Topic 11: Valvular Defects Flashcards
1
Q
Hemi-Fontan Procedure
Bi-directional Cavopulmonary Anastomosis
A
Anastamosis PA/Right atrial appendage
SVC is patched
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
3
Q
Absent Pulmonary Valve associated with what?
A
VSD
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.
5
Q
Absent Pulmonary Valve AKA ?
A
AKA. TOF with Absent Pulmonary Valve
6
Q
Absent Pulmonary Valve treatment? (3)
A
Plication of the Pulmonary Arteries
Pulmonary Valve Replacement
VSD Closure
7
Q
Absent Pulmonary Valve shunting?
A
R -> L shunting
systemic desaturation
8
Q
Absent Pulmonary Valve respiratory involvement ?
A
Respiratory impairment
Compression of airway = compromised sats
9
Q
Absent Pulmonary Valve three aspects?
A
- Absent Pulmonary Valve
- Dilated Pulmonary Arteries
- VSD
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
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
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
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
14
Q
Pulmonary Atresia with Intact Ventricular Septum: treatment? 4
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
Pulmonary Atresia with Intact Ventricular Septum Post operative course:
A
Prone to hemodynamic instability
Possibly delay chest closure
Length of Stay: 1-2 weeks
16
Q
Pulmonary Atresia with Intact Ventricular Septum characteristics? 5
A
- ASD
- Atretic Pulmonary Valve
- PDA
- Hypoplastic RV
- Hypoplastic TV
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
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.
19
Q
Pulmonary Atresia–with VSD
AKA
A
Aka. TOF with Pulmonary Atresia (Extreme form of TOF)
Wide variations
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
21
Q
Pulmonary Atresia–with VSD
VSD - size
A
Large VSD
May have an ASD
Wide variations
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
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
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
25
Pulmonary Stenosis (PS)- % of CHD
10% of Congenital Heart Diseases
26
Pulmonary Stenosis (PS) - is what?
Pulmonary Valve and/or RV outflow tract is
restricted
Range from Mild to Severe
27
Pulmonary Stenosis (PS) causes what?
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
28
Pulmonary Stenosis Type
| Supravalvular Stenosis
Pulmonary artery lumen above the pulmonary valve opening is narrowed
Can be main or branch PA
29
Pulmonary Stenosis Type: 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
30
Pulmonary Stenosis Type: Subvalvular
| Stenosis (Infundibular)
RVOT stenosis, below Pulmonary Valve
| Obstructed by muscular tissue
31
Pulmonary Stenosis - May be classified by RV Pressure- Mild?
45mmHg or less
32
Pulmonary Stenosis - May be classified by RV Pressure - Moderate???
46-89mmHg
| Moderate pulmonary stenosis (or higher), will see RVH
33
Pulmonary Stenosis - May be classified by RV Pressure - Severe??
90mmHg (suprasystemic)
Will develop right heart failure
PS in infancy is always severe
34
Pulmonary Stenosis if there is an ASD what will occur?
Right to left shunting will occur
| Cyanosis
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
Pulmonary Stenosis - types?
A. Supravalvular
B. Valvular Stenosis
C. Subvalvular/Infundibular Stenosis
39
Aortic Stenosis, Types?
Supravalvular
Subvalvular
Critical Aortic Stenosis
40
Aortic Stenosis -- occurrance in CHD?
Congenital AS-10% of all congenital heart diseases
Acyanotic lesion
High risk for sudden cardiac death
41
Aortic Stenosis - what happens?
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
Aortic Stenosis - what do you develop?
Develop LVH
- decreased ventricular function
- myocardial ischemia
- High risk for sudden cardiac death
43
Supravalvular Aortic Stenosis
Constriction of the aorta just above the valve due to fibrous membrane or hypoplastic aortic arch
44
Supravalvular Aortic Stenosis - how often is it seen, and with what population?
Uncommon
Seen in patients with Williams Syndrome
Familial form
45
Supravalvular Aortic Stenosis - can lead to what?
Can lead to LVH, LV dysfunction, ischemia and risk of sudden death
46
Supravalvular Aortic Stenosis - correction?
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
AS–SubAortic Stenosis - presents as ?
```
Rare in infancy
Presents as:
Fibromuscular stenosis
Hypertrophic Obstructive Cardiomyopathy
In infancy usually associated with Coarctation or interrupted aortic arch
```
48
AS–SubAortic Stenosis - can lead to?
Can lead to LVH
Arrhythmias
Sudden death
49
AS–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
50
AS–SubAortic Stenosis - correction determined by?
Done when obstruction is moderate to severe -----gradient determines!!!
51
Aortic valve annular hypoplasia and subvalvular obstruction - correction??
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
AS-Critical Aortic Stenosis presents when?
Severe form of congenital AS
Presents in neonatal period
Symptoms become more acute as the PDA closes
53
AS-Critical Aortic Stenosis - severity depends on what?
| what is required?
