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Congenital Defects & Pediatrics IA > Valvular Defects- Topic 11 > Flashcards

Valvular Defects- Topic 11 Flashcards

1
Q

Absent pulmonary valve

A

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

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2
Q

What is absent pulmonary valve AKA?

A

TOF w/ absent pulmonary valve

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3
Q

What kind of impairment is seen with absent pulmonary valve?

A

Respiratory impairment

Compression of airway = compromised sats

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4
Q

What kind of shunting is seen with absent pulmonary valve?

A

R to L shunting (systemic desaturation)

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5
Q

Absent Pulmonary Valve: Treatment

A

Plication of the pulmonary arteries
Pulmonary valve replacement
vsd closure

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6
Q

What 3 things are associated with absent pulmonary valve?

A
  1. Absent pulmonary valve
  2. Dilated pulmonary arteries
  3. VSD
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7
Q

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

A

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
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8
Q

Coronary Artery Sinusoids

A

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

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9
Q

PA w/ IVS: Pathophysiology

A

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

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10
Q

PA w/ IVS: Treatment

A

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

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11
Q

PA w/ IVS: Post op Course

A

Prone to hemodynamic instability

Possibly delay chest closure

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12
Q

PA w/ IVS: LOS

A

1-2 weeks

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13
Q

Pulmonary Atresia w/ IVS Associated Problems

A
  1. ASD
  2. Atretic Pulmonary Valve
  3. PDA
  4. Hypoplastic RV
  5. Hypoplastic TV
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14
Q

Pulmonary Atresia w/ IVS Associated Problems

A
  1. ASD
  2. Atretic Pulmonary Valve
  3. PDA
  4. Hypoplastic RV
  5. Hypoplastic TV
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15
Q

Pulmonary Atresia w/ VSD AKA

A

TOF w/ pulmonary atresia (Extreme form of TOF)

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16
Q

Pulmonary Atresia w/ VSD

A

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)

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17
Q

Pulmonary Atresia w/ VSD: Pathophysiology

A

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

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18
Q

Pulmonary Stenosis (PS) is what percent of CHD?

A

10%

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19
Q

Pulmonary Stenosis (PS)

A

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

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20
Q

Pulmonary Stenosis Types

A

Supravalvular Stenosis
Valvular Stenosis
Subvalvular Stenosis (Infundibular)

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21
Q

Supravalvular Stenosis

A

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

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22
Q

Valvular Stenosis

A

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

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23
Q

Subvalvular Stenosis (Infundibular)

A

RVOT stenosis, below pulmonary valve

obstructed by muscular tissue

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24
Q

Pulmonary Stenosis may be classified by what?

A

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

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25
Q

PS in infancy is always __________.

A

Severe

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26
Q

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

A

Right to left shunting will occur (cyanosis)

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27
Q

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

A

RVH

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28
Q

If PS is purely valvular….

A

Balloon valvuloplasty

Commisurotomy- incise the fused commisures via direct vision

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29
Q

Repair of Infundibular Stenosis

A

Hypertrophied muscle in the outflow tract is resected

30
Q

Repair of Supravalvular Stenosis

A

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

31
Q

Aortic Stenosis is what percent of all CHD?

A

10 %

32
Q

What type of lesion is AS?

A

acyanotic lesion

33
Q

Aortic Stenosis

A

Narrowing of the aortic valve or thickening of the leaflets, bicuspid or unicuspid valve

34
Q

What is AS associated with?

A

PDA, MA or coarc

35
Q

What does AS cause?

A

Increase in pressure/tension within the LV

36
Q

What do pts with AS develop?

A

LVH

  • decreased ventricular function
  • myocardial ischemia
37
Q

Pts with AS are at high risk for what?

A

Sudden cardiac death

38
Q

Types of AS?

A

Supravalvular
Subvalvular
Critical Aortic Stenosis

39
Q

Supravalvular AS

A

Constriction of the aorta just above hte valve due to fibrous membrane or hypoplastic aortic arch
Uncommon
-Seen in patients with Williams Syndrome
-Familial form
Can lead to LVH, LV dysfunction, ischemia and risk of sudden death

40
Q

Williams Syndrome

A

rare neurological disorder, distinctive, “elfin” facial appearance, along with a low nasal bridge; an unusually cheerful demeanor and ease with strangers; developmental delay coupled with strong language skills; and cardiovascular problems, such as supravalvular aortic stenosis and transient high blood calcium.

41
Q

Williams Syndrome

A

rare neurological disorder, distinctive, “elfin” facial appearance, along with a low nasal bridge; an unusually cheerful demeanor and ease with strangers; developmental delay coupled with strong language skills; and cardiovascular problems, such as supravalvular aortic stenosis and transient high blood calcium.

