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Pediatrics > VALVULAR DEFECTS > Flashcards

VALVULAR DEFECTS Flashcards

1
Q

complete AVC Palliation

A

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

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2
Q

Complete AVC Repair Staged repair

A

HFP/BDG

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3
Q

Complete AVC Repair Final stage

A

Fontan

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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

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5
Q

Absent Pulmonary Valve

 AKA.

A

TOF with Absent Pulmonary Valve

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6
Q

Absent Pulmonary Valve Respiratory impairment

A

 R L shunting  systemic desaturation

 Compression of airway = compromised sats

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7
Q

Absent Pulmonary Valve Treatment:

A

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

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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

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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

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10
Q

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

A

Large ASD will decompress RA

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11
Q

Pulmonary Atresia with Intact Ventricular Septum

 Pathophysiology

A

Pulmonary Blood flow entirely dependent on PDA

 Requires PGE-1 infusion after birth

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12
Q

Pulmonary Atresia with Intact Ventricular Septum shunting

A

R L shunting atrially

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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

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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

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15
Q

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

A

 Open the atretic Pulmonary valve via transcatheter or

surgical valvotomy

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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

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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

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18
Q

Pulmonary Atresia
with Intact
Ventricular Septum CHARCTERISTICS

A
  1. ASD 2. Atretic Pulmonary Valve 3. PDA 4. Hypoplastic RV 5. Hypoplastic TV
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19
Q

Pulmonary Atresia – with VSD

 Aka.

A

TOF with Pulmonary Atresia (Extreme form of TOF)

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20
Q

Pulmonary Atresia – with VSD is

A

Failure of the development of the pulmonary valve

 Underdeveloped RV outflow tract and main PA

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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

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22
Q

Pulmonary Atresia – with VSD RV

A

Normal development of the RV

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23
Q

Pulmonary Atresia – with VSD (VSD + ASD)

A

arge VSD  May have an ASD

 Wide variations

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24
Q

Pulmonary Atresia – With VSD

 PathophysiologY

A

 Complete intracardiac mixing

 Systemic desaturation/ cyanosis

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25
Q

Pulmonary Atresia – With VSD aortopulmonary collaterals

A

 Porgressive stenosis  Hypoxemia

 “True pulmonary arteries” are hypoplastic

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26
Q

Pulmonary Atresia – With VSD repair

 Confluent branch PAs which are

A

fed by ductus. Complete surgical repair  Placement of RV to PA conduit (Rastelli Procedure)  Close VSD

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27
Q

Pulmonary Atresia – With VSD repair Hypoplastic branch PAs with aortopulmonary vessels

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

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28
Q

Pulmonary Stenosis (PS) prevalence

A

10% of Congenital Heart Diseases

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29
Q

Pulmonary Stenosis (PS) what is it?

A

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

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30
Q

Pulmonary Stenosis (PS) causes

A

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

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31
Q

Pulmonary Stenosis Types

 Supravalvular Stenosis

A

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

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32
Q

Pulmonary Stenosis Types valvular stenosis

A

 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

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33
Q

Pulmonary Stenosis Types subvalvular stenosis

A

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

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34
Q

Pulmonary Stenosis

 May be classified by RV Pressure

A

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

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35
Q

PS in infancy is always

A

severe

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36
Q

(PS ) If there is an ASD –

A

Right to left shunting will occur

 Cyanosis

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37
Q

Moderate pulmonary stenosis (or higher), will see

A

RVH

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38
Q

Repair of Pulmonary Stenosis

 If the defect is purely valvular:

A

 Balloon valvuloplasty  Commisurotomy - incise the fused commisures via direct vision

39
Q

Repair of Pulmonary Stenosis Infundibular Stensosis:

A

 Hypertrophied muscle in the outflow tract is resected

40
Q

Repair of Pulmonary Stenosis Supravalvular Stenosis:

A

 Depends where stenotic lesion is  Remove stenosis/ balloon angioplasty or stent  Patch repair/ enlargement (eyeball like)

41
Q

Aortic Stenosis prevalence

A

Congenital AS -10% of all congenital heart diseases

 Acyanotic lesion

42
Q

Aortic Stenosis what is it

A

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

43
Q

Aortic Stenosis what its associated and what it causes

A

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
Q

Supravalvular Aortic Stenosis prevalence what it is and when its seen

A

 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
Q

AS – SupraValvular Aortic Stenosis

 Correction

A

 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
Q

SubAortic Stenosis prevalence how it presents and what it causes

A

 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
Q

AS – SubAortic stenosis

 Correction

A

 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
Q

Subvalvular obstruction correction

A

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

49
Q

Subvalvular obstruction konno procedure

A

(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
Q

Critical Aortic Stenosis

A

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
Q

AS- Critical Aortic Stenosis

 Correction:

