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Peds > 10. AVSD/ECD/AVC Defects- Exam 3 > Flashcards

10. AVSD/ECD/AVC Defects- Exam 3 Flashcards

1
Q

Definition: AVSD/ECD/AVC Defects

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

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2
Q

Incomplete fusion of the endocardial cushions

which form what 3 things

A

primum atrial septum
A-V valves
inlet ventricular septum

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3
Q

Incidence of congenital heart disease is approximately what

A

8 per 1000 live births.

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4
Q

AVSD is the ____th most common occurring CHD

A

5th

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5
Q

AVSD is commonly associated with _______ and cardiac malformations such as (3)

A
  • Down’s syndrome

- Tetralogy of Fallot (TOF), Double outlet right ventricle (DORV), and sub-aortic stenosis (SAS)

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6
Q

AVSD is present in __% of patients with heterotaxy syndrome.

A

60%

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7
Q

heterotaxy=

A

Certain organs forming on the opposite side of the body

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8
Q

Children with a complete AV canal fail to thrive in the first few months of life. Patients may survive the first few years of life if the ___ is high. Why?

A

PVR

High PVR decreases left to right shunting , increases LVEF

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9
Q

If AV canal is repaired between 4-6 months of life, survival is over ___%

A

> 80%.

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10
Q

An endocardial cushion defect (ECD) consists of what?

A

defects in lower atrial and upper ventricular septa, and deficiencies in the mitral/tricuspid valves.
Also called AVC, or AVSD

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11
Q

Atrioventricular septal defects can be classified into one of what three categories

A
  • Complete (CAVSD)
  • Partial (PAVSD)
  • Transitional (TAVSD)
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12
Q

Further Classification of AVSD include

A

Balanced or Unbalanced

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13
Q

Balanced AVSD=

A

Ventricles are equal in size
•Size is relatively normal
•Both left and right AV valves may equally share the common AV valve orifice. This arrangement is termed a balanced defect.

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14
Q

Unbalanced AVSD=

A

One of the ventricles may be hypoplastic

•Size will be different

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15
Q

Atrial and Ventricular septation and development occur during what days

A

day 27-37

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16
Q

Outflow tract septation occus on what day

A

day 29

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17
Q

AV septal defects occur at the embryonic age of ___ days when fusion of the _______ fails.

A

34-36

endocardial cushions

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18
Q

fusion of the endocardial cushions fails when what happens

A

occurs when the endocardial cushion fibroblasts fail to migrate normally to form the septum of the AVC

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19
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).

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20
Q

What is the end result of AVSD embryology development

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 valves becomes offset
  • Anterior leaflet of the AV valve extends across septum
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21
Q

during the development of the AVSD, what happens if the leaflet opens preferentially toward a ventricle?

A

(limiting flow to the other ventricle), hypoplasia occurs and creates an unbalanced AVSD

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22
Q

Complete AVSD=

A

there are defects in all structures formed by the endocardial cushions.
• Therefore, there are defects (holes) in the atrial and ventricular septal, and the AV valve remains undivided or “common”.

23
Q

describe the mixing of a complete AVSD

A

All chambers mix

-so sats in all 4 chambers will be equal

24
Q

Partial AVSD=

A

the part of the ventricular septum formed by the endocardial cushions has filled in: (no VSD) !!!

25
Q

with a partial AVSD, how does the ventricular septum formed by the endocardial cushions has filled in

A

Fills in by tissue from the AV valves or
directly from the endocardial cushion tissue causing
•tricuspid and mitral valves dividing into two distinct valves (may or may not be functional)

26
Q

with a partial AVSD- what might be affected

A

Valvular geometry

27
Q

partial AVSDs are primarily where

A

primarily in the atrial septum and mitral valve

28
Q

partial AVSDs located in the atrial septum and mitral valve are referred to as what? what is it associated with?

A

referred to as an ostium primum atrial septal defect, and is usually associated with a cleft in the mitral valve that causes the valve to leak (mitral regurgitation-MR) !!!!

29
Q

what 2 things are often displaced with partial AVSDs

A
  • Conduction system disruption (A-V node displaced inferiorly between coronary sinus and ventricular crest)
  • Coronary sinus ostium often displaced
30
Q

what are 2 Associated anomalies with partial AVSDs

A

PDA

persistent LSVC [may have to use 3 cannulas]

31
Q

Transitional AVSDs look similar to the complete form of atrioventricular septal defect, but what the difference

A

the leaflets of the common AV valve are stuck to the ventricular septum, thereby effectively dividing the valve into two valves and closing most of the hole between the ventricles.

