11. Valvular Defects- Exam 3 Flashcards
1
Q
Hemi-Fontan Procedure
(Bi-directional Cavopulmonary Anastomosis)=
A
Anastamosis PA/Right atrial appendage
SVC is patched
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
3
Q
Extracardiac Completion Fontan=
A
conduit outside the RA carrying flow from the IVC to the SVC to the right PA
4
Q
Absent Pulmonary Valve=
A
Rare defect
Pulmonary valve tissue not formed or incomplete
4+ PI
5
Q
what does an Absent Pulmonary Valve cause
A
Flood pulmonary arteries (pulmonary overcirculation)
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
7
Q
Absent Pulmonary Valve is associated with what
A
VSD
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
9
Q
Absent Pulmonary Valve treatment
A
Plication of the Pulmonary Arteries
Pulmonary Valve Replacement
VSD Closure
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
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
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
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
14
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 stent= Need shunt b/c RV is poorly compliant and hypertrophied. Poor RV output
15
Q
PAw /IVS post-op course
A
Prone to hemodynamic instability
Possibly delay chest closure
16
Q
PAw /IVS length of stay
A
1-2 weeks
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
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
19
Q
Pulmonary Atresia with VSD: describe the mixing
A
Complete intracardiac mixing
Systemic desaturation/ cyanosis
20
Q
Pulmonary Atresia with VSD: describe collateral flow
A
Aortopulmonary collaterals
-Porgressive stenosis
-Hypoxemia
“True pulmonary arteries” are hypoplastic
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
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
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
24
Q
what is the incidence rate of Pulmonary Stenosis
A
10% of Congenital Heart Diseases
Range from Mild to Severe
25
Pulmonary Stenosis (PS): what are the 3 types?
Supravalvular Stenosis
Valvular Stenosis
Subvalvular Stenosis (Infundibular)
26
Supravalvular Stenosis=
Pulmonary artery lumen above the pulmonary valve opening is narrowed
Can be main or branch PA
27
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
28
Subvalvular Stenosis (Infundibular)=
RVOT stenosis, below Pulmonary Valve
| Obstructed by muscular tissue
29
Pulmonary Stenosis may be classified by RV Pressure.
| Mild=
Mild: 45mmHg or less
30
Pulmonary Stenosis may be classified by RV Pressure.
| Moderate=
Moderate: 46-89mmHg
| --start trying to intervene
31
Pulmonary Stenosis may be classified by RV Pressure.
| Severe=
Severe: 90mmHg (suprasystemic)
| --Will develop right heart failure
32
PS in infancy is always _____
severe
33
with PS, If there is an ASD- what will happen
Right to left shunting will occur
| -Cyanosis
34
Moderate pulmonary stenosis (or higher), will see ___
RVH
35
Repair of Pulmonary Stenosis
| If the defect is purely valvular:
Balloon valvuloplasty
| Commisurotomy - incise the fused commisures via direct vision
36
Repair of Pulmonary Stenosis
| Infundibular Stensosis:
Hypertrophied muscle in the outflow tract is resected
37
Repair of Pulmonary Stenosis
| Supravalvular Stenosis:
Depends where stenotic lesion is
Remove stenosis/ balloon angioplasty or stent
Patch repair/ enlargement (eyeball like)
38
Aortic Stenosis=
Acyanotic lesion
| Narrowing of the aortic valve or thickening of the leaflets, bicuspid or unicuspid valve
39
Aortic Stenosis incidence rate
10% of all congenital heart diseases
40
Aortic Stenosis is associated with what
PDA, MS, or Coarctaction
41
Aortic Stenosis causes what
Causes increase in pressure/tension within the LV
