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Flashcards in VALVULAR DEFECTS Deck (93):

complete AVC Palliation

Shunt (Qp/Qs) Too much flow  Not enough flow


Complete AVC Repair Staged repair



Complete AVC Repair Final stage



Absent Pulmonary Valve

 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
 AKA.

TOF with Absent Pulmonary Valve


Absent Pulmonary Valve Respiratory impairment

 R L shunting  systemic desaturation
 Compression of airway = compromised sats


Absent Pulmonary Valve Treatment:

Plication of the Pulmonary Arteries  Pulmonary Valve Replacement  VSD Closure


Pulmonary Atresia with intact ventricular septum (PA w/IVS)

 Complete atresia of pulmonary valve  Pulmonary valve fails to form late in development. PA is normal size


Pulmonary Atresia with intact ventricular septum (PA w/IVS) RV and Tricuspid Valve

hypoplastic. 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) ASD

Large ASD will decompress RA


Pulmonary Atresia with Intact Ventricular Septum
 Pathophysiology

Pulmonary Blood flow entirely dependent on PDA
 Requires PGE-1 infusion after birth


Pulmonary Atresia with Intact Ventricular Septum shunting

R L shunting atrially


Pulmonary Atresia with Intact Ventricular Septum coronary perfusion

ependent on increased driving forces of obstructed RV (RV increased resistance is good)
 Decompressing RV = Ischemia


Pulmonary Atresia with Intact Ventricular Septum Treatment:

 PGE-1 to maintain duct patency
 RV dependent Sinusoids  Balloon atrial septostomy to decompress the RA


Pulmonary Atresia with Intact Ventricular Septum Treatment: NO RV dependent Sinusoids

 Open the atretic Pulmonary valve via transcatheter or
surgical valvotomy


Pulmonary Atresia with Intact Ventricular Septum 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: and LOS

 Prone to hemodynamic instability  Possibly delay chest closure
 Length of Stay: 1-2 weeks


Pulmonary Atresia
with Intact
Ventricular Septum CHARCTERISTICS

1. ASD 2. Atretic Pulmonary Valve 3. PDA 4. Hypoplastic RV 5. Hypoplastic TV


Pulmonary Atresia – with VSD
 Aka.

TOF with Pulmonary Atresia (Extreme form of TOF)


Pulmonary Atresia – with VSD is

Failure of the development of the pulmonary valve
 Underdeveloped RV outflow tract and main PA


Pulmonary Atresia – with VSD branch PA may be

confluent and fed by ductus or discontinuous and hypoplastic.
 Discontinous – Pulmonary blood flow provided via Aortopulmonary Collaterals


Pulmonary Atresia – with VSD RV

Normal development of the RV


Pulmonary Atresia – with VSD (VSD + ASD)

arge VSD  May have an ASD
 Wide variations


Pulmonary Atresia – With VSD
 PathophysiologY

 Complete intracardiac mixing
 Systemic desaturation/ cyanosis


Pulmonary Atresia – With VSD aortopulmonary collaterals

 Porgressive stenosis  Hypoxemia
 “True pulmonary arteries” are hypoplastic


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


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


Pulmonary Stenosis (PS) prevalence

10% of Congenital Heart Diseases


Pulmonary Stenosis (PS) what is it?

Pulmonary Valve and/or RV outflow tract is
 Range from Mild to Severe


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


Pulmonary Stenosis Types
 Supravalvular Stenosis

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


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


Pulmonary Stenosis Types subvalvular stenosis

 RVOT stenosis, below Pulmonary Valve  Obstructed by muscular tissue
Pulmonary Stenosis


Pulmonary Stenosis
 May be classified by RV Pressure

 Mild: 45mmHg or less
 Moderate: 46-89mmHg
 Severe: 90mmHg (suprasystemic)  Will develop right heart failure


PS in infancy is always



(PS ) If there is an ASD –

Right to left shunting will occur
 Cyanosis


Moderate pulmonary stenosis (or higher), will see



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)


Aortic Stenosis prevalence

Congenital AS -10% of all congenital heart diseases
 Acyanotic lesion


Aortic Stenosis what is it

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


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


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


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


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


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


Subvalvular obstruction correction

Aortic valve annular hypoplasia and subvalvular obstruction
 Cannot just replace the valve
 Must enlarge the annulus


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.


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


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)


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


Aortic Insufficiency

Aortic valve fails to close completely immediately after systole


AI symptoms

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


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


why is ross procedure of choice in kids

 Follow up studies show the pulmonary autograft grows !!!!!!!!
 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 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.


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


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


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


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


Ebstein’s Anomaly – In Neonate
 Neonatal presentation

 Cyanosis due to RV dysfunction
 Functional PV “atresia”


Ebstein’s Anomaly – In Neonate requires

PDA patency for pulmonary blood flow


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


Ebstein’s Anomaly – In Neonate venous return

Venous return to the heart goes thru an ASD/PFO to the LA.


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


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
 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


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


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


Tricuspid Atresia Severe cyanosis – complete mixing of blood causes

 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

 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). Not usually serious  Manydon’tevenknowtheyhaveit  Manylivewithitasymptomaticforyears


Mitral Valve Prolapse symptoms

SOB, Palpitations, Chest pain.  Etiologyoftheseunclear


Mitral Valve Prolapse treatment

 Doesn’trequiretreatmentunlesssignificantmitralinsufficiencyispresent  UsuallyonlysurgicalwithsevereMitralInsufficiencyandsymptomatic.


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.


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


Mitral Valve Stenosis
 Treatment

 Pulmonary edema – improved with diuretics  Surgical MV repair or replacement



 Transcatheter pulmonary balloon valvuloplasty  Results equal to open surgical valvotomy
 Careful determination of anatomy via Transthoracic echo and angiograms.


Percutaneous Pulmonary Valve Insertion
 For patients with

failed RV to PA conduits (Rastelli)  Stenosis or regurgitation


Percutaneous Pulmonary Valve Insertion Transcatheter-

-delivered valve that has been mounted within a balloon-expandable stent


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


Transcatheter Aortic Valve Implantation
 For patients witH

calcific aortic stenosis


Transcatheter Aortic Valve Implantation Bioprosthetic valves sewn

within a balloon-expanded or self-
expanding stent  Same valve as their PERIMOUNT Magna


Transcatheter Aortic Valve Implantation INSERTION

Retrograde transarterial insertion  Requires femoral-iliac arteries to accommodate a 18-24fr delivery
 Direct transapical insertion


Transcatheter Aortic Valve Implantation Ventricle is paced rapidly to

limit cardiac output for device positioning and expansion


Transcatheter Aortic Valve Implantation Position too high or too low

 Paravalvular leaks  Embolization


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


`Percutaneous Mitral Valve Repair
 Delivered by AND GUIDED BY

a transvenous, transseptal approach
 Guided by TEE


Percutaneous Mitral Valve Repair implanted on the

valve  Grabs middle portions of the anterior and posterior
mitral leaflets  Creating edge to edge repair


Percutaneous Mitral Valve Repair has been used

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