Valvular Defects- Topic 11 Flashcards Preview

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Flashcards in Valvular Defects- Topic 11 Deck (71)
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1

Absent pulmonary valve

Rare defect
Pulmonary valve tissue not formed or incomplete
4+ PI
Flood pulmonary arteries (pulmonary overcirculation)
Massive dilation of pulmonary arteries- lead to extrinsic compression of the bronchial airway (abnormal development of bronchial tree)
Associated with VSD

2

What is absent pulmonary valve AKA?

TOF w/ absent pulmonary valve

3

What kind of impairment is seen with absent pulmonary valve?

Respiratory impairment
Compression of airway = compromised sats

4

What kind of shunting is seen with absent pulmonary valve?

R to L shunting (systemic desaturation)

5

Absent Pulmonary Valve: Treatment

Plication of the pulmonary arteries
Pulmonary valve replacement
vsd closure

6

What 3 things are associated with absent pulmonary valve?

1. Absent pulmonary valve
2. Dilated pulmonary arteries
3. VSD

7

Pulmonary atresia with intact ventricular septum (PA w/ IVS)

Complete atresia of pulmonary valve; pulmonary valve fails to form late in development
-RV and Tricuspid valve hypoplastic
-PA is normal size
-Large ASD will decompress RA
-Severe hypoplasia of RV results in creation of Coronary Artery Sinusoids

8

Coronary Artery Sinusoids

Think of as shunts
fistula between the RV and coronaries
can be catastrophic

9

PA w/ IVS: Pathophysiology

Pulmonary Blood flow entirely dependent on PDA
-requires PGE-1 infusion after birth
R to L shunting atrially
Coronary perfusion dependent on increased driving forces of obstructed RV (RV increases R is good)
-Decompressing RV = ischemia

10

PA w/ IVS: Treatment

PGE-1 to maintain duct patency
RV dependent Sinusoids; balloon atrial septostomy to decompress the RA
-NO RV dependent Sinusoids- open the atretic pulmonary valve via transcatheter or surgical valvotomy
Systemic to PA shunt or PDA shunt- needs shunt b/c RV is poorly compliant and hypertrophied
Poor RV output

11

PA w/ IVS: Post op Course

Prone to hemodynamic instability
Possibly delay chest closure

12

PA w/ IVS: LOS

1-2 weeks

13

Pulmonary Atresia w/ IVS Associated Problems

1. ASD
2. Atretic Pulmonary Valve
3. PDA
4. Hypoplastic RV
5. Hypoplastic TV

14

Pulmonary Atresia w/ IVS Associated Problems

1. ASD
2. Atretic Pulmonary Valve
3. PDA
4. Hypoplastic RV
5. Hypoplastic TV

15

Pulmonary Atresia w/ VSD AKA

TOF w/ pulmonary atresia (Extreme form of TOF)

16

Pulmonary Atresia w/ VSD

Failure of the development of the pulmonary valve
underdeveloped RV outflow tract and main PA
Branch PAs may be confluent and fed by ductus or discontinuous and hypoplastic
Discontinuous- pulmonary blood flow provided via aortopulmonary collaterals
normal development of the RV
Large VSD
May have ASD (wide variations)

17

Pulmonary Atresia w/ VSD: Pathophysiology

Complete intracardiac mixing- systemic desaturation/cyanosis
Aortopulmonary collaterals
-Progressive stenosis
-Hypoxemia
True pulmonary arteries are hypoplastic
Confluent branch PAs, which are fed by ductus
Hypoplastic branch PAs w/ aortopulmonary vessels

18

Pulmonary Stenosis (PS) is what percent of CHD?

10%

19

Pulmonary Stenosis (PS)

Pulmonary Valve and/or RV outflow tract is restricted
Range from mild to severe
PS causes obstruction to the ejection of blood from the RV (forces increase RV tension development)
-increased work load of the ventricle
-severe and/or prolonged = RVH

20

Pulmonary Stenosis Types

Supravalvular Stenosis
Valvular Stenosis
Subvalvular Stenosis (Infundibular)

21

Supravalvular Stenosis

Pulmonary artery lumen above the pulmonary valve opening is narrowed
Can be main or branch PA

22

Valvular Stenosis

Leaflets of PV thickened/fused at edges
Valve doesn't open fully
May see post-stenotic dilation of the main PA
Valve may be bicuspid

23

Subvalvular Stenosis (Infundibular)

RVOT stenosis, below pulmonary valve
obstructed by muscular tissue

24

Pulmonary Stenosis may be classified by what?

RV pressure
Mild: 45 mmHg or less
Moderate: 46-89 mmHg
Severe: 90 mmHg (suprasystemic) - will develop right heart failure

25

PS in infancy is always __________.

Severe

26

What will happen in pulmonary stenosis if there is an ASD?

Right to left shunting will occur (cyanosis)

27

With moderate pulmonary stenosis (or higher), you will see what?

RVH

28

If PS is purely valvular....

Balloon valvuloplasty
Commisurotomy- incise the fused commisures via direct vision

29

Repair of Infundibular Stenosis

Hypertrophied muscle in the outflow tract is resected

30

Repair of Supravalvular Stenosis

Depends where stenotic lesion is
Remove stenosis/balloon angioplasty or stent
Patch repair/enlargement (eyeball like)