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Peds: Congenital Defects (exam 3) > Topic 11: Valvular Defects > Flashcards

Topic 11: Valvular Defects Flashcards

1
Q

Hemi-Fontan Procedure

Bi-directional Cavopulmonary Anastomosis

A

Anastamosis PA/Right atrial appendage

SVC is patched

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2
Q

Absent Pulmonary Valve - what happens?

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 leads to abnormal development of bronchial tree.
Associated with VSD

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3
Q

Absent Pulmonary Valve associated with what?

A

VSD

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4
Q

Absent Pulmonary Valve leads to?

A

Massive dilation of Pulmonary Arteries

Lead to extrinsic compression of the bronchial airway leads to abnormal development of bronchial tree.

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5
Q

Absent Pulmonary Valve AKA ?

A

AKA. TOF with Absent Pulmonary Valve

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6
Q

Absent Pulmonary Valve treatment? (3)

A

Plication of the Pulmonary Arteries
Pulmonary Valve Replacement
VSD Closure

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7
Q

Absent Pulmonary Valve shunting?

A

R -> L shunting

systemic desaturation

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8
Q

Absent Pulmonary Valve respiratory involvement ?

A

Respiratory impairment

Compression of airway = compromised sats

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9
Q

Absent Pulmonary Valve three aspects?

A
  1. Absent Pulmonary Valve
  2. Dilated Pulmonary Arteries
  3. VSD
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10
Q

Pulmonary Atresia with intact ventricular septum (PA w/IVS)
what fails to form?
valves involed how?

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*
Fistula between the RV and coronaries 
* Can be catastrophic
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11
Q

Pulmonary Atresia with intact ventricular septum (PA w/IVS) what is created by the severe hypoplasia of RV?

A

Severe hypoplasia of RV results in creation of Coronary Artery Sinusoids*
Fistula between the RV and coronaries
* Can be catastrophic

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12
Q

Pulmonary Atresia with Intact Ventricular Septum: Pathophysiology
Pulm BF? shunting?

A

Pulmonary Blood flow entirely dependent on PDA
Requires PGE-1 infusion after birth
R-> L shunting atrially
Coronary perfusion dependent on increased driving forces of obstructed RV (RV increased resistance is good)
Decompressing RV = Ischemia

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13
Q

Pulmonary Atresia with Intact Ventricular Septum: Pathophysiology
Coronary perfusion dependent on what?

A

Coronary perfusion dependent on increased driving forces of obstructed RV (RV increased resistance is good)
Decompressing RV = Ischemia

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14
Q

Pulmonary Atresia with Intact Ventricular Septum: treatment? 4

A
  1. PGE-1 to maintain duct patency
    RV dependent Sinusoids
  2. Balloon atrial septostomy to decompress the RA
  3. NO RV dependent Sinusoids
    Open the atretic Pulmonary valve via transcatheter or surgical valvotomy
  4. Systemic to PA shunt or PDA stent
    Need shunt b/c RV is poorly compliant and hypertrophied
    Poor RV output
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15
Q

Pulmonary Atresia with Intact Ventricular Septum Post operative course:

A

Prone to hemodynamic instability
Possibly delay chest closure
Length of Stay: 1-2 weeks

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16
Q

Pulmonary Atresia with Intact Ventricular Septum characteristics? 5

A
  1. ASD
  2. Atretic Pulmonary Valve
  3. PDA
  4. Hypoplastic RV
  5. Hypoplastic TV
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17
Q

Pulmonary Atresia–with VSD

AKA

A

Aka. TOF with Pulmonary Atresia (Extreme form of TOF)
Discontinous–Pulmonary blood flow provided via Aortopulmonary Collaterals
Normal development of the RV
Large VSD
May have an ASD
Wide variations

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18
Q

Pulmonary Atresia–with 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.

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19
Q

Pulmonary Atresia–with VSD

AKA

A

Aka. TOF with Pulmonary Atresia (Extreme form of TOF)

Wide variations

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20
Q

Pulmonary Atresia–with VSD -

how is pulm BF affected?