A

 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
Q

AS- Critical Aortic Stenosis

 Post operative course AND LOS

A

 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
Q

Aortic Insufficiency

A

Aortic valve fails to close completely immediately after systole

54
Q

AI symptoms

A

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

55
Q

Ross Procedure

A

 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
Q

why is ross procedure of choice in kids

A

 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
Q

Ross Procedure done as root replacement

A

 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
Q

Aortic Surgical Repair Ross Procedure

 BUT…

A

 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
Q

Ebstein’s Malformation/ Anomaly what it is pathophysiology prevalence

A

“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
Q

Ebstein’s Anomaly Orientation of the valve divides the RV into 2 parts

A

 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
Q

Ebstein’s Anomaly - Symptoms

A

 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
Q

Ebstein’s Anomaly – In Neonate

 Neonatal presentation

A

 Cyanosis due to RV dysfunction

 Functional PV “atresia”

63
Q

Ebstein’s Anomaly – In Neonate requires

A

PDA patency for pulmonary blood flow

64
Q

Ebstein’s Anomaly – In Neonate PV does not open due to

A

(normal formation) due to inability of RV to generate pressure in excess of PA pressure

65
Q

Ebstein’s Anomaly – In Neonate venous return

A

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

66
Q

Ebstein’s Malformation/ Anomaly

 Surgery is indicated with symptoms  Repair:

A

 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
Q

Postnatal correction of Ebstein’s

 Repair

A

 Plicate the atrialized portion of the RV  Reconstruct the Tricuspid valve annulus  Close the ASD  Resect the redundant atrial wall.

68
Q

Neonatal correction of Ebstein’s

 Repair described by Starns, et al. (CHLA)

A

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
Q

Tricuspid Atresia prevalence what it is what it causes

A

 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
Q

Tricuspid Atresia
 Clinical Features:
 Mortality rate is high

A

 50% die within 6 months  15-30% survive the first year without surgery  10% live to 10 years without surgery

71
Q

Tricuspid Atresia Severe cyanosis – complete mixing of blood causes

A

 Clubbing  Dyspnea  Fatigue

 Right heart failure

72
Q

Tricuspid Atresia

 Surgical Correction

A

 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
Q

Mitral Valve Insufficiency

A

 Incomplete closure or absence of the mitral valve  Increased filling of LV
 Leads to dilation and hypertrophy

74
Q

Mitral Valve Insufficiency Clinical Presentation:

A

 Palpitations, Fatigue, Orthopnea, Pulmonary Edema

75
Q

Mitral Valve Prolapse

A

 Mitral valve leaflets prolapse into the LA during systole  MVP associated with Mitral Insufficiency (MR). Not usually serious  Manydon’tevenknowtheyhaveit  Manylivewithitasymptomaticforyears

76
Q

Mitral Valve Prolapse symptoms

A

SOB, Palpitations, Chest pain.  Etiologyoftheseunclear

77
Q

Mitral Valve Prolapse treatment

A

 Doesn’trequiretreatmentunlesssignificantmitralinsufficiencyispresent  UsuallyonlysurgicalwithsevereMitralInsufficiencyandsymptomatic.

78
Q

Mitral Valve Stenosis

A 
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
Q

Mitral Valve Stenosis effects on LA and RV

A

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
Q

Mitral Valve Stenosis

 Treatment

A

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

81
Q

Valvuloplasty

A

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

82
Q

Percutaneous Pulmonary Valve Insertion

 For patients with

A

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

83
Q

Percutaneous Pulmonary Valve Insertion Transcatheter-

A

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

84
Q

Percutaneous Pulmonary Valve Insertion WHAT IS IT

A

Palliative procedure
 Extends life to RV to PA conduit
 High long term failure rate of valves in the pulmonary position
 Melody Valve

85
Q

Transcatheter Aortic Valve Implantation

 For patients witH

A

calcific aortic stenosis

86
Q

Transcatheter Aortic Valve Implantation Bioprosthetic valves sewn

A

within a balloon-expanded or self-

expanding stent  Same valve as their PERIMOUNT Magna

87
Q

Transcatheter Aortic Valve Implantation INSERTION

A

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

88
Q

Transcatheter Aortic Valve Implantation Ventricle is paced rapidly to

A

limit cardiac output for device positioning and expansion

89
Q

Transcatheter Aortic Valve Implantation Position too high or too low

A

 Paravalvular leaks  Embolization

90
Q

Percutaneous Mitral Valve Repair

 Multiple different types of devices  MitraClip

A

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
Q

`Percutaneous Mitral Valve Repair

 Delivered by AND GUIDED BY

A

a transvenous, transseptal approach

 Guided by TEE

92
Q

Percutaneous Mitral Valve Repair implanted on the

A

valve  Grabs middle portions of the anterior and posterior

mitral leaflets  Creating edge to edge repair

93
Q

Percutaneous Mitral Valve Repair has been used

A

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

Pediatrics (14 decks)

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