Topic 11: Valvular Defects Flashcards
Hemi-Fontan Procedure
Bi-directional Cavopulmonary Anastomosis
Anastamosis PA/Right atrial appendage
SVC is patched
Absent Pulmonary Valve - what happens?
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
Absent Pulmonary Valve associated with what?
VSD
Absent Pulmonary Valve leads to?
Massive dilation of Pulmonary Arteries
Lead to extrinsic compression of the bronchial airway leads to abnormal development of bronchial tree.
Absent Pulmonary Valve AKA ?
AKA. TOF with Absent Pulmonary Valve
Absent Pulmonary Valve treatment? (3)
Plication of the Pulmonary Arteries
Pulmonary Valve Replacement
VSD Closure
Absent Pulmonary Valve shunting?
R -> L shunting
systemic desaturation
Absent Pulmonary Valve respiratory involvement ?
Respiratory impairment
Compression of airway = compromised sats
Absent Pulmonary Valve three aspects?
- Absent Pulmonary Valve
- Dilated Pulmonary Arteries
- VSD
Pulmonary Atresia with intact ventricular septum (PA w/IVS)
what fails to form?
valves involed how?
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
Pulmonary Atresia with intact ventricular septum (PA w/IVS) what is created by the severe hypoplasia of RV?
Severe hypoplasia of RV results in creation of Coronary Artery Sinusoids*
Fistula between the RV and coronaries
* Can be catastrophic
Pulmonary Atresia with Intact Ventricular Septum: Pathophysiology
Pulm BF? shunting?
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
Pulmonary Atresia with Intact Ventricular Septum: Pathophysiology
Coronary perfusion dependent on what?
Coronary perfusion dependent on increased driving forces of obstructed RV (RV increased resistance is good)
Decompressing RV = Ischemia
Pulmonary Atresia with Intact Ventricular Septum: treatment? 4
- 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
Pulmonary Atresia with Intact Ventricular Septum Post operative course:
Prone to hemodynamic instability
Possibly delay chest closure
Length of Stay: 1-2 weeks
Pulmonary Atresia with Intact Ventricular Septum characteristics? 5
- ASD
- Atretic Pulmonary Valve
- PDA
- Hypoplastic RV
- Hypoplastic TV
Pulmonary Atresia–with VSD
AKA
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
Pulmonary Atresia–with VSD
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.
Pulmonary Atresia–with VSD
AKA
Aka. TOF with Pulmonary Atresia (Extreme form of TOF)
Wide variations
Pulmonary Atresia–with VSD -
how is pulm BF affected?
Discontinous–Pulmonary blood flow provided via Aortopulmonary Collaterals
Normal development of the RV
Pulmonary Atresia–with VSD
VSD - size
Large VSD
May have an ASD
Wide variations
Pulmonary Atresia–w VSD Pathophysiology 3
Complete intracardiac mixing
-Systemic desaturation/ cyanosis
Aortopulmonary collaterals
-Porgressive stenosis
-Hypoxemia
“True pulmonary arteries” are hypoplasticPulmonary Atresia–With VSD
Confluent branch PAs which are fed by ductus what is done?
Complete surgical repair
Placement of RV to PA conduit (Rastelli Procedure)
Close VSD
Pulmonary Atresia–With VSD
Hypoplastic branch PAs with aortopulmonary vessels what is done?
-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
Pulmonary Stenosis (PS)- % of CHD
10% of Congenital Heart Diseases
Pulmonary Stenosis (PS) - is what?
Pulmonary Valve and/or RV outflow tract is
restricted
Range from Mild to Severe
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
Pulmonary Stenosis Type
Supravalvular Stenosis
Pulmonary artery lumen above the pulmonary valve opening is narrowed
Can be main or branch PA
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
Pulmonary Stenosis Type: Subvalvular
Stenosis (Infundibular)
RVOT stenosis, below Pulmonary Valve
Obstructed by muscular tissue
Pulmonary Stenosis - May be classified by RV Pressure- Mild?
45mmHg or less
Pulmonary Stenosis - May be classified by RV Pressure - Moderate???
46-89mmHg
Moderate pulmonary stenosis (or higher), will see RVH
Pulmonary Stenosis - May be classified by RV Pressure - Severe??
90mmHg (suprasystemic)
Will develop right heart failure
PS in infancy is always severe
Pulmonary Stenosis if there is an ASD what will occur?