A

Discontinous–Pulmonary blood flow provided via Aortopulmonary Collaterals
Normal development of the RV

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21
Q

Pulmonary Atresia–with VSD

VSD - size

A

Large VSD
May have an ASD
Wide variations

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22
Q

Pulmonary Atresia–w VSD Pathophysiology 3

A 
Complete intracardiac mixing
      -Systemic desaturation/ cyanosis
Aortopulmonary collaterals
     -Porgressive stenosis
     -Hypoxemia
“True pulmonary arteries” are hypoplastic
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23
Q

Pulmonary Atresia–With VSD

Confluent branch PAs which are fed by ductus what is done?

A

Complete surgical repair
Placement of RV to PA conduit (Rastelli Procedure)
Close VSD

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24
Q

Pulmonary Atresia–With VSD

Hypoplastic branch PAs with aortopulmonary vessels what is done?

A

-Surgical approach is varied and patient specific
-Unifocalization of Aortopulmonary (A-P) collaterals
-RVOT reconstruction
Staged or do it all together and incorporate AP collateral unifocalization into the RVOT conduit
-Eventual closure of the VSD after RVOT
reconstruction/unifocalization
Ensure pulmonary flow adequate

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25
Q

Pulmonary Stenosis (PS)- % of CHD

A

10% of Congenital Heart Diseases

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26
Q

Pulmonary Stenosis (PS) - is what?

A

Pulmonary Valve and/or RV outflow tract is
restricted
Range from Mild to Severe

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27
Q

Pulmonary Stenosis (PS) causes what?

A

PS causes obstruction to the ejection of blood from the RV (forces RV tension development)
Increased work load of the ventricle
Severe and/or Prolonged = Right Ventricular Hypertrophy

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28
Q

Pulmonary Stenosis Type

Supravalvular Stenosis

A

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

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29
Q

Pulmonary Stenosis Type: 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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30
Q

Pulmonary Stenosis Type: Subvalvular

Stenosis (Infundibular)

A

RVOT stenosis, below Pulmonary Valve

Obstructed by muscular tissue

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31
Q

Pulmonary Stenosis - May be classified by RV Pressure- Mild?

A

45mmHg or less

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32
Q

Pulmonary Stenosis - May be classified by RV Pressure - Moderate???

A

46-89mmHg

Moderate pulmonary stenosis (or higher), will see RVH

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33
Q

Pulmonary Stenosis - May be classified by RV Pressure - Severe??

A

90mmHg (suprasystemic)
Will develop right heart failure
PS in infancy is always severe

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34
Q

Pulmonary Stenosis if there is an ASD what will occur?

A

Right to left shunting will occur

Cyanosis

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35
Q

Repair of Pulmonary Stenosis - If the defect is purely valvular?

A

Balloon valvuloplasty

Commisurotomy-incise the fused commisures via direct vision

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36
Q

Repair of Pulmonary Stenosis, Infundibular Stensosis?

A

Hypertrophied muscle in the outflow tract is resected

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37
Q

Repair of Pulmonary Stenosis, Supravalvular Stenosis??

A

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

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38
Q

Pulmonary Stenosis - types?

A

A. Supravalvular
B. Valvular Stenosis
C. Subvalvular/Infundibular Stenosis

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39
Q

Aortic Stenosis, Types?

A

Supravalvular
Subvalvular
Critical Aortic Stenosis

40
Q

Aortic Stenosis – occurrance in CHD?

A

Congenital AS-10% of all congenital heart diseases
Acyanotic lesion
High risk for sudden cardiac death

41
Q

Aortic Stenosis - what happens?

A

Narrowing of the aortic valve or thickening of the leaflets, bicuspid or unicuspid valve
Associated with PDA, MS, or Coarctaction
Causes increase in pressure/tension within the LV

42
Q

Aortic Stenosis - what do you develop?

