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Peds > 11. Valvular Defects- Exam 3 > Flashcards

11. Valvular Defects- Exam 3 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

Intracardiac Completion Fontan=

A

intra-atrial lateral baffle directs IVC flow to the SVC which is connected to the right PA. a fenestration is made inside a pressure relief

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3
Q

Extracardiac Completion Fontan=

A

conduit outside the RA carrying flow from the IVC to the SVC to the right PA

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4
Q

Absent Pulmonary Valve=

A

Rare defect
Pulmonary valve tissue not formed or incomplete
4+ PI

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5
Q

what does an Absent Pulmonary Valve cause

A

Flood pulmonary arteries (pulmonary overcirculation)

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6
Q

Absent Pulmonary Valves cause flooding of the pulmonary arteries (pulmonary overcirculation). What does this in turn cause

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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7
Q

Absent Pulmonary Valve is associated with what

A

VSD

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8
Q

what type of shunting does an Absent Pulmonary Valve cause

A

Respiratory impairment
R–> L shunting
systemic desaturation
Compression of airway = compromised sats

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9
Q

Absent Pulmonary Valve treatment

A

Plication of the Pulmonary Arteries
Pulmonary Valve Replacement
VSD Closure

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10
Q

Pulmonary Atresia with intact ventricular septum
(PA w/IVS)=
AKA. TOF with Absent Pulmonary Valve

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
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11
Q

with a PA w/IVS, Severe hypoplasia of RV results in creation of what

A

Coronary Artery Sinusoids= Fistula between the RV and coronaries
* Can be catastrophic

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12
Q

describe the flow/shunting for PA w/IVS

A

Pulmonary Blood flow entirely dependent on PDA
Requires PGE-1 infusion after birth
R–> L shunting atrially

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13
Q

with a PA w/IVS, what is Coronary perfusion dependent on

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

PA w/IVS treatment

A
  1. PGE-1 to maintain duct patency
  2. RV dependent Sinusoids= 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

PAw /IVS post-op course

A

Prone to hemodynamic instability

Possibly delay chest closure

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16
Q

PAw /IVS length of stay

A

1-2 weeks

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17
Q

Pulmonary Atresia – with VSD=

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

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.
  • Normal development of the RV
  • Large VSD
  • May have an ASD
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18
Q

Pulmonary Atresia with VSD: branch PA’s may be discontinuous. describe the flow in this case

A

Discontinous – Pulmonary blood flow provided via Aortopulmonary Collaterals

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19
Q

Pulmonary Atresia with VSD: describe the mixing

A

Complete intracardiac mixing

Systemic desaturation/ cyanosis

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20
Q

Pulmonary Atresia with VSD: describe collateral flow

A

Aortopulmonary collaterals
-Porgressive stenosis
-Hypoxemia
“True pulmonary arteries” are hypoplastic

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21
Q

Pulmonary Atresia with VSD: Confluent branch PAs which are fed by ductus require what treatment

A

Complete surgical repair

  • Placement of RV to PA conduit (Rastelli Procedure)
  • Close VSD
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22
Q

Pulmonary Atresia with VSD: Hypoplastic branch PAs with aortopulmonary vessels require what treatment

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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23
Q

Pulmonary Stenosis (PS)=

A
  • Pulmonary Valve and/or RV outflow tract is restricted
  • PS causes obstruction to the ejection of blood from the RV (forces increased RV tension development)
  • Increased work load of the ventricle
  • Severe and/or Prolonged = RV Hypertrophy
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24
Q

what is the incidence rate of Pulmonary Stenosis

A

10% of Congenital Heart Diseases

Range from Mild to Severe

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25
Q

Pulmonary Stenosis (PS): what are the 3 types?

A

Supravalvular Stenosis
Valvular Stenosis
Subvalvular Stenosis (Infundibular)

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26
Q

Supravalvular Stenosis=

A

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

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27
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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28
Q

Subvalvular Stenosis (Infundibular)=

A

RVOT stenosis, below Pulmonary Valve

Obstructed by muscular tissue

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29
Q

Pulmonary Stenosis may be classified by RV Pressure.

