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Utah / kirklin > Congenital 2 > Flashcards

Congenital 2 Flashcards

1
Q

Clinical presentation of a Partial AV Canal

A

Partial AV Canal

a) Atrial shunt only: presents as an ASD
b) Shunt + Regurgitation: CHF

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2
Q

Clinical presentation of complete av canal

A

Complete AV Canal

a) Progressive CHF with tachypnea, tachycardia, poor peripheral perfusion, and FTT

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3
Q

Timing of AVSD repair

A

Partial

(1) Optimal age 1-2 y if valves are competent
(2) Earlier if there is CHF

Complete

(1) Optimal age is 2-6 months
(2) Earlier if there is CHF

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4
Q

Tetralogy of fallot

VSD

A

TOF VSD:

  • Malaligned VSD
  • Perimembranous 75-80%
  • Juxta-aortc
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5
Q

Aorta in TOF

  • General morphology
  • % Right sided arch and morphology of branches
A

Aorta in TOF

  • General morphology : Always large
  • % Right sided arch and morphology of branches :

a) Right arch 25% of cases
b) 90% will have mirror image branching head vessels

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6
Q

TOF - pulmonary valve morphology

A

TOF Pulmonary valve morphology:

Stenosis : 75%

valves are usually thickened

Cusps are teathered and free edges are shortened, pulling the commissures inward

Bicuspid – 50% - 2/3

alternatively the valve may be absent or atretic

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7
Q

TOF - morphology of the Pulmonary annulus

A

Pulmonary Annulus (Ring)- muscular structure at the RV /PA junction

a) Typically hypoplastic in relationship to the aortic annulus

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8
Q

Initial presentation of TOF -

Oxygenation and cyanosis

what is the cyanosis indicative of ?

###

A

a small percentage of patients present with deep neonatal cyanosis following the closure of the PDA.

The infants with profound cyanosis typically have severe valvar level stenosis, or near atresia.

###

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9
Q

Presentation and Natural history Acyanotic TOF patients

A

Presentation of acyanotic TOF patients

  • may have normal arterial oxygen saturation measurements
  • may have signs of pulmonary overcirculation if the pulmonary stenosis is mild.
  • initial mild desaturation in the range of 80 – 90% depending
  • may be delayed from the closure of the PDA

e) Acyanotic:
(1) decreasing baseline saturations à progressive right ventricular hypertrophy and subvalvar stenosis
(2) Acyanotic and mildly cyanotic à “ TET spells ”
(a) result from dynamic changes in the relative pulmonary and systemic resistances
(b) leads to severe right to left shunting
3. Clinical exam:
a) Varying degrees of cyanosis
b) Clubbing
c) Auscultation: systolic murmur from the LVOT obstruction
(1) This may disappear during a TET spell
(a) Little or no VSD murmur: à of the large size of the VSD and systemic level pressure in the right ventricle.
(2) No second heart sound 2/2 the low pressures across the pulmonary valve
d) Chest X-rays:
(1) may show the classic “ boot - shaped ” heart resulting from right ventricular hypertrophy and small pulmonary arterial knob

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10
Q

Murmur of TOF

A

Auscultation: systolic murmur from the RVOT obstruction

This may disappear during a TET spell

Little or no VSD murmur: of the large size of the VSD and systemic level pressure in the right ventricle.

No split second heart sound 2/2 the low pressures across the pulmonary valve

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11
Q

Grading of cardiac murmurs

A
  • Grade 1 = very faint
  • Grade 2 = quiet but heard immediately
  • Grade 3 = moderately loud
  • Grade 4 = loud
  • Grade 5 = heard with stethoscope partly off the chest
  • Grade 6 = no stethoscope needed
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12
Q

Grade 1 Murmur

A
  • Grade 1 = very faint
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13
Q

Grade 2 murmur

A

Grade 2 = quiet but heard immediately

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14
Q

TOF w pulmonary stenosis: Outcomes

  • Initial Hospital mortality:
  • 20 year survival:
A

TOF w pulmonary stenosis: Outcomes

  • Initial Hospital mortality: 2-5%
  • 20 year survival: 87%
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15
Q

TOF w pulmonary stenosis: Outcomes

Initial Hospital mortality?

A

TOF w pulmonary stenosis: Outcomes

Initial Hospital mortality: 2-5%

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16
Q

Tetralogy of Fallot w Pulmonary Stenosis

20 year survival:

A

Tetralogy of Fallot w Pulmonary Stenosis

20 year survival: 87%

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17
Q

Tetralogy of Fallot w Pulmonary Stenosis

% requiring surgical re-operation at 30 years?

