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ACHD > AVSD > Flashcards

AVSD Flashcards

1
Q

Other names for AVSD

A
  • Common AV canal defect
  • AVSD
  • AV canal defect
  • AV communis
  • Endocardial cushion defect
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2
Q

Associations of AVSD

A
  • 75% of complete AVSD occurs with trisomy 21
  • Associated with heterotaxy syndrome
  • Can occur with ToF
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3
Q

What role do the superior and inferior endocardial cushions have?

A

Superior and inferior endocardial cushions help from outlet portion of atrium as well as inlet portion of ventricular septum

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4
Q

AVSD embryology

A

Failure of fusion of superior and inferior endocardial cushions

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5
Q

Types of AVSD

A

Complete
Partial
Transitional

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6
Q

What is a Partial AVSD?

A

Characterised by presence of ostium primum ASD and clefts in the anterior leaflets of the left AV valves. Typically 2 distinct AV valves with separate annuli.
Anterior and posterior bridging leaflets are fused centrally creating left and right sided orifices

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7
Q

What is a transitional AVSD?

A

2 distinct AV annuli with defects in the atrial and ventricular septum. Restrictive VSD.

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8
Q

What is a complete AVSD?

A

ASD and unrestrictive VSD leaving a complete, common AV annulus with an inferior and superior bridging leaflet

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9
Q

What is a common AV annulus

A

Guarded by 5 leaflets: Right antero-superior leaflet, Right mural leaflet, superior and inferior bridging leaflet and the left mural leaflet.
Mural = lateral

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10
Q

What classification system is used for complete AVSD?

A

Rastelli Classification
Types A, B and C
Based on morphology of anterior bridging leaflet and degree of bridging and its chordal attachment

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11
Q

Mechanisms of LVOTO in complete AVSD

A

1) Elongate LVOT (‘goose neck’) due to direction of blood flow through CAVV
2) Abnormal chordal attachments to the LV side of septum
3) Discrete sub aortic stenosis
4) Septal hypertrophy
5) Anomalous anterolateral papillary muscles
6)
Aneurysm of membranous septum into LVOT
7) Arch hypoplasia and coarctation

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12
Q

Partial AVSD presentation

A

RV volume overload from L-R shunt at atrial level
Left AV valve regurgitation
AF

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13
Q

Transitional AVSD presentation

A

Features similar to Partial AVSD
May have left AV valve regurgitation
Minimal ventricular level shunting due to restrictive VSD

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14
Q

Complete AVSD presentation

A

Unrestricted PBF dominates picture in childhood
HF and failure to thrive
Pulmonary vasculature disease by age 1

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15
Q

Physical Examination Findings of AVSD

A

Unprepared partial/transitional aVSD: Wide and fixed splitting o S2; PESM; Tricuspid MDM; RV+; PSM; Complete, unprepared AVSD has Eisenmenger’s (Loud P2)

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16
Q

ECG findings of AVSD

A

LAD
1st degree HB/ A Flutter/AF
LAE; LVH
RVH if PHTN or RVOTO

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17
Q

Goals of Echo Exam

A
  • Define primum ASD borders
  • VSD and direction of shunting
  • Morphology and function of the AV valve, looking for AV valve regurgitation or stenosis
  • LVOTO
18
Q

Surgical Options of AVSD

A

1) Classic Single Patch technique

2) Two Patch technique

19
Q

Possible placements of suture line

A

To the right or to the left

20
Q

Suture line to the Right

A

Line stays to right to ventricular septal crest and crosses RBB).
Causes small R-L shunt
Mouth of CS drains into the LA
Wide curve around AVN

21
Q

Suture line to the left

A

Mouth of CS to RA
Stays on left side of conduction tissue and requires shallow bites to not impinge on non-branching bundle, particularly inferior bridging leaflet.

22
Q

Partial AVSD Outcome for adult repair complications

A
  • Atrial arrhythmias
  • CHB
  • Sub-aortic stenosis
  • Recurrent left AV valve regurgitation through the cleft
  • Left AV valve stenosis
23
Q

Indications for reoperation AVSD

A

1) Left AV valve stenosis, regurgitation or prosthesis dysfunction that causes symptoms, arrhythmias or increase in LV dimensions or dysfunction
2) LVOTO (MPG >50 mmHg; PPG > 70 mmHg; MPG<50 with significant left AVVR or AR)
3) Residual or recurrent ASD or VSD that meet criteria for closure

24
Q

Medical therapy for AVSD

A

ACE-i or diuretics for CHF associated with AV valve regurgitation
Anti-arrhythmics or BB for arrhythmias
If prior repair - arrhythmia should prompt investigation of haemodynamics

25
Q

Pregnancy in AVSD

A

Trisomy 21 is commonly seen in association with AVSD, and have 50% risk of transmitting Downs and other genetic defects to offspring
Pre-conception counselling important to ensure no significant haemodynamic lesions present

26
Q

Most common unoperated adult presentation

A

Partial AVSD

27
Q

What causes abnormal activation sequence of the ventricles?

A

AVN is positioned inferoposterior to coronary sinus

BoH and LBB are displaced posteriorly

28
Q

What percentage of congenital cardiac defects do AVSDs account for?

A

3%

29
Q

What % of patients with AVSD have Down’s?

A

35%

30
Q

What percentage of complete AVSDs occur in Down patients?

A

> 75%

31
Q

What percentage of partial AVSDs occur in non-Down patients?

A

> 90%

32
Q

By what age are unprepared partial AVSDs symptomatic?

A

Age 40

33
Q

When can endocardial pacing cause an increased risk of paradoxical emboli?

A

In cases of residual intertribal or inter ventricular communications.
Epicardial pacing may be required

34
Q

How long should patients with AVSD (operated and unoperated) be followed-up?

A

Life-long

35
Q

How often should a surgically repaired AVSD without significant residual abnormalities be followed up?

A

At least every 2-3 years

36
Q

What patients should you avoid cardiac surgery in complete AVSD?

A

Eisenmenger physiology.
If in doubt, PVR testing
Indication for intervention = VSD

37
Q

When should Partial AVSD surgical closure be performed?

A

Significant RV volume overload (ASD guidelines)

38
Q

When should patients undergo valve surgery?

A

1) Symptomatic patients with moderate to severe AV valve regurgitation
2) Asymptomatic patients with moderate or severe left sided valve regurgitation and LVESD > 45 mm and/or impaired LV function (LVEF <60%) when other causes of LV impairment excluded

39
Q

When should patients have surgical repair considered?

A

Asymptomatic patients with moderate or severe left sided AV valve regurgitation who have signs of LV volume overload and a substrate of regurgitation that is amenable for surgical repair

40
Q

Exercise/sports advice for patients with AVSD

A

Uncomplicated, repaired AVSD - no physical activity restriction
Patients with important residual problems require individual recommendations

41
Q

Pregnancy advice for patients with AVSD

A

Well tolerated in patients with complete repair and no significant residual lesions.
Unoperated AVSD –> increased risk of paradoxical embolisation
Closure of any significant ASD should be considered
Severe PAH - pregnancy contraindicated
Residual left AV valve regurgitation with no indication for surgery tolerate pregnancy relatively well, although arrhythmias and worsening of AV valve regurgitation may occur
Recurrence risk of CHD is relatively high and up to 11%. Genetic counselling is necessary

42
Q

IE prophylaxis in patients with AVSD

A

Recommended only for high risk patients

ACHD (8 decks)

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