A

Develop LVH

  • decreased ventricular function
  • myocardial ischemia
  • High risk for sudden cardiac death
43
Q

Supravalvular Aortic Stenosis

A

Constriction of the aorta just above the valve due to fibrous membrane or hypoplastic aortic arch

44
Q

Supravalvular Aortic Stenosis - how often is it seen, and with what population?

A

Uncommon
Seen in patients with Williams Syndrome
Familial form

45
Q

Supravalvular Aortic Stenosis - can lead to what?

A

Can lead to LVH, LV dysfunction, ischemia and risk of sudden death

46
Q

Supravalvular Aortic Stenosis - correction?

A

Aorta is incised into each sinus of valsalva
Counter incision is made in the aorta above the obstruction
Stenotic segment is removed
2 segments are interdigitated
CPB is short to moderate

47
Q

AS–SubAortic Stenosis - presents as ?

A 
Rare in infancy
Presents as:
Fibromuscular stenosis
Hypertrophic Obstructive Cardiomyopathy
In infancy usually associated with Coarctation or interrupted aortic arch
48
Q

AS–SubAortic Stenosis - can lead to?

A

Can lead to LVH
Arrhythmias
Sudden death

49
Q

AS–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

50
Q

AS–SubAortic Stenosis - correction determined by?

A

Done when obstruction is moderate to severe —–gradient determines!!!

51
Q

Aortic valve annular hypoplasia and subvalvular obstruction - correction??

A

Cannot just replace the valve
Must enlarge the annulus
Konno Procedure (often done with Ross 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

52
Q

AS-Critical Aortic Stenosis presents when?

A

Severe form of congenital AS
Presents in neonatal period
Symptoms become more acute as the PDA closes

53
Q

AS-Critical Aortic Stenosis - severity depends on what?

what is required?

A 
Severity depends on degree of obstruction
Valve may be bicuspid or unicuspid
LV abnormalities can occur
Dilation, decreased function
Early surgical intervention required
54
Q

AS-Critical Aortic Stenosis - goal of correction??

A

Goal of correction–to relieve obstruction of flow of blood through the aortic valve without causing AI
Can do percutaneous balloon valvotomy
Surgery–AV visualized and incised at the commissures
Commissurotomy may be hard due to abnormal valve development (shape is a factor)

55
Q

AS-Critical Aortic 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
Length of stay: 1-3 weeks
56
Q

Aortic Insufficiency - describe

A

Aortic valve fails to close completely immediately

after systole

57
Q

Aortic Insufficiency - symptoms?

A

LV dilation
Decreased CO
CHF
Exercise intolerance, Dyspnea on Exertion, Dizziness,
Pulsating headaches, increased pulse pressure,
pulmonary congestion, edema

58
Q

Aortic Insufficiency - how do you fix it??

A

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

59
Q

What is the main great benefit for Aortic Insufficiency aortic valve replacement - pulm autograft?

A

Follow up studies show the pulmonary autograft grows !!!!!!!!
THE ONLY AORTIC VALVE REPLACEMENT OPTION TO DO SO
Makes this the AVR procedure of choice for small
children/ pediatrics (rough in adults)
Starting to become popular in young adult population
as well.
No anticoagulation required post op

60
Q

Ross Procedure - Aortic Root replacement – How??

what is key?

A 
Valves visually inspected
Ensure suitablity (pt. selection is key)
Pulmonary Valve excised
Aortic valve excised
Leave coronary arteries as buttons
Done as root replacement
Proximal pulmonary autograft put in position of native aortic root
Coronaries implanted
Distal end connected to aorta
Cryopreserved Valved
Homograft inserted into original pulmonary root position
BUT
More extensive procedure/ operation that just an AVR
Usually required to replace the pulmonary homograft
later in life
Patient growth
Degeneration of graft
CPB time–Moderate to long
61
Q

Ebstein’s Malformation/ Anomaly - aka

A

“atrialized RV”

62
Q

Ebstein’s Malformation/ Anomaly - occurance?

A

Rare congenital anomaly

0.5% of all Congenital Heart Diseases

63
Q

Ebstein’s Malformation/ Anomaly - what is it?

