetic patch (Dacron) ASD closed with pericardial patch Valve repair technique: -Attempt to repair the abnormal valve. This is accomplished by suturing/cutting the cleft (the cut in the valve leaflets) to recreate a two-leaflet mitral valve. -The tricuspid valve may also be repaired
43
Bi-Ventricular Repair (AVSD)
VSD often closed with a synthetic patch (Dacron) ASD closed with pericardial patch Valve repair technique: -Attempt to repair the abnormal valve. This is accomplished by suturing/cutting the cleft (the cut in the valve leaflets) to recreate a two-leaflet mitral valve. -The tricuspid valve may also be repaired
44
Univentricular Repair (AVSD)
The eventual goal of surgical repair is to separate pulmonary and venous outflow, and is usually done with staged procedures, culminating in the Fontan Procedure
45
Univentricular Repair (AVSD): Stage ONe
Blalock Taussig Shunt (BT): usually performed within the first few days after birth, and establishes systemic-to-pulmonary artery shunt between the brachiocephalic artery or the right subclavian artery, to the right pulmonary artery via (usually) a tubed homograft or synthetic graft
46
Univentricular Repair (AVSD): Stage TWo
Bi-Directional Genn PRocedure or Hemi-Fontan: usually performed at 4-6 months after birth as a bridge to Fontan completion. The BT shunt and pulmonary artery band is usually removed. The superior vena cava is then attached to right pulmonary artery, creating a systemic venous to pulmonary connection
47
Univentricular Repair (AVSD): Stage TWo
Bi-Directional Glenn PRocedure or Hemi-Fontan: usually performed at 4-6 months after birth as a bridge to Fontan completion. The BT shunt and pulmonary artery band is usually removed. The superior vena cava is then attached to right pulmonary artery, creating a systemic venous to pulmonary connection
48
Univentricular Repair (AVSD): Stage Two
Bi-Directional Glenn PRocedure or Hemi-Fontan: usually performed at 4-6 months after birth as a bridge to Fontan completion. The BT shunt and pulmonary artery band is usually removed. The superior vena cava is then attached to right pulmonary artery, creating a systemic venous to pulmonary connection
49
Hemi-Fontan Procedure: Bi-directional Cavopulmonary Anastomosis
Anatomosis PA/Right atrial appendage | SVC is patched
50
Univentricular Repairs AVSD: Stage Three
Fontan completion: usually performed 2-3 years of age; the IVC is connceted to the right pulmonary artery vai a tunnel like patch within the right atrium (Lateral Tunnel Fontan), or by creating a conduit for IVC flow outside the right atrium (Extracardiac Fontan)
51
Fontan: Intracardiac
Atrial Baffle | Lateral Tunnel
52
What does the fontan fenetration act as ?
A pop-off valve
53
Why is AV canal a concern?
If not treated, this heart defect can cause lung disease Larger volume of blood than normal must be handled by the right side of the heart causes higher volume than normal and higher pressure than normal in the blood vessels in the lungs
54
Pathophysiology AV Canal
The lungs are able to cope with this extra volume of blood at high pressure for a while Lungs become damaged by this extra volume of blood at high pressure The blood vessels in the lungs get thicker With time, these changes in the lungs become irreversible
55
CPB Circuit Considerations of AVSD
Palliation Stage: Shunts usually done early with small size to prevent damage caused by flow and pressure
56
PA Band
Off CPB
57
BT Shunt
off CPB
58
Central Shunt
both on and off CPB
59
CPB Circuit Considerations of AVSD: Surgical Repair Bi-Ventricular Cannulation
Arterial: Aortic cannulation Venous: Bicaval cannulation LV vent: flexible vent when the herat is open Aortic cross-clamp w/ multiple antegrade CP dosing CPB time is moderate in length
60
CPB Circuit Considerations of AVSD: Surgical Repair Univentricular
Bi-Directional Glenn Shunt - Single atrial cannula (circ arrest- short or off- pump) - Aortic Arterial Fontan: Single atrial cannula (DHCA) Aortic arterial

Congenital Defects & Pediatrics IA (17 decks)

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