A

Tetralogy of Fallot w Pulmonary Stenosis

By 30 years – 50% will need a re-operation

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18
Q

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early post operative death Death:

A

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early Death:

  1. Age: < 1-3 months or > 4 years
  2. RV/LV pressure ratio: > 0.7 – in the or or 24 hrs post op
  3. Severity of the annular hypoplasia
  4. Trans annular patch
  5. Previous palliative operations
  6. Multiple VSD
  7. Co-existing cardiac anomalies
  8. Down Syndrome
  9. Large AP collaterals
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19
Q

Tetralogy of Fallot with Pulmonary Atresia

Source of pulmonary blood flow:

A

no continuity from RV to PA

All pulmonary blood flow comes from ductus or collaterals

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20
Q

Tetralogy of Fallot with Pulmonary Atresia

Frequency of discontinuity between right and left pulmonary arteries?

A

Tetralogy of Fallot with Pulmonary Atresia

Frequency of discontinuity between right and left pulmonary arteries?

RPA and LPA in discontinuity in 20-30%

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21
Q

Tetralogy of Fallot with Pulmonary Atresia

Associated with what congenital syndrome ?

A

Tetralogy of Fallot with Pulmonary Atresia

Associated with 22q11 deletion (DiGeorge / velocardiofacial syndrome)

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22
Q

Tetralogy of Fallot with Pulmonary Atresia

Anatomic Subtypes -

A

Tetralogy of Fallot with Pulmonary Atresia

Anatomic Subtypes -

  • Type A (50%): Native pulmonary arties only – duct dependent
  • Type B (25%): Hypoplastic PA + MAPCA
  • Type C ( 25%): Absent PA’s only MAPCA
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23
Q

Type A Tetralogy of Fallot with Pulmonary Atresia

A

Tetralogy of Fallot with Pulmonary Atresia

Anatomic Subtypes -

Type A (50%): Native pulmonary arties only – duct dependent

Type B (25%): Hypoplastic PA + MAPCA

Type C ( 25%): Absent PA’s only MAPCA

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24
Q

Anatomic Type B Tetralogy of Fallot with Pulmonary Atresia

A

Tetralogy of Fallot with Pulmonary Atresia

Anatomic Subtypes -

Type A (50%): Native pulmonary arties only – duct dependent

Type B (25%): Hypoplastic PA + MAPCA

Type C ( 25%): Absent PA’s only MAPCA

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25
Q

Anatomic Type C Tetralogy of Fallot with Pulmonary Atresia

A

Tetralogy of Fallot with Pulmonary Atresia

Anatomic Subtypes -

Type A (50%): Native pulmonary arties only – duct dependent

Type B (25%): Hypoplastic PA + MAPCA

Type C ( 25%): Absent PA’s only MAPCA

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26
Q

TOF with Pulmonary Atresia - MAPCA:

A

TOF with Pulmonary Atresia - MAPCA: major aortopulmonary collateral arteries

  • Present in up to 60% of TOF/PA
  • Derived from splanchnic vascular plexus
  • Highly variable
  • 1-6 in number
  • originate from mid thoracic aorta
  • Serpiginous course
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27
Q

Syndromes associated with Pulmonary stenosis with intact ventricular septum

A

Noonan syndrome

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28
Q

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Pulmonary Valve:

A

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Pulmonary Valve:

  • Trileaflet – 70%
  • Well defined valve with fused commissures form a fiberous cone
  • 10-20% a dysplastic valve causes the stenosis
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29
Q

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Pulmonary Artery?

A

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Pulmonary Artery:

Hypoplastic secondary to low blood flow

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30
Q

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of theRight Ventricle?

A

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of theRight Ventricle:

  • Mild to moderate reduction in the cavity size
  • Concentric hyperplasia
  • Endocardial fibrosis is common
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31
Q

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Tricuspid?

A

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Tricuspid:

Normal annulus in 50%

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32
Q

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Right Atrium?

A

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Morphology of the Right Atrium:

Nearly always a PFO or an ASD, which results in a right to left shunt

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33
Q

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Myocardial blood flow?