- Develop LVH
- decreased ventricular function
- myocardial ischemia
42
Aortic Stenosis has a high risk for what
sudden cardiac death
43
Aortic Stenosis has a high risk for what
sudden cardiac death
44
Aortic Stenosis Types
Supravalvular
Subvalvular
Critical Aortic Stenosis
45
AS – SupraValvular Aortic Stenosis=
Constriction of the aorta just above the valve due to fibrous membrane or hypoplastic aortic arch
46
AS – SupraValvular Aortic Stenosis overall occurrence
Uncommon
Seen in patients with Williams Syndrome
Familial form
47
AS – SupraValvular Aortic Stenosis can lead to what
Can lead to LVH, LV dysfunction, ischemia and risk of sudden death
48
AS – SupraValvular Aortic Stenosis treatment
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
AS – SubAortic Stenosis= how does it present
Presents as:
Fibromuscular stenosis
Hypertrophic Obstructive Cardiomyopathy
50
AS – SubAortic Stenosis: rare in infancy- but if it occurs-what is it associated with
In infancy usually associated with Coarctation or interrupted aortic arch
51
AS – SubAortic Stenosis can lead to what
Can lead to LVH
Arrhythmias
Sudden death
52
AS – SubAortic Stenosis treatment
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
Subvalvular obstruction=
Aortic valve annular hypoplasia and subvalvular obstruction
Cannot just replace the valve-Must enlarge the annulus
-Konno Procedure (often done with Ross Procedure)
54
Konno Procedure (often done with Ross Procedure)=
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
AS- Critical Aortic Stenosis=
Severe form of congenital AS
Valve may be bicuspid or unicuspid
-LV abnormalities can occur
-Dilation, decreased function
56
AS- Critical Aortic Stenosis: how does it present
Presents in neonatal period
- Symptoms become more acute as the PDA closes
- Severity depends on degree of obstruction
* *Early surgical intervention required
57
AS- Critical Aortic Stenosis: goal of correction
to relieve obstruction of flow of blood through the aortic valve without causing AI
58
AS- Critical Aortic Stenosis: treatment
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
AS- Critical Aortic Stenosis: Post operative 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
60
AS- Critical Aortic Stenosis: length of stay
1-3 weeks
61
Aortic Insufficiency=
Aortic valve fails to close completely immediately after systole
62
Aortic Insufficiency symptoms
- LV dilation
- Decreased CO
- CHF
- Exercise intolerance, Dyspnea on Exertion, Dizziness, Pulsating headaches, increased pulse pressure, pulmonary congestion, edema
63
Aortic Insufficiency symptoms
- LV dilation
- Decreased CO
- CHF
- Exercise intolerance, Dyspnea on Exertion, Dizziness, Pulsating headaches, increased pulse pressure, pulmonary congestion, edema
64
Aortic Surgical Repair
| 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
65
what is unique about the Ross procedure
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
does the Ross procedure require anti-coagulation post-op
nope
67
Ross Procedure: Valves visually inspected to ensure what
Ensure suitablity (pt. selection is key)
68
Ross Procedure: after the pulmonary and aortic valves are excised, the coronary arteries are left how?
Leave coronary arteries as buttons
69
Ross Procedure is done as a root replacement. describe the steps
- 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
with a Ross Procedure- what usually happens later in life
Usually required to replace the pulmonary homograft later in life
- Patient growth
- Degeneration of graft
71
Ross procedure CPB time?
Moderate to long
72
Ebstein’s Malformation/ Anomaly=
“atrialized RV”
- downward displacement of the posterior and septal leaflets of the tricuspid valve.
