VALVULAR DEFECTS Flashcards
(93 cards)
1
Q
complete AVC Palliation
A
Shunt (Qp/Qs) Too much flow Not enough flow
2
Q
Complete AVC Repair Staged repair
A
HFP/BDG
3
Q
Complete AVC Repair Final stage
A
Fontan
4
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 leads to abnormal development of bronchial tree.
Associated with VSD
5
Q
Absent Pulmonary Valve
AKA.
A
TOF with Absent Pulmonary Valve
6
Q
Absent Pulmonary Valve Respiratory impairment
A
R L shunting systemic desaturation
Compression of airway = compromised sats
7
Q
Absent Pulmonary Valve Treatment:
A
Plication of the Pulmonary Arteries Pulmonary Valve Replacement VSD Closure
8
Q
Pulmonary Atresia with intact ventricular septum (PA w/IVS)
A
Complete atresia of pulmonary valve Pulmonary valve fails to form late in development. PA is normal size
9
Q
Pulmonary Atresia with intact ventricular septum (PA w/IVS) RV and Tricuspid Valve
A
hypoplastic. Severe hypoplasia of RV results in creation of Coronary Artery Sinusoids*
Fistula between the RV and coronaries * Can be catastrophic
10
Q
Pulmonary Atresia with intact ventricular septum (PA w/IVS) ASD
A
Large ASD will decompress RA
11
Q
Pulmonary Atresia with Intact Ventricular Septum
Pathophysiology
A
Pulmonary Blood flow entirely dependent on PDA
Requires PGE-1 infusion after birth
12
Q
Pulmonary Atresia with Intact Ventricular Septum shunting
A
R L shunting atrially
13
Q
Pulmonary Atresia with Intact Ventricular Septum coronary perfusion
A
ependent on increased driving forces of obstructed RV (RV increased resistance is good)
Decompressing RV = Ischemia
14
Q
Pulmonary Atresia with Intact Ventricular Septum Treatment:
A
PGE-1 to maintain duct patency
RV dependent Sinusoids Balloon atrial septostomy to decompress the RA
15
Q
Pulmonary Atresia with Intact Ventricular Septum Treatment: NO RV dependent Sinusoids
A
Open the atretic Pulmonary valve via transcatheter or
surgical valvotomy
16
Q
Pulmonary Atresia with Intact Ventricular Septum Systemic to PA shunt or PDA stent
A
Need shunt b/c RV is poorly compliant and hypertrophied Poor RV output
17
Q
Pulmonary Atresia with intact ventricular septum
Post operative course: and LOS
A
Prone to hemodynamic instability Possibly delay chest closure
Length of Stay: 1-2 weeks
18
Q
Pulmonary Atresia
with Intact
Ventricular Septum CHARCTERISTICS
A
- ASD 2. Atretic Pulmonary Valve 3. PDA 4. Hypoplastic RV 5. Hypoplastic TV
19
Q
Pulmonary Atresia – with VSD
Aka.
A
TOF with Pulmonary Atresia (Extreme form of TOF)
20
Q
Pulmonary Atresia – with VSD is
A
Failure of the development of the pulmonary valve
Underdeveloped RV outflow tract and main PA
21
Q
Pulmonary Atresia – with VSD branch PA may be
A
confluent and fed by ductus or discontinuous and hypoplastic.
Discontinous – Pulmonary blood flow provided via Aortopulmonary Collaterals
22
Q
Pulmonary Atresia – with VSD RV
A
Normal development of the RV
23
Q
Pulmonary Atresia – with VSD (VSD + ASD)
A
arge VSD May have an ASD
Wide variations
24
Q
Pulmonary Atresia – With VSD
PathophysiologY
A
Complete intracardiac mixing
Systemic desaturation/ cyanosis
25
Pulmonary Atresia – With VSD aortopulmonary collaterals
Porgressive stenosis Hypoxemia
| “True pulmonary arteries” are hypoplastic
26
Pulmonary Atresia – With VSD repair
| Confluent branch PAs which are
fed by ductus. Complete surgical repair Placement of RV to PA conduit (Rastelli Procedure) Close VSD
27
Pulmonary Atresia – With VSD repair Hypoplastic branch PAs with aortopulmonary vessels
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
28
Pulmonary Stenosis (PS) prevalence
10% of Congenital Heart Diseases
29
Pulmonary Stenosis (PS) what is it?