A

Cyanotic Legion
Leaflets of the tricuspid valve are normally attached
to the fibrous annulus
Ebstein’s patients have a downward displacement of the posterior and septal leaflets of the tricuspid valve.
Have an enlarged sail-like anterior leaflet

64
Q

Ebsteins Malformations/Anomaly — Orientation of the valve divides the RV into what 2 parts?

A 
---Proximal RV
Portion of the RV on the atrial side of inferior displaced
tricspid valve
Thinned 
“atrialized”
---Distal/ Functional RV
PFO/ ASD is common
65
Q

Ebsteins Malformations/Anomaly - clinical presentation? 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
66
Q

Ebsteins Malformations/Anomaly: Neonatal presentation

A

Cyanosis due to RV dysfunction
Functional PV “atresia”
Requires PDA patency for pulmonary blood flow
PV does not open (normal formation) due to inability of RV to generate pressure in excess of PA pressure
Venous return to the heart goes thru an ASD/PFO to
the LA.

67
Q

Ebsteins Malformations/Anomaly: Neonatal presentation - what does it require??

A

PDA patency for pulmonary BF

68
Q

Ebsteins Malformations/Anomaly - how to correct??

A

Surgery is indicated with symptoms
Repair:
Ideally–want to create normal functioning tricuspid valve and close the atrial communications.
Ie. Create complete separation of pulmonary and systemic circulations
2 methods –Post-natal or Prenatal

69
Q

Postnatal correction of Ebstein’s - Repair

A

Plicate the atrialized portion of the RV
Reconstruct the Tricuspid valve annulus
Close the ASD
Resect the redundant atrial wall

70
Q

Neonatal correction of Ebstein’s?

A

Tricuspid valve orifice is closed with a patch
Careful of the conduction pathways
Create unrestricted flow across the ASD
Resect the septum
Plicate the redundant atrialized RV tissue
Divide the PDA
Pulmonary blood flow provided via systemic to PA shunt
Bidirectional Glenn shunt and eventually and Fontan completion

71
Q

Tricuspid Atresia

A

3% of all Congenital Heart Disease

72
Q

Tricuspid Atresia is what??

A

Cyanotic Lesion
Absence of tricuspid valve
Prevents normal right heart circulation
Blood returning from the RA must flow through an ASD/PFO
VSD or PDA must be present to permit blood flow to pulmonary circulation

73
Q

Tricuspid Atresia - what must be present?

A

VSD or PDA must be present to permit blood flow to pulmonary circulation

74
Q

Tricuspid Atresia - mortality rate?

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

75
Q

Tricuspid Atresia - symptoms?

A 
Severe cyanosis–complete mixing of blood
Clubbing
Dyspnea
Fatigue
Right heart failure
76
Q

Tricuspid Atresia - Surgical Correction

A

Limited to increasing pulmonary blood flow
Use one of the systemic to PA shunts or Rashkind procedure
** Cannot do valve replacement because the RV is
under developed

77
Q

Mitral Valve Insufficiency - is what? leads to what?

A

Incomplete closure or absence of the mitral valve
Increased filling of LV
Leads to dilation and hypertrophy

78
Q

Mitral Valve Insufficiency, Clinical Presentation:

A

Palpitations, Fatigue, Orthopnea, Pulmonary Edema

79
Q

Mitral Valve Prolapse

A

Mitral valve leaflets prolapse into the LA during systole
MVP associated with Mitral Insufficiency (MR)

Kinda like the MV valve is toooo tight of an opening

80
Q

Mitral Valve Prolapse - symptoms? etiology?

A 
Not usually serious
Many don’t even know they have it
Many live with it asymptomatic for years
Symptoms include: SOB, Palpitations, Chest pain.
Etiology of these unclear
81
Q

Mitral Valve Prolapse - treatment?

A

Doesn’t require treatment unless significant mitral insufficiency is present
Usually only surgical with severe Mitral Insufficiency and symptomatic

82
Q

Mitral Valve Stenosis – congenital occurance?

