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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
Q

After fusing with the endocardial cushion, if there is a small residual opening at the ECC it is called what?

A

Ostium primum ASD (AVSD)

26
Q

Embryology of AVSD

A
  • 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
Q

Complete AVSD

A

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
Q

Complete AVSD

A

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
Q

Partial AVSD

A

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
Q

What fills in in a partial AVSD?

A

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
Q

Partial AVSD defect is primarily in the __________ and _______.

A

Atrial septum; mitral valve

32
Q

Partial AVSD is referred to as a _______ defect, and is usually associated with a _____________ that causes the valve to leak (MR).

A

Ostium primum atrial septal defect

cleft in the mitral valve

33
Q

Partial AVSD Considerations

A
  • Conduction system disruption (AV node displaced inferiorly between coronary sinus and ventricular crest)
  • Coronary sinus ostium often displaced
  • Associated anomalies: PDA, persistent LSVC
34
Q

What anomalies are partial AVSD usually associated with?

A

PDA

persistant LSVC

35
Q

Transitional AVSD

A

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
Q

Surgical Repair of AVSD

A

Palliation
Complete AVSD Repair
*It’s all about pulmonary flow

37
Q

Surgical Repair of AVSD: Palliation

A 
For excessive pulmonary flow
PA Band:
-Increases PVR
-Decreases pulmonary flow
-decreases pulmonary over-circulation
38
Q

Palliation for insufficent pulmonary flow (AVSD)

A

IN patients with inadequate pulmonary flow/hypoxemia, a Blalock-Taussig-Thomas shunt or central shunt will be used

39
Q

Surgical Repair of AVSD: Palliation (overview)

A

PA Band
B-T Shunt
Central shunt

40
Q

Complete Repair of AVSD

A

The treatment of choice for an AVSD

41
Q

Two types of Complete Repair :AVSD

A

Bi-ventricular Repair

Univentricular REpair

42
Q

Bi-Ventricular Repair (AVSD)

A

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
Q

Bi-Ventricular Repair (AVSD)

A

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
Q

Univentricular Repair (AVSD)

A

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
Q

Univentricular Repair (AVSD): Stage ONe

A

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
Q

Univentricular Repair (AVSD): Stage TWo

A

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
Q

Univentricular Repair (AVSD): Stage TWo

A

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
Q

Univentricular Repair (AVSD): Stage Two

A

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
Q

Hemi-Fontan Procedure: Bi-directional Cavopulmonary Anastomosis

A

Anatomosis PA/Right atrial appendage

SVC is patched

50
Q

Univentricular Repairs AVSD: Stage Three

A

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
Q

Fontan: Intracardiac

A

Atrial Baffle

Lateral Tunnel

52
Q

What does the fontan fenetration act as ?

A

A pop-off valve

53
Q

Why is AV canal a concern?

A

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
Q

Pathophysiology AV Canal

A

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
Q

CPB Circuit Considerations of AVSD

A

Palliation Stage: Shunts usually done early with small size to prevent damage caused by flow and pressure

56
Q

PA Band

57
Q

BT Shunt

58
Q

Central Shunt

A

both on and off CPB

59
Q

CPB Circuit Considerations of AVSD: Surgical Repair Bi-Ventricular

Cannulation

A

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
Q

CPB Circuit Considerations of AVSD: Surgical Repair Univentricular

A

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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