Pulmonary Valve and/or RV outflow tract is
restricted
Range from Mild to Severe
30
Pulmonary Stenosis (PS) causes
bstruction 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
31
Pulmonary Stenosis Types
| Supravalvular Stenosis
Pulmonary artery lumen above the pulmonary valve opening is
narrowed Can be main or branch PA
32
Pulmonary Stenosis Types 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
33
Pulmonary Stenosis Types subvalvular stenosis
RVOT stenosis, below Pulmonary Valve Obstructed by muscular tissue
Pulmonary Stenosis
34
Pulmonary Stenosis
| May be classified by RV Pressure
Mild: 45mmHg or less
Moderate: 46-89mmHg
Severe: 90mmHg (suprasystemic) Will develop right heart failure
35
PS in infancy is always
severe
36
(PS ) If there is an ASD –
Right to left shunting will occur
| Cyanosis
37
Moderate pulmonary stenosis (or higher), will see
RVH
38
Repair of Pulmonary Stenosis
| If the defect is purely valvular:
Balloon valvuloplasty Commisurotomy - incise the fused commisures via direct vision
39
Repair of Pulmonary Stenosis Infundibular Stensosis:
Hypertrophied muscle in the outflow tract is resected
40
Repair of Pulmonary Stenosis Supravalvular Stenosis:
Depends where stenotic lesion is Remove stenosis/ balloon angioplasty or stent Patch repair/ enlargement (eyeball like)
41
Aortic Stenosis prevalence
Congenital AS -10% of all congenital heart diseases
| Acyanotic lesion
42
Aortic Stenosis what is it
Narrowing of the aortic valve or thickening of the leaflets, bicuspid or unicuspid valve
43
Aortic Stenosis what its associated and what it causes
Associated with PDA, MS, or Coarctaction
Causes increase in pressure/tension within the LV
Develop LVH decreased ventricular function myocardial ischemia
High risk for sudden cardiac death
44
Supravalvular Aortic Stenosis prevalence what it is and when its seen
Constriction of the aorta just above the 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
45
AS – 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
46
SubAortic Stenosis prevalence how it presents and what it causes
Rare in infancy
Presents as: Fibromuscular stenosis Hypertrophic Obstructive Cardiomyopathy
In infancy usually associated with Coarctation or interrupted aortic arch
Can lead to LVH Arrhythmias Sudden death
47
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
48
Subvalvular obstruction correction
Aortic valve annular hypoplasia and subvalvular obstruction
Cannot just replace the valve
Must enlarge the annulus
49
Subvalvular obstruction 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.
50
Critical Aortic Stenosis
Severe form of congenital AS
Presents in neonatal period
Symptoms become more acute as the PDA closes Severity depends on degree of obstruction
Valve may be bicuspid or unicuspid LV abnormalities can occur
Dilation, decreased function Early surgical intervention required
51
AS- Critical Aortic Stenosis
| 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)
52
AS- Critical Aortic Stenosis
| Post operative course AND LOS
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
53
Aortic Insufficiency
Aortic valve fails to close completely immediately after systole
54
AI symptoms
LV dilation Decreased CO CHF
Exercise intolerance, Dyspnea on Exertion, Dizziness, Pulsating headaches, increased pulse pressure, pulmonary congestion, edema
55
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
56
why is ross procedure of choice in kids
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
57
Ross Procedure 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.
