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

Valvular Defects- Topic 11 Flashcards

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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
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)
31
Aortic Stenosis is what percent of all CHD?
10 %
32
What type of lesion is AS?
acyanotic lesion
33
Aortic Stenosis
Narrowing of the aortic valve or thickening of the leaflets, bicuspid or unicuspid valve
34
What is AS associated with?
PDA, MA or coarc
35
What does AS cause?
Increase in pressure/tension within the LV
36
What do pts with AS develop?
LVH - decreased ventricular function - myocardial ischemia
37
Pts with AS are at high risk for what?
Sudden cardiac death
38
Types of AS?
Supravalvular Subvalvular Critical Aortic Stenosis
39
Supravalvular AS
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
Williams Syndrome
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
Williams Syndrome
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
Supravalvular Aortic Stenosis Correction
``` 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
Subaortic Stenosis
Rare in infancy | In infancy usually associated with coarc or interrupted aortic arch
44
Subaortic Stenosis Presents as
1. Fibromuscular Stenosis | 2. Hypertrophic OBstructive Cardiomyopathy
45
Subaortic Stenosis can lead to LVH causing...
Arrhythmias | Sudden death
46
Subaortic Stenosis Correction
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
Konno Procedure
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
Critical Aortic Stenosis
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
Critical Aortic Stenosis: Goal of Correction ***
to relive obstruction of flow of blood through the aortic valve without causing AI
50
Critical Aortic stenosis; Correction
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
CriticalAortic Stenosis: Post op course
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
Critical Aortic Stenosis: LOS
1- 3 weeks
53
Critical Aortic Stenosis: LOS
1- 3 weeks
54
Critical Aortic Stenosis: LOS
1- 3 weeks
55
Aortic Insufficiency
Aortic valve fails to close completely immediately after systole
56
AI Symptoms
LV dilation Decreased CO CHF Exercise intolerance, dyspnea on exertion, dizziness, pulsating headaches, increased pulse pressure, pulmonary congestion, edema
57
Ross Procedure: Aortic Valve Replacement
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
AVR procedure of choice for small children/pediatrics (rough in adults)
ross procedure; can be used on young adults
59
What kind of anticoagulation is required post op for a ross procedure?
No anticoagulation required ***
60
What is key in ensuring suitability in the ross procedure
Patient selection is key
61
What percent of all CHD is Ebstein's Malfomation/Anomaly
0.5% (rare)
62
Ebstein's Malformation/Anomaly
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
Ebstein's Malformation AKA
"Atrialized RV"
64
Ebstein's Malformation AKA
"Atrialized RV"
65
What are the 2 parts of Ebstein's Anomaly
Orientation of the valve divides the RV: 1. PRoximal RV 2. Distal/Functional RV
66
Proximal RV in Ebstein's Malformation
Portion of the RV on the atrial side of the inferior displaced tricuspid valve; thinned, "atrialized"
67
What is also common with Ebstein's Malformation?
PFO/ASD common
68
Ebstein Anomaly- Symptoms
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
Neonatal presentation of Ebstein's Anomaly
Cyanosis due to RV dysfunction; function PV "atresia"
70
What percent of all CHD is tricuspid atresia?
3%
71
Clinical Features of Tricuspid Atresia
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 (17 decks)

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