Mild=

A

Mild: 45mmHg or less

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30
Q

Pulmonary Stenosis may be classified by RV Pressure.

Moderate=

A

Moderate: 46-89mmHg

–start trying to intervene

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31
Q

Pulmonary Stenosis may be classified by RV Pressure.

Severe=

A

Severe: 90mmHg (suprasystemic)

–Will develop right heart failure

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32
Q

PS in infancy is always _____

A

severe

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33
Q

with PS, If there is an ASD- what will happen

A

Right to left shunting will occur

-Cyanosis

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34
Q

Moderate pulmonary stenosis (or higher), will see ___

A

RVH

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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

Aortic Stenosis=

A

Acyanotic lesion

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

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39
Q

Aortic Stenosis incidence rate

A

10% of all congenital heart diseases

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40
Q

Aortic Stenosis is associated with what

A

PDA, MS, or Coarctaction

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41
Q

Aortic Stenosis causes what

A

Causes increase in pressure/tension within the LV

  • Develop LVH
  • decreased ventricular function
  • myocardial ischemia
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42
Q

Aortic Stenosis has a high risk for what

A

sudden cardiac death

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43
Q

Aortic Stenosis has a high risk for what

A

sudden cardiac death

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44
Q

Aortic Stenosis Types

A

Supravalvular
Subvalvular
Critical Aortic Stenosis

45
Q

AS – SupraValvular Aortic Stenosis=

A

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

46
Q

AS – SupraValvular Aortic Stenosis overall occurrence

A

Uncommon
Seen in patients with Williams Syndrome
Familial form

47
Q

AS – SupraValvular Aortic Stenosis can lead to what

A

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

48
Q

AS – SupraValvular Aortic Stenosis treatment

A
  1. Aorta is incised into each sinus of valsalva
  2. Counter incision is made in the aorta above the obstruction
  3. Stenotic segment is removed
  4. 2 segments are interdigitated
  5. CPB is short to moderate/have to give ostial cpg
49
Q

AS – SubAortic Stenosis= how does it present

A

Presents as:
Fibromuscular stenosis
Hypertrophic Obstructive Cardiomyopathy

50
Q

AS – SubAortic Stenosis: rare in infancy- but if it occurs-what is it associated with

A

In infancy usually associated with Coarctation or interrupted aortic arch

51
Q

AS – SubAortic Stenosis can lead to what

A

Can lead to LVH
Arrhythmias
Sudden death

52
Q

AS – SubAortic Stenosis treatment

A
  1. Done when obstruction is moderate to severe
    (gradient determines)
  2. Aorta is opened just above the AV
  3. Leaflets are retracted to expose the obstructive tissue below the valve
  4. As much obstructive tissue as possible is excised
  5. Careful to avoid damage to mitral valve, AV conduction system, or AV leaflets.
  6. CPB is short
53
Q

Subvalvular obstruction=

A

Aortic valve annular hypoplasia and subvalvular obstruction
Cannot just replace the valve-Must enlarge the annulus
-Konno Procedure (often done with Ross Procedure)

54
Q

Konno Procedure (often done with Ross Procedure)=

A
  1. Aortic Valve removed
  2. Incision made into ventricular septum (to Left of right coronary ostia)
  3. Patched open- Widens LVOT- Allows placement of larger graft/prosthetic valve
  4. Replace aortic root with cryopreserved homograft or pulmonary autograft- Insert into newly opened LV outflow tract
55
Q

AS- Critical Aortic Stenosis=

A

Severe form of congenital AS
Valve may be bicuspid or unicuspid
-LV abnormalities can occur
-Dilation, decreased function

56
Q

AS- Critical Aortic Stenosis: how does it present

A

Presents in neonatal period

  • Symptoms become more acute as the PDA closes
  • Severity depends on degree of obstruction
  • *Early surgical intervention required
57
Q