A

Rare congenital heart disease

83
Q

Mitral Valve Stenosis – what happens??

A

Narrowing of the mitral valve
Most common valvular defect
Leaflets are abnormally thickened
MV annulus may be small
Chordae may only be attached to 1 papillary muscle creating a parachute mitral valve.
LA dilation
Increased LA pressures
Increased pulmonary venous, pulmonary arteriolar,
pulmonary artery, and RV systolic pressures

84
Q

Mitral Valve Stenosis - leads to what??

A

Leads to pulmonary hypertension
Pulmonary Edema
Right Heart Failure

85
Q

Mitral Valve Stenosis - pressure changes?

A

Increased LA pressures
Increased pulmonary venous, pulmonary arteriolar,
pulmonary artery, and RV systolic pressures

86
Q

Mitral Valve Stenosis - leads to what??

A

Increased pulmonary venous, pulmonary arteriolar,
pulmonary artery, and RV systolic pressures:
Leads to pulmonary hypertension
Pulmonary Edema
Right Heart Failure

87
Q

Mitral Valve Stenosis treatment?

A

Pulmonary edema–improved with diuretics

Surgical MV repair or replacement

88
Q

Valvuloplasty - what do they do??

A

Transcatheter pulmonary balloon valvuloplasty
Results equal to open surgical valvotomy
Careful determination of anatomy via Transthoracic echo and angiograms.

its like they stick a balloon through the stenosed valve and open the balloon to force it open to make a better space – hopefully it sticks

89
Q

Percutaneous Pulmonary Valve Insertion – what is it?

A

Transcatheter-delivered valve that has been mounted within a balloon-expandable stent

Palliative procedure!!!
Extends life to RV to PA conduit
High long term failure rate of valves in the pulmonary
position
Melody Valve (fake stent valve they put in)

(For patients with failed RV to PA conduits (Rastelli) Stenosis or regurgitation)

90
Q

Percutaneous Pulmonary Valve Insertion - for patients with what?

A

For patients with failed RV to PA conduits (Rastelli) Stenosis or regurgitation
PALLIATIVE

91
Q

Transcatheter Aortic Valve Implantation - what is it?

A

Bioprosthetic valves sewn w/in a balloon-expanded or self-expanding stent (Same valve as their PERIMOUNT Magna)
Retrograde transarterial insertion (Requires femoral-iliac arteries to accommodate a 18-24fr delivery system)
Direct transapical insertion
Ventricle is paced rapidly to limit CO for device
positioning and expansion
Position too high or too low -Paravalvular leaks, Embolization

(calcific aortic stenosis)

92
Q

Transcatheter Aortic Valve Implantation - if valve is positioned too high or too low what happens?

A

Paravalvular leaks

Embolization

93
Q

Transcatheter Aortic Valve Implantation - retrograde insertion requires what?

A

Requires femoral-iliac arteries to accommodate a 18-24fr delivery system

94
Q

Transcatheter Aortic Valve Implantation - used with what patients?

A

For patients with calcific aortic stenosis

95
Q

Edwards Sapen

A

Transcatheter Aortic Valve Implantation
Can be inserted transapical or tranfemoral
stent looking

96
Q

Percutaneous Mitral Valve Repair – MitraClip

A

Only device to complete enrollment in randomized clinical trials
Designed to perform edge to edge repair of the mitral valve.
Other devices attempt to create an annuloplasty
Have not reached randomized trial phase yet
Technical issues

it looks like a metal clip they put where the MV regurg is and it clips it shut so no more regurg

97
Q

Percutaneous Mitral Valve Repair - how is it done?

A

Delivered by a transvenous, transseptal approach
Guided by TEE
Implanted on the valve
Grabs middle portions of the anterior and posterior
mitral leaflets
Creating edge to edge repair
Has been used on MVP, Flail leaflets, annular dilation, mitral regurg secondary to CM

it looks like a metal clip they put where the MV regurg is and it clips it shut so no more regurg

Peds: Congenital Defects (exam 3) (4 decks)

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