58
Aortic Surgical Repair Ross Procedure
| 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
59
Ebstein’s Malformation/ Anomaly what it is pathophysiology prevalence
“atrialized RV”
Rare congenital anomaly 0.5% of all Congenital Heart Diseases 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
60
Ebstein’s Anomaly Orientation of the valve divides the RV into 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
61
Ebstein’s 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
62
Ebstein’s Anomaly – In Neonate
| Neonatal presentation
Cyanosis due to RV dysfunction
| Functional PV “atresia”
63
Ebstein’s Anomaly – In Neonate requires
PDA patency for pulmonary blood flow
64
Ebstein’s Anomaly – In Neonate PV does not open due to
(normal formation) due to inability of RV to generate pressure in excess of PA pressure
65
Ebstein’s Anomaly – In Neonate venous return
Venous return to the heart goes thru an ASD/PFO to the LA.
66
Ebstein’s Malformation/ Anomaly
| 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 Prenatal
67
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.
68
Neonatal correction of Ebstein’s
| Repair described by Starns, et al. (CHLA)
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
69
Tricuspid Atresia prevalence what it is what it causes
3% of all Congenital Heart Disease
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
70
Tricuspid Atresia
Clinical Features:
Mortality rate is high
50% die within 6 months 15-30% survive the first year without surgery 10% live to 10 years without surgery
71
Tricuspid Atresia Severe cyanosis – complete mixing of blood causes
Clubbing Dyspnea Fatigue
| Right heart failure
72
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.
73
Mitral Valve Insufficiency
Incomplete closure or absence of the mitral valve Increased filling of LV
Leads to dilation and hypertrophy
74
Mitral Valve Insufficiency Clinical Presentation:
Palpitations, Fatigue, Orthopnea, Pulmonary Edema
75
Mitral Valve Prolapse
Mitral valve leaflets prolapse into the LA during systole MVP associated with Mitral Insufficiency (MR). Not usually serious Manydon’tevenknowtheyhaveit Manylivewithitasymptomaticforyears
76
Mitral Valve Prolapse symptoms
SOB, Palpitations, Chest pain. Etiologyoftheseunclear
77
Mitral Valve Prolapse treatment
Doesn’trequiretreatmentunlesssignificantmitralinsufficiencyispresent UsuallyonlysurgicalwithsevereMitralInsufficiencyandsymptomatic.
78
Mitral Valve Stenosis
```
Rare congenital heart disease
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.
```
79
Mitral Valve Stenosis effects on LA and RV
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
80
Mitral Valve Stenosis
| Treatment
Pulmonary edema – improved with diuretics Surgical MV repair or replacement
81
Valvuloplasty
Transcatheter pulmonary balloon valvuloplasty Results equal to open surgical valvotomy
Careful determination of anatomy via Transthoracic echo and angiograms.
82
Percutaneous Pulmonary Valve Insertion
| For patients with
failed RV to PA conduits (Rastelli) Stenosis or regurgitation
83
Percutaneous Pulmonary Valve Insertion Transcatheter-
-delivered valve that has been mounted within a balloon-expandable stent
84
Percutaneous Pulmonary Valve Insertion WHAT IS IT
Palliative procedure
Extends life to RV to PA conduit
High long term failure rate of valves in the pulmonary position
Melody Valve
85
Transcatheter Aortic Valve Implantation
| For patients witH
calcific aortic stenosis
86
Transcatheter Aortic Valve Implantation Bioprosthetic valves sewn
within a balloon-expanded or self-
| expanding stent Same valve as their PERIMOUNT Magna
87
Transcatheter Aortic Valve Implantation INSERTION
Retrograde transarterial insertion Requires femoral-iliac arteries to accommodate a 18-24fr delivery
system
Direct transapical insertion
88
Transcatheter Aortic Valve Implantation Ventricle is paced rapidly to
limit cardiac output for device positioning and expansion
89
Transcatheter Aortic Valve Implantation Position too high or too low
Paravalvular leaks Embolization
90
Percutaneous Mitral Valve Repair
| Multiple different types of devices 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
91
`Percutaneous Mitral Valve Repair
| Delivered by AND GUIDED BY
a transvenous, transseptal approach
| Guided by TEE
92
Percutaneous Mitral Valve Repair implanted on the
valve Grabs middle portions of the anterior and posterior
| mitral leaflets Creating edge to edge repair
93
Percutaneous Mitral Valve Repair has been used
on MVP, Flail leaflets, annular dilation, mitral regurg secondary to CM