AS- Critical Aortic Stenosis: goal of correction

A

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

58
Q

AS- Critical Aortic Stenosis: treatment

A
  1. Can do percutaneous balloon valvotomy
  2. Surgery – AV visualized and incised at the commissures
    - Commissurotomy may be hard due to abnormal valve development (shape is a factor)
59
Q

AS- Critical Aortic Stenosis: Post operative 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
60
Q

AS- Critical Aortic Stenosis: length of stay

61
Q

Aortic Insufficiency=

A

Aortic valve fails to close completely immediately after systole

62
Q

Aortic Insufficiency symptoms

A
  • LV dilation
  • Decreased CO
  • CHF
  • Exercise intolerance, Dyspnea on Exertion, Dizziness, Pulsating headaches, increased pulse pressure, pulmonary congestion, edema
63
Q

Aortic Insufficiency symptoms

A
  • LV dilation
  • Decreased CO
  • CHF
  • Exercise intolerance, Dyspnea on Exertion, Dizziness, Pulsating headaches, increased pulse pressure, pulmonary congestion, edema
64
Q

Aortic Surgical Repair

Ross Procedure=

A

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
65
Q

what is unique about the Ross procedure

A

pulmonary autograft grows- its the only aortic valve replacement 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
66
Q

does the Ross procedure require anti-coagulation post-op

67
Q

Ross Procedure: Valves visually inspected to ensure what

A

Ensure suitablity (pt. selection is key)

68
Q

Ross Procedure: after the pulmonary and aortic valves are excised, the coronary arteries are left how?

A

Leave coronary arteries as buttons

69
Q

Ross Procedure is done as a root replacement. describe the steps

A
  • 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
70
Q

with a Ross Procedure- what usually happens later in life

A

Usually required to replace the pulmonary homograft later in life

  • Patient growth
  • Degeneration of graft
71
Q

Ross procedure CPB time?

A

Moderate to long

72
Q

Ebstein’s Malformation/ Anomaly=

A

“atrialized RV”

  • downward displacement of the posterior and septal leaflets of the tricuspid valve.
  • enlarged sail-like anterior leaflet
73
Q

Ebstein’s Malformation/ Anomaly occurrence rate

A

Rare congenital anomaly
0.5% of all Congenital Heart Diseases
Cyanotic Legion

74
Q

Ebstein’s Malformation/ Anomaly: Orientation of the valve divides the RV into 2 parts- describe them

A
  1. Proximal RV= Portion of the RV on the atrial side of inferior displaced tricspid valve- Thinned-“atrialized”
  2. Distal/ Functional RV
75
Q

what other defects are common with an Ebstein’s Malformation/ Anomaly

76
Q

Ebstein’s Malformation/ Anomaly: symptoms

A
  • 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
77
Q

Ebstein’s Malformation/ Anomaly: Neonatal presentation

A

Cyanosis due to RV dysfunction

Functional PV “atresia”

78
Q

Ebstein’s Malformation/ Anomaly: requires what for pulmonary blood flow

A

Requires PDA patency

  • PV does not open 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
79
Q

Ebstein’s Malformation/ Anomaly: goal of surgical correction

A
  • want to create normal functioning tricuspid valve and close the atrial communications.
  • Create complete separation of pulmonary and systemic circulations
  • -2 methods= Post-natal or Prenatal
80
Q

Ebstein’s Malformation/ Anomaly: Postnatal correction

A

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

81
Q

Ebstein’s Malformation/ Anomaly: Neonatal correction

A
  1. Tricuspid valve orifice is closed with a patch-Careful of the conduction pathways
  2. Create unrestricted flow across the ASD, Resect the septum
  3. Plicate the redundant atrialized RV tissue
  4. Divide the PDA- Pulmonary blood flow provided via systemic to PA shunt. Bidirectional Glenn shunt and eventually and Fontan completion
82
Q