42
Q

Supravalvular Aortic Stenosis Correction

A 
Aorta incised into each sinus valsalva
counter incision is made in the aorta above the obstruction
stenoic segment is removed
2 segments are interdigitated
CPB is short to moderate
43
Q

Subaortic Stenosis

A

Rare in infancy

In infancy usually associated with coarc or interrupted aortic arch

44
Q

Subaortic Stenosis Presents as

A
  1. Fibromuscular Stenosis

2. Hypertrophic OBstructive Cardiomyopathy

45
Q

Subaortic Stenosis can lead to LVH causing…

A

Arrhythmias

Sudden death

46
Q

Subaortic Stenosis Correction

A

Done when obstruction is moderate to severe (gradient determines)
Aorta is opened just above the AV
Leaflets are retracted to expose the obstructive tissue below the valve
As much obstructive tissue as possible is excised
Careful to avoid damage to mitral valve, AV conduction system or AV leaflets
CPB is short

47
Q

Konno Procedure

A

aortic valve removed
incision made into ventricular septum (to left of right coronary ostia)
patched open- widens LVOT; allows placement of larger graft/prosthetic valve
Replace aortic root with cryopreserved homograft or pulmonary autograft
-insert into newly opened LV outflow tract

48
Q

Critical Aortic Stenosis

A

Severe form of congenital AS
presents in neonatal period
Symptoms become more acute as the PDA closes
severity depends on the degree of obstruction
Valve may be bicuspid or unicuspid
LV abdormalities can occur
dilation, decresaed function (early surgical intervention required)

49
Q

Critical Aortic Stenosis: Goal of Correction ***

A

to relive obstruction of flow of blood through the aortic valve without causing AI

50
Q

Critical Aortic stenosis; Correction

A

can do percutaneous balloon valvulotomy
Surgery- av visualized and incised at the commissures
Commissurotomy may be hard due to abnormal valve development (shape is a factor)

51
Q

CriticalAortic Stenosis: Post op course

A

depends on the degree of LV dysfunction preoperatively (ECMO-VAD)
depends on the success of the procedure
Will most likely require an aortic valve replacement later in life

52
Q

Critical Aortic Stenosis: LOS

A

1- 3 weeks

53
Q

Critical Aortic Stenosis: LOS

A

1- 3 weeks

54
Q

Critical Aortic Stenosis: LOS

A

1- 3 weeks

55
Q

Aortic Insufficiency

A

Aortic valve fails to close completely immediately after systole

56
Q

AI Symptoms

A

LV dilation
Decreased CO
CHF
Exercise intolerance, dyspnea on exertion, dizziness, pulsating headaches, increased pulse pressure, pulmonary congestion, edema

57
Q

Ross Procedure: Aortic Valve Replacement

A

Use patient’s own pulmonary Valve- move to the aortic position
RVOT is reconstructed with a pulmonary homograft
Coronary arteries are re-implanted on the autograft
Pulmonary autograft grows; the only aortic valve replacement option to do so

58
Q

AVR procedure of choice for small children/pediatrics (rough in adults)

A

ross procedure; can be used on young adults

59
Q

What kind of anticoagulation is required post op for a ross procedure?

A

No anticoagulation required ***

60
Q

What is key in ensuring suitability in the ross procedure

A

Patient selection is key

61
Q

What percent of all CHD is Ebstein’s Malfomation/Anomaly

A

0.5% (rare)

62
Q

Ebstein’s Malformation/Anomaly

A

Leaflets of the tricuspid valve are normally attached to hte firbous annulus; ebsteins patients have downward displacement of hte posterior and septal leaflets of hte tricuspid valve; have an enlarged sail-like anterior leaflet

63
Q

Ebstein’s Malformation AKA

A

“Atrialized RV”

64
Q

Ebstein’s Malformation AKA

A

“Atrialized RV”

65
Q

What are the 2 parts of Ebstein’s Anomaly

A

Orientation of the valve divides the RV:

  1. PRoximal RV
  2. Distal/Functional RV
66
Q

Proximal RV in Ebstein’s Malformation

A

Portion of the RV on the atrial side of the inferior displaced tricuspid valve; thinned, “atrialized”

67
Q

What is also common with Ebstein’s Malformation?

A

PFO/ASD common

68
Q

Ebstein Anomaly- Symptoms

A

Anatomic severity is variable
TV insufficiency
TI possibly combined with stenosis
RV and RA dysfunction- results in cyanosis, RV failure
Wide range of symptoms: dyspnea, cyanosis, clubbing
Arrhythmias are common: cause of sudden death

69
Q

Neonatal presentation of Ebstein’s Anomaly

A

Cyanosis due to RV dysfunction; function PV “atresia”

70
Q

What percent of all CHD is tricuspid atresia?

A

3%

71
Q

Clinical Features of Tricuspid Atresia

A

Mortality rate is high
50% die within 6 months
15-30% survive the first year without surgery
10% live to 10 years without surgery

Congenital Defects & Pediatrics IA (17 decks)

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