A

Pulmonary stenosis with intact ventricular septum - Neonatal presentation

Myocardial blood flow:

  • 10% sinusoids
  • 2% RV coronary artery fistulae
  • Rarely, RV dependent on coronary blood flow
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34
Q

Pulmonary stenosis with intact ventricular septum- neonatal presentation

  1. survival of those without surgical treatment ?
  2. survival of those with surgical treatment ?
A

Pulmonary stenosis with intact ventricular septum- neonatal presentation

  1. survival of those without surgical treatment - certain death
  2. survival of those with surgical treatment - 6% mortality
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35
Q

Pulmonary stenosis with intact ventricular septum- neonatal presentation

Preferred initial therapy?

What makes this technique difficult?

Success Rates?

Failure Rate?

Restenosis Rate?

A

Pulmonary stenosis with intact ventricular septum- neonatal presentation

Preferred initial therapy: Balloon valvotomy - quickly replacing surgery

What makes this technique difficult: Infundibular hyptertrophy increases the risk and reduces the chance of success

Success Rates: 50-90% success rate

Failure Rate: 50% fail due to inability to cross the valve

Restenosis Rate: 25% restenosis rate

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36
Q

Pulmonary stenosis with intact ventricular septum- neonatal presentation

Operative intervention- surgical technique ?

A

Pulmonary stenosis with intact ventricular septum- neonatal presentation

Operative intervention- surgical technique ?

Surgical valvotomy –

(a) with cardioplegic arrest
(b) valvotomy with inflow occlusion

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37
Q

Infants/ Children/ and Adults Pulmonary Stenosis with intact ventricular septum

Associated conditions:

####

A

Infants/ Children/ and Adults Pulmonary Stenosis with intact ventricular septum

Associated conditions:

a) Noonan Syndrome
b) Intrauterine Rubella

###

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38
Q

Infants/ Children/ and Adults Pulmonary Stenosis with intact ventricular septum

Murmur:

A

Infants/ Children/ and Adults Pulmonary Stenosis with intact ventricular septum

Murmur: Harsh systolic murmur with an ejection click

Right ventricular heave

###

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39
Q

Infants/ Children/ and Adults Pulmonary Stenosis with intact ventricular septum

ECG?

A

Infants/ Children/ and Adults Pulmonary Stenosis with intact ventricular septum

ECG?

(1) Prominent P-waves
(2) Right axis deviation
(3) R wave in v1 corrleates with RV pressure and pulmonary stenosis

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40
Q

Diagnosis ?

A

Retro-esophageal right subclvian artery

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41
Q

Pulmonary Atresia with intact ventricular Septum

Mild RVOT obsctuction

RV to PA gradent

A

25mmHg

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42
Q

Pulmonary Atresia with intact ventricular Septum

Mild RVOT obsctuction -Peak RV Presssure?

A

50mmHg

43
Q

Pulmonary Atresia with intact ventricular Septum

RV to PA gradient 25, with a peak pressure of 50mmHg?

what is the degree of obstruction and consequential survival?

A

Pulmonary Atresia with intact ventricular Septum

RV to PA gradient 25, with a peak pressure of 50mmHg?

severe obstruction and normal srvival

44
Q

what is the major determinant when figuring out Pulmonary Atresia with intact ventricular septum

A

the myocarial perfusion is dependent on sinusoids and coronary arteriy fisulae, which ultimately govern the clinical course

45
Q

Initial preoperative management of pulmonary artresia with inact ventricular septum?

A

PGE1

Balance PA and systemic blood flow

46
Q

PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Morphology?

Pulmonary Arteries:

Right Ventricle:

Tricuspid Valve:

Right Atrium:

A
  • Pulmonary Arteries:
    • Trunk is usually near normal in size
    • RPA and LPA are normal or Slightly Hypoplastic
  • Right Ventricle
    • Hypoplastic with myocardial hypertrophy in 90%
    • Reduced cavity size
    • Endocardial fibroelastosis
  • Tricuspid Valve
    • Dysplastic in most cases
    • TR is typical
    • TV size correlates directly with RV cavity size
  • Right Atrium
    • Always in enlarged secondary to ASD
47
Q

PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Morphology of Right Ventricle?

A
  • Right Ventricle
    • Hypoplastic with myocardial hypertrophy in 90%
    • Reduced cavity size
    • Endocardial fibroelastosis
48
Q

PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Morphology of Pulmonary Arteries?