Tricuspid Atresia=

A

Cyanotic Lesion

Absence of tricuspid valve

83
Q

Tricuspid Atresia occurrence

A

3% of all Congenital Heart Disease

84
Q

Tricuspid Atresia prevents normal right heart circulation. What does this mean

A
  • Blood returning from the RA must flow through an ASD/ PFO

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

85
Q

Tricuspid Atresia: Mortality rate is high
__% die within 6 months
____% survive the first year without surgery
__% live to 10 years without surgery

A

50%
15-30%
10%

86
Q

Tricuspid Atresia: describe the mixing

A

Severe cyanosis – complete mixing of blood

  • Clubbing
  • Dyspnea
  • Fatigue
  • Right heart failure
87
Q

Tricuspid Atresia: describe the mixing

A

Severe cyanosis – complete mixing of blood

  • Clubbing
  • Dyspnea
  • Fatigue
  • Right heart failure
88
Q

Tricuspid Atresia: treatment

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.
89
Q

Mitral Valve Insufficiency=

A

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

90
Q

Mitral Valve Insufficiency: clinical presentation

A

Palpitations, Fatigue, Orthopnea, Pulmonary Edema

91
Q

Mitral Valve Prolapse=

A

Mitral valve leaflets prolapse into the LA during systole

-MVP associated with Mitral Insufficiency (MR)

92
Q

Mitral Valve Prolapse occurrence

A

Not usually serious

  • Many don’t even know they have it
  • Many live with it asymptomatic for years
93
Q

Mitral Valve Prolapse symptoms

A

SOB, Palpitations, Chest pain.

-Etiology of these unclear

94
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
95
Q

Mitral Valve Stenosis

A
  • Narrowing of the mitral valve
  • Leaflets are abnormally thickened
  • MV annulus may be small
  • Chordae may only be attached to 1 papillary muscle creating a parachute mitral valve
96
Q

Mitral Valve Stenosis occurrence

A

Rare congenital heart disease

Most common valvular defect

97
Q

Mitral Valve Stenosis clinical presentation

A
  • LA dilation
  • Increased LA pressures
  • Increased pulmonary venous, pulmonary arteriolar, pulmonary artery, and RV systolic pressures
  • Leads to pulmonary hypertension, Pulmonary Edema, Right Heart Failure
98
Q

Mitral Valve Stenosis: treatment

A

Pulmonary edema – improved with diuretics

Surgical MV repair or replacement

99
Q

Valvuloplasty=

A
  • Transcatheter pulmonary balloon valvuloplasty. Results equal to open surgical valvotomy
  • Careful determination of anatomy via Transthoracic echo and angiograms
100
Q

Percutaneous Pulmonary Valve Insertion=

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
Example: Melody Valve

101
Q

Percutaneous Pulmonary Valve Insertion is used for patients with what

A

For patients with failed RV to PA conduits (Rastelli)

-Stenosis or regurgitation

102
Q

Transcatheter Aortic Valve Implantation=

A

Bioprosthetic valves sewn within a balloon-expanded or self-expanding stent
-Same valve as their PERIMOUNT Magna

103
Q

Transcatheter Aortic Valve Implantation is used for patients with what

A

For patients with calcific aortic stenosis

104
Q

Transcatheter Aortic Valve Implantation procedure

A
  1. Retrograde transarterial insertion- Requires femoral-iliac arteries to accommodate a 18-24fr delivery system
  2. Direct transapical insertion
  3. Ventricle is paced rapidly to limit cardiac output for device positioning and expansion
105
Q

during Transcatheter Aortic Valve Implantation, if it is positioned too high or low, what happens

A

Paravalvular leaks

Embolization

106
Q

Percutaneous Mitral Valve Repair device

A

MitraClip

  • 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
107
Q

Percutaneous Mitral Valve Repair procedure

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
108
Q

Percutaneous Mitral Valve Repair technique has been used for what other defects

A

MVP, Flail leaflets, annular dilation, mitral regurg secondary to CM

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