32
Q

how does a transitional AVSD behave

A

behaves more like a partial atrioventricular septal defect, even thought it looks more like a complete atrioventricular septal defect.

33
Q

how are AVSDs repaired

A

Palliation
Complete AVSD Repair
•It’s all about pulmonary blood flow!!

34
Q

Palliation for EXCESSIVE pulmonary flow:

A

PA Band=
•Increases PVR
•Decreases Pulmonary Flow
•Decreases Pulmonary Over-circulation

35
Q

Palliation for INSUFFICIENT pulmonary flow:

A

Blalock-Taussig-Thomas shunt or central shunt will be used.

36
Q

The treatment of choice for an AVSD is what strategy?

A

complete surgical repair. [2 types]

  • Bi-ventricular Repair
  • Univentricular Repair
37
Q

Bi-ventricular Repair: what are the VSD and ASD closed with?

A

The VSD is often closed with a synthetic patch (Dacron).

The ASD is often closed with a pericardial patch

38
Q

Bi-ventricular Repair: technique

A
  • 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
39
Q

Univentricular Repair goal

A

separate pulmonary and venous outflow, and is usually done with staged procedures, culminating in the Fontan Procedure

40
Q

Univentricular Repairs AVSD: stage 1=

Blalock-Taussig (BT) shunt

A
  1. usually performed within the first few days after birth
  2. establishes a systemic-to-pulmonary artery shunt btwn the brachiocephalic artery or the right subclavian artery, to the right PA via (usually) a tubed homograft or synthetic graft.
41
Q

Univentricular Repairs AVSD: stage 2=

Bi-Directional Glenn Procedure or Hemi-Fontan

A
  1. usually performed at 4-6 months after birth as a bridge to Fontan completion.
  2. The BT shunt and PA band is usually removed. The SVC is then attached to right PA, creating a systemic venous-to-pulmonary connection
    - -Hemi-Fontan Procedure= Anastamosis PA/Right atrial appendage then SVC is patched
42
Q

Univentricular Repairs AVSD: stage 3=

Fontan Completion

A
  1. Usually performed at 2-3 years of age
  2. the IVC is connected to the right PA via a tunnel like patch within the RA (Lateral Tunnel Fontan), or by creating a conduit for IVC flow outside the RA (Extracardiac Fontan).
43
Q

what is the purpose of the Fontan Fenestration

A

acts as a pop-off valve

44
Q

Why is atrioventricular 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 which causes higher volume than normal and higher pressure than normal in the blood vessels in the lungs

45
Q

if AVSDs are left untreated and lung disease occurs, The lungs are able to cope with this extra volume of blood at high pressure for a while…but what ultimately happens

A

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

46
Q

CPB Circuit Considerations of AVSD

•Palliation stage

A

Shunts: Usually done early with small size to prevent damage caused by flow and pressure

47
Q

CPB Circuit Considerations of AVSD:

May/may not utilize CPB (standby)

A

PA Band: off CPB
BT shunt: off CPB
Central Shunt: both on and off CPB

48
Q

CPB Circuit Considerations of AVSD

•Surgical Repair: Bi-ventricular- Venous+Arterial cannulas

A

Arterial: Aortic cannulation
Venous: Bicaval cannulation

49
Q

CPB Circuit Considerations of AVSD

•Surgical Repair: Bi-ventricular: venting

A

LV Vent: Flexible vent when the heart is open

50
Q

CPB Circuit Considerations of AVSD

•Surgical Repair: Bi-ventricular: cardioplegia

A

Aotic Cross-Clamp w/ multiple antegrade CP dosing

51
Q

CPB Circuit Considerations of AVSD

•Surgical Repair: Bi-ventricular: length of procedure

A

CPB time is moderate in length

52
Q

CPB Circuit Considerations of AVSD
Surgical Repair: Univentricular
•Bi-Directional Glenn Shunt: Venous+Arterial Cannula

A

Venous= Single Atrial Cannula
Arterial: Aortic cannulation
•(Circulatory arrest – short or off-pump)

53
Q

CPB Circuit Considerations of AVSD
Surgical Repair: Univentricular
•Fontan: Venous+Arterial Cannula

A

Venous= Single Atrial Cannula
Arterial: Aortic cannulation
DHCA

Peds (17 decks)

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