- enlarged sail-like anterior leaflet
73
Ebstein’s Malformation/ Anomaly occurrence rate
Rare congenital anomaly
0.5% of all Congenital Heart Diseases
Cyanotic Legion
74
Ebstein’s Malformation/ Anomaly: Orientation of the valve divides the RV into 2 parts- describe them
1. Proximal RV= Portion of the RV on the atrial side of inferior displaced tricspid valve- Thinned-“atrialized”
2. Distal/ Functional RV
75
what other defects are common with an Ebstein’s Malformation/ Anomaly
PFO/ ASD
76
Ebstein’s Malformation/ Anomaly: symptoms
- 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
Ebstein’s Malformation/ Anomaly: Neonatal presentation
Cyanosis due to RV dysfunction
| Functional PV “atresia”
78
Ebstein’s Malformation/ Anomaly: requires what for pulmonary blood flow
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
Ebstein’s Malformation/ Anomaly: goal of surgical correction
- 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
Ebstein’s Malformation/ Anomaly: Postnatal correction
Plicate the atrialized portion of the RV
Reconstruct the Tricuspid valve annulus
Close the ASD
Resect the redundant atrial wall
81
Ebstein’s Malformation/ Anomaly: Neonatal correction
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
Tricuspid Atresia=
Cyanotic Lesion
| Absence of tricuspid valve
83
Tricuspid Atresia occurrence
3% of all Congenital Heart Disease
84
Tricuspid Atresia prevents normal right heart circulation. What does this mean
- 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
Tricuspid Atresia: Mortality rate is high
__% die within 6 months
____% survive the first year without surgery
__% live to 10 years without surgery
50%
15-30%
10%
86
Tricuspid Atresia: describe the mixing
Severe cyanosis – complete mixing of blood
- Clubbing
- Dyspnea
- Fatigue
- Right heart failure
87
Tricuspid Atresia: describe the mixing
Severe cyanosis – complete mixing of blood
- Clubbing
- Dyspnea
- Fatigue
- Right heart failure
88
Tricuspid Atresia: treatment
- 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
Mitral Valve Insufficiency=
Incomplete closure or absence of the mitral valve
Increased filling of LV
-Leads to dilation and hypertrophy
90
Mitral Valve Insufficiency: clinical presentation
Palpitations, Fatigue, Orthopnea, Pulmonary Edema
91
Mitral Valve Prolapse=
Mitral valve leaflets prolapse into the LA during systole
| -MVP associated with Mitral Insufficiency (MR)
92
Mitral Valve Prolapse occurrence
Not usually serious
- Many don’t even know they have it
- Many live with it asymptomatic for years
93
Mitral Valve Prolapse symptoms
SOB, Palpitations, Chest pain.
| -Etiology of these unclear
94
Mitral Valve Prolapse treatment
- Doesn’t require treatment unless significant mitral insufficiency is present
- Usually only surgical with severe Mitral Insufficiency and symptomatic
95
Mitral Valve Stenosis
- 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
Mitral Valve Stenosis occurrence
Rare congenital heart disease
| Most common valvular defect
97
Mitral Valve Stenosis clinical presentation
- 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
Mitral Valve Stenosis: treatment
Pulmonary edema – improved with diuretics
| Surgical MV repair or replacement
99
Valvuloplasty=
- Transcatheter pulmonary balloon valvuloplasty. Results equal to open surgical valvotomy
- Careful determination of anatomy via Transthoracic echo and angiograms
100
Percutaneous Pulmonary Valve Insertion=
-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
Percutaneous Pulmonary Valve Insertion is used for patients with what
For patients with failed RV to PA conduits (Rastelli)
| -Stenosis or regurgitation
102
Transcatheter Aortic Valve Implantation=
Bioprosthetic valves sewn within a balloon-expanded or self-expanding stent
-Same valve as their PERIMOUNT Magna
103
Transcatheter Aortic Valve Implantation is used for patients with what
For patients with calcific aortic stenosis
104
Transcatheter Aortic Valve Implantation procedure
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
during Transcatheter Aortic Valve Implantation, if it is positioned too high or low, what happens
Paravalvular leaks
| Embolization
106
Percutaneous Mitral Valve Repair device
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
Percutaneous Mitral Valve Repair procedure
- 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
Percutaneous Mitral Valve Repair technique has been used for what other defects
MVP, Flail leaflets, annular dilation, mitral regurg secondary to CM