Right to left shunting will occur
Cyanosis
Repair of Pulmonary Stenosis - If the defect is purely valvular?
Balloon valvuloplasty
Commisurotomy-incise the fused commisures via direct vision
Repair of Pulmonary Stenosis, Infundibular Stensosis?
Hypertrophied muscle in the outflow tract is resected
Repair of Pulmonary Stenosis, Supravalvular Stenosis??
Depends where stenotic lesion is
Remove stenosis/ balloon angioplasty or stent
Patch repair/ enlargement (eyeball like)
Pulmonary Stenosis - types?
A. Supravalvular
B. Valvular Stenosis
C. Subvalvular/Infundibular Stenosis
Aortic Stenosis, Types?
Supravalvular
Subvalvular
Critical Aortic Stenosis
Aortic Stenosis – occurrance in CHD?
Congenital AS-10% of all congenital heart diseases
Acyanotic lesion
High risk for sudden cardiac death
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
Aortic Stenosis - what do you develop?
Develop LVH
- decreased ventricular function
- myocardial ischemia
- High risk for sudden cardiac death
Supravalvular Aortic Stenosis
Constriction of the aorta just above the valve due to fibrous membrane or hypoplastic aortic arch
Supravalvular Aortic Stenosis - how often is it seen, and with what population?
Uncommon
Seen in patients with Williams Syndrome
Familial form
Supravalvular Aortic Stenosis - can lead to what?
Can lead to LVH, LV dysfunction, ischemia and risk of sudden death
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
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
AS–SubAortic Stenosis - can lead to?
Can lead to LVH
Arrhythmias
Sudden death
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
AS–SubAortic Stenosis - correction determined by?
Done when obstruction is moderate to severe —–gradient determines!!!
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
AS-Critical Aortic Stenosis presents when?
Severe form of congenital AS
Presents in neonatal period
Symptoms become more acute as the PDA closes
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
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)
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
Aortic Insufficiency - describe
Aortic valve fails to close completely immediately
after systole
Aortic Insufficiency - symptoms?
LV dilation
Decreased CO
CHF
Exercise intolerance, Dyspnea on Exertion, Dizziness,
Pulsating headaches, increased pulse pressure,
pulmonary congestion, edema
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
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
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
Ebstein’s Malformation/ Anomaly - aka
“atrialized RV”
Ebstein’s Malformation/ Anomaly - occurance?
Rare congenital anomaly
0.5% of all Congenital Heart Diseases
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
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
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
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.
Ebsteins Malformations/Anomaly: Neonatal presentation - what does it require??
PDA patency for pulmonary BF
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
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
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
Tricuspid Atresia
3% of all Congenital Heart Disease
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
Tricuspid Atresia - what must be present?
VSD or PDA must be present to permit blood flow to pulmonary circulation
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
Tricuspid Atresia - symptoms?
Severe cyanosis–complete mixing of blood Clubbing Dyspnea Fatigue Right heart failure
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
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
Mitral Valve Insufficiency, Clinical Presentation:
Palpitations, Fatigue, Orthopnea, Pulmonary Edema
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
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
Mitral Valve Prolapse - treatment?
Doesn’t require treatment unless significant mitral insufficiency is present
Usually only surgical with severe Mitral Insufficiency and symptomatic
Mitral Valve Stenosis – congenital occurance?
Rare congenital heart disease
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
Mitral Valve Stenosis - leads to what??
Leads to pulmonary hypertension
Pulmonary Edema
Right Heart Failure
Mitral Valve Stenosis - pressure changes?
Increased LA pressures
Increased pulmonary venous, pulmonary arteriolar,
pulmonary artery, and RV systolic pressures
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
Mitral Valve Stenosis treatment?
Pulmonary edema–improved with diuretics
Surgical MV repair or replacement
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
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)
Percutaneous Pulmonary Valve Insertion - for patients with what?
For patients with failed RV to PA conduits (Rastelli) Stenosis or regurgitation
PALLIATIVE
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)
Transcatheter Aortic Valve Implantation - if valve is positioned too high or too low what happens?
Paravalvular leaks
Embolization
Transcatheter Aortic Valve Implantation - retrograde insertion requires what?
Requires femoral-iliac arteries to accommodate a 18-24fr delivery system
Transcatheter Aortic Valve Implantation - used with what patients?
For patients with calcific aortic stenosis
Edwards Sapen
Transcatheter Aortic Valve Implantation
Can be inserted transapical or tranfemoral
stent looking
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
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