```
Severity depends on degree of obstruction
Valve may be bicuspid or unicuspid
LV abnormalities can occur
Dilation, decreased function
Early surgical intervention required
```
54
AS-Critical Aortic Stenosis - goal of correction??
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
AS-Critical Aortic 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
Length of stay: 1-3 weeks
```
56
Aortic Insufficiency - describe
Aortic valve fails to close completely immediately
| after systole
57
Aortic Insufficiency - symptoms?
LV dilation
Decreased CO
CHF
Exercise intolerance, Dyspnea on Exertion, Dizziness,
Pulsating headaches, increased pulse pressure,
pulmonary congestion, edema
58
Aortic Insufficiency - how do you fix it??
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
What is the main great benefit for Aortic Insufficiency aortic valve replacement - pulm autograft?
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
Ross Procedure - Aortic Root replacement -- How??
| what is key?
```
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
Ebstein’s Malformation/ Anomaly - aka
“atrialized RV”
62
Ebstein’s Malformation/ Anomaly - occurance?
Rare congenital anomaly
| 0.5% of all Congenital Heart Diseases
63
Ebstein’s Malformation/ Anomaly - what is it?
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
Ebsteins Malformations/Anomaly --- Orientation of the valve divides the RV into what 2 parts?
```
---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
Ebsteins Malformations/Anomaly - clinical presentation? 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
```
66
Ebsteins Malformations/Anomaly: Neonatal presentation
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
Ebsteins Malformations/Anomaly: Neonatal presentation - what does it require??
PDA patency for pulmonary BF
68
Ebsteins Malformations/Anomaly - how to correct??
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
Postnatal correction of Ebstein’s - Repair
Plicate the atrialized portion of the RV
Reconstruct the Tricuspid valve annulus
Close the ASD
Resect the redundant atrial wall
70
Neonatal correction of Ebstein’s?
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
Tricuspid Atresia
3% of all Congenital Heart Disease
72
Tricuspid Atresia is what??
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
Tricuspid Atresia - what must be present?
VSD or PDA must be present to permit blood flow to pulmonary circulation
74
Tricuspid Atresia - mortality rate?
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
Tricuspid Atresia - symptoms?
```
Severe cyanosis–complete mixing of blood
Clubbing
Dyspnea
Fatigue
Right heart failure
```
76
Tricuspid Atresia - Surgical Correction
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
Mitral Valve Insufficiency - is what? leads to what?
Incomplete closure or absence of the mitral valve
Increased filling of LV
Leads to dilation and hypertrophy
78
Mitral Valve Insufficiency, Clinical Presentation:
Palpitations, Fatigue, Orthopnea, Pulmonary Edema
79
Mitral Valve Prolapse
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
Mitral Valve Prolapse - symptoms? etiology?
```
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
Mitral Valve Prolapse - treatment?
Doesn’t require treatment unless significant mitral insufficiency is present
Usually only surgical with severe Mitral Insufficiency and symptomatic
82
Mitral Valve Stenosis -- congenital occurance?
Rare congenital heart disease
83
Mitral Valve Stenosis -- what happens??
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
Mitral Valve Stenosis - leads to what??
Leads to pulmonary hypertension
Pulmonary Edema
Right Heart Failure
85
Mitral Valve Stenosis - pressure changes?
Increased LA pressures
Increased pulmonary venous, pulmonary arteriolar,
pulmonary artery, and RV systolic pressures
86
Mitral Valve Stenosis - leads to what??
Increased pulmonary venous, pulmonary arteriolar,
pulmonary artery, and RV systolic pressures:
Leads to pulmonary hypertension
Pulmonary Edema
Right Heart Failure
87
Mitral Valve Stenosis treatment?
Pulmonary edema–improved with diuretics
| Surgical MV repair or replacement
88
Valvuloplasty - what do they do??
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
Percutaneous Pulmonary Valve Insertion -- what is it?
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
Percutaneous Pulmonary Valve Insertion - for patients with what?
For patients with failed RV to PA conduits (Rastelli) Stenosis or regurgitation
PALLIATIVE
91
Transcatheter Aortic Valve Implantation - what is it?
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
Transcatheter Aortic Valve Implantation - if valve is positioned too high or too low what happens?
Paravalvular leaks
| Embolization
93
Transcatheter Aortic Valve Implantation - retrograde insertion requires what?
Requires femoral-iliac arteries to accommodate a 18-24fr delivery system
94
Transcatheter Aortic Valve Implantation - used with what patients?
For patients with calcific aortic stenosis
95
Edwards Sapen
Transcatheter Aortic Valve Implantation
Can be inserted transapical or tranfemoral
stent looking
96
Percutaneous Mitral Valve Repair -- 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
it looks like a metal clip they put where the MV regurg is and it clips it shut so no more regurg
97
Percutaneous Mitral Valve Repair - how is it done?
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