A
  • Pulmonary Arteries:
    • Trunk is usually near normal in size
    • RPA and LPA are normal or Slightly Hypoplastic
49
Q

PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Morphology of Tricuspid Valve:

A
  • Tricuspid Valve
    • Dysplastic in most cases
    • TR is typical
    • TV size correlates directly with RV cavity size
50
Q

PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Right Atrium morphology:

A
  • Right Atrium
    • Always in enlarged secondary to ASD
51
Q

Pulmonary Atresia with Intact Ventricular Septum

Most Significant Issue in Surgical Planning

A

CORONARY PEFUSION

  • Sinusioids
    • RV sinusoids are seen in 50% of cases
    • RV to coronary fistulae
    • This is inversely correlated to the TV diameter, RV cavity size, degree of TR:
    • Related to RV pressures
  • RV Dependent coronary Circulation
    • Frequency: 20% of cases
    • Depends on the proximal coronary artery stenosis
    • Difficult to decide how much myocardium is at risk when the hypertensive RV is decompressed
    • Small amount of myocardial ischemia may cause arrhythmias
52
Q

Pulmonary Atresia with Intact Ventricular Septum

CORONARY PEFUSION

How often are RV sinusoids seen?

A

RV sinusoids are seen in 50% of cases

53
Q

Pulmonary Atresia with Intact Ventricular Septum

CORONARY PEFUSION

Frequency of RV sinusoids ?

Upon what is their devellopment dependent?

A

Pulmonary Atresia with Intact Ventricular Septum

RV sinusoids are seen in 50% of cases

This is inversely correlated to the TV diameter, RV cavity size, degree of TR:

Related to RV pressures

Based on z-score:

Z score 0: 5%

Z score -3: 68%

Z score -5: 96%

54
Q

Pulmonary Atresia with Intact Ventricular Septum

Frequency of RV Dependent coronary Circulation?

A

Frequency: 20% of cases

55
Q

Pulmonary atresia with intact ventricular septum

Risk factors for Repair

A

Small Diameter Tricuspid valve

RV dependent myocardial blood flow

56
Q

Pulmonary Atresia with Intact Ventricular Septum

Surgical management:

Risk factors for surgery?

A

Pulmonary Atresia with Intact Ventricular Septum

Surgical management-Risk factors for surgery:

  1. Small diameter TV
  2. RV dependent blood flow

###

57
Q

Surgical management of Pulmonary Atresia with Intact Ventricular Septum

Goals of Surgery?

A

Surgical management of Pulmonary Atresia with Intact Ventricular Septum

Goals of Surgery?

  1. Relief of the RVOTO – valvotomy or transannular patch
  2. Creation of a Systemic – pulmonary shunt
  3. Relief of the RVOTO and Shut
  4. Ligation of the Ductus
58
Q

Surgical management of Pulmonary Atresia with Intact Ventricular Septum

Upon what is the Treatment Strategy based?

A
  1. z score
  2. right heart morphology
  3. coronary circulation
59
Q

Surgical management of Pulmonary Atresia with Intact Ventricular Septum for neonate with

Z score 0 to -2?

A

Surgical management of Pulmonary Atresia with Intact Ventricular Septum for neonate with Z score 0 to -2: Nearly Normal RV and TV

  • Ideal to perform RVOT relief alone
60
Q

Surgical management of Pulmonary Atresia with Intact Ventricular Septum for neonate with Z score -2 to -3?

A

mild-mod TV hypoplasia

Relief of the RVOTO + Shunt

2 ventricle repair if adequate RV/TV

Single ventricle if inadequate growth

61
Q

Surgical management of Pulmonary Atresia with Intact Ventricular Septum for neonate with Z score Z score ?

A

Z score < -3: Severe TV annular hypoplasia

  • Shunt alone
  • 6-12 months of age bi-directional Glenn
  • 1-4 years of age –fontan procedure
62
Q

Vascular ring - general definition

A

Vascular ring: abnormality of the aortic arch that results in either complete or partial encirclement of the trachea or the esophagus

63
Q

Vascular Sling: general defintion

A

Vascular Sling: Left PA originates from the Right PA and passes between the trachea and esophagus

64
Q

Double aortic arch - which arch is dominant? how often?

A
  • Right Arch: Larger in 50%
  • Left Arch: Larger in 25%
  • The arches are balanced in 25%
65
Q

Varriants of right sided aortic arch?

which one is a complete vascular ring?

A
  1. Right Arch (Mirror Image Branching)
    1. NOT a complete vascular ring
  2. Right arch with aberrant LSCA
    1. IS a complete vascular ring
66
Q

What conditions are associated with right aortic arch with mirror image branching?

A
  1. Right Arch (Mirror Image Branching)
    1. Commonly associated with TOF and truncus
67
Q

Varities of incomplete vascular rings

A

Incomplete Vascular Rings:

  1. Innominate artery compression syndrome
  2. Pulmonary artery sling (RPA off of the left PA)
  3. Left arch with aberrant right subclavian artery
68
Q

Surgical Repair of a PA Sling

A

Per Utah:

  1. Median Sternotomy
  2. Cardiopulmonary bypass
  3. Reimplant the LPA into the main PA
  4. Tracheoplasty for the complete rings – this is the most important part of determining the success of the procedure
  5. Pericardial flap between the great vessels and the tracheal reconstruction.
69
Q

General definition of Truncus ateriosus

A

Failure of development of the aortocopulmonary and infundibular septums

Therefore a single arterial vessel from the heart overriding the ventricular septum giving rise to the:

  • systemic
  • coronary
  • pulmonary circulation

There is always a VSD

70
Q

side preference of the tuncus in TA?

A

Truncal artery –

Overrides the ventricular septum in 50%

Overides the right in 40%

Left in 10%

71
Q

Truncal valve in truncus arteriosus

what is the structure of the leaflets?

% deviation from tricuspid?

A

Truncal valve Leafelets in truncus arteriosus

    • Leaflet number is variable
      • 65% are tricuspid
      • 25% are quadracuspid
      • balance are bicuspid or five leaflets
72
Q

What is the annular structure in the Truncal valve of truncus arteriosus?

A

Annular structure

Fiberous continuity between the posterior leaflets and the anterior leaflet of the mitral valve as with the normal heart.

73
Q

frequency of physiologic dysfunction in the Truncal valve in truncus arteriosus?

A

Function:

50% have truncal insufficiency

Severe myxomatous changes are associated with truncal valve insufficiency

74
Q

Describe the coronaries of truncus arteriossus

A

Coronary blood flow:

  • Usually arise from the sinus of Valsalva
  • A minority (20%) arise centrally or normally within the sinus
  • The Majority (80%) are at the _margin of the sinu_s or the upper margin of the commissure
  • At least 1/3 – _one coronary orifice (usually the left) comes above the ST-ridg_e
  • 50% have abnormalities- such as
    • Osteal Stenosis
    • Unually high take off (esp the LAD)
    • Large conal and diagonal branches
75
Q

Describe the VSD of Truncus arteriosus ?

What does this mean for the ventricular structures?

A

VSD:

  • Ventricular Septal Defect is high an anterior
  • Large
  • Typically under the truncal artery (so the truncal valve is the superior margin)

Ventricles:

  • RV conal septum is absent
  • LVOTO is relatively normal
76
Q

Abrnomalities associated with Truncus Arteriosus

A

Associated Abrnomaities

IAA (B) or coarcation 10-20%

Right aortic arch 25-30%

Persistent left superior vc 10%

Chromosome 22q11 deleton

DeGeorge Syndrome

77
Q

truncus

what percent of CHD ?

A

Natural History

  • 2.8% of CHD
    *
78
Q

Natural history of truncus arteriosus

  • % die within first month
  • % die within frist year
A

Natural History

  • 50% die in first month
  • 88% die in the first year
79
Q

what is the most common kind of reoperation for Truncus

A

Reoperation for the RV to PA Conduit

80
Q

Presentaion of child with pulmonary atreasia with IVS

A
  • Well developed babies
  • Early and rapid cyanosis
  • Absent P2
  • CXR clear oligemic lung fields
  • EKG normal or absence of RVH
  • Echo diagnostic
81
Q

Congenital aortic stenosis

valve gradient considered severe

A
    • Severe stenosis – Gradient > 75mmHg
82
Q

Presentation of neonatal aortic stenosis

A
  • Neonate/Infant:
    • Most severe valve deformity
    • Severe stenosis – Gradient > 75mmHg
    • Reduced Ventricular Function
    • Needs to be differentiated from HLHS
      *
83
Q

Supravalvular aortic stenosis

Associated lesions

A
  1. Subvalvular LVOTO
  2. Multiple peripheral pulmonary artery stenosis
  3. Stenosis of major systemic arteries
  4. Familial form
  5. Williams Syndrome
84
Q

What is williams syndrome

A
  • Devellopmental delay, elefin facies, hypercalcemia
  • Elastin gene mutation on chromosome 7q11.23
  • Sudden death at a young age is common
85
Q

Surgery for congenital supravalvular aortic stenosis

frequency of reoperation

A

Many patients (25-30%) require operation in 10-20 years.

86
Q

Sinus of Valsalva Aneurysm

Morphology

A
  1. Separation of : the aortic media of the sinus from the media adjacent to the hinge of the aortic leaflet
  2. Typically absence of normal elastic tissue
  3. Weak area gives way to aortic pressure
  4. Excavation into the underlying tissue –>
  5. Ruptues into the adjacent coronary chamber as a “windsock:”
87
Q

Sinus of Valsalva Aneurysm

Associated Pathology

A
  • The “windsock” may rupture –> generating a fistula most commonly into the RV
  • VSD
  • Aortic valve (Prolapse with the VSD)
  • Bicuspid Aortic valve
  • Pulmonic stenosis
88
Q

Sinus of Valsalva Aneurysm

Where do the fistulas most commonly form?

A

The “windsock” may rupture –> generating a fistula most commonly into the RV

89
Q

Sinus of Valslava Aneurysm

VSD: frequency associated

A

30-50%

90
Q

Sinus of Valslava Aneurysm

VSD -

  • frequency ?
  • Morphology?
A

VSD

  • 30-50%
  • Perimembranous right 1/3 of the sinus
  • Sub arterial – leftward sinus
  • Hinge line of the aorticleaflet separates the aneurysm of the VSD
91
Q

Sinus of valsalva aneurysm

Related aortic valve issues

A

Prolapse of the leaflet with with the VSD

Bicuspid valve

92
Q

Sinus of Valslava Aneurysm

Nature of pulmonic stenosis

A

Pulmonic stenosis

Usually subvalvular related to the windsock in the RVOT

93
Q

Stenosis of the Aortic Valve

Indications for surgery in infancy

A
  • Gradient > 75mmHg
  • Depressed ventricular function
  • Symptoms of ischemia or syncope
    *
94
Q

congenital stenotic aortic valve

factors to consider with patient presenting as an older infant or child:

A
  • With moderate stenosis in older infants or children:
    • Sudden death is rare if the gradient < 75mmHg
    • Valve replacement is inevitable
95
Q

Congenital aortic valve stenosis

options for intervention:

Neonate/infant:

Child:

Adult/Elderly:

A

Neonate/infant: BAV/Valvotomy for survival

Child: BAV/Valvotomy for palliation

Adult/Elderly: AVR

96
Q

varriations of subvalvular aortic stenosis

A
  1. Localized form (Membranous- Fibrous)
  2. Diffuse form (Tunnel, Muscular HOCM)
97
Q

Key features of the localized form of subvalvular aortic stenosis

A
  1. Discrete fibrous or fibromuscular obstruction of the LVOT below the aortic valve
  2. More prominent anterior and laterally
  3. Aortic valve annulus is is smaller than normal
  4. VSD is commonly associated and stenosis may develop after spontaneous closure of the VSD.
98
Q

Key features of the diffuse form subvalvular aortic stenosis

A

Diffuse form (Tunnel, Muscular HOCM)

  1. Spectrum of defects and varying degrees of aortic obstruction
  2. Most severe form is the long tunnel and small aortic annulus
  3. Aortic valve is usually tricuspid and normal or with thickened leaflets
  4. LV is concentrically hypertrophy
99
Q

Most severe form of diffuse subvalvular aortic stenosis

A

​

  1. Most severe form is the long tunnel and small aortic annulus
100
Q

Morphology of the aortic valve in the setting of subvalvular aortic stenosis

A

​

  1. Aortic valve is usually tricuspid and normal or with thickened leaflets
101
Q

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early post opreative death :

  • Impact of age?
A

Increased risk if:

Age: < 1-3 months or > 4 years

102
Q

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early post opreative death :

RV/LV pressure ratio: ?

A

_RV/LV pressure rati_o: > 0.7 – in the OR or 24 hrs post op

103
Q

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early post opreative death :

Structural considerations (4)?

A

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early post opreative death : Structural considerations?

  1. Severity of the annular hypoplasia
  2. Trans annular patch
  3. Multiple VSD
  4. Large AP collateral
104
Q

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early post opreative death :

Comorbidities and history impacting ?

A

Tetralogy of Fallot w Pulmonary Stenosis

Risk factors for early post opreative death : Comorbidities impacting ?

  1. Previous palliative operations
  2. Co-existing cardiac anomalies
  3. Down Syndrome

Utah / kirklin (12 decks)

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