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
2
Q
Clinical presentation of complete av canal
A
Complete AV Canal
a) Progressive CHF with tachypnea, tachycardia, poor peripheral perfusion, and FTT
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
4
Q
Tetralogy of fallot
VSD
A
TOF VSD:
- Malaligned VSD
- Perimembranous 75-80%
- Juxta-aortc
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
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
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
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.
###
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
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
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
12
Q
Grade 1 Murmur
A
- Grade 1 = very faint
13
Q
Grade 2 murmur
A
Grade 2 = quiet but heard immediately
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%
15
Q
TOF w pulmonary stenosis: Outcomes
Initial Hospital mortality?
A
TOF w pulmonary stenosis: Outcomes
Initial Hospital mortality: 2-5%
16
Q
Tetralogy of Fallot w Pulmonary Stenosis
20 year survival:
A
Tetralogy of Fallot w Pulmonary Stenosis
20 year survival: 87%
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
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:
- Age: < 1-3 months or > 4 years
- RV/LV pressure ratio: > 0.7 – in the or or 24 hrs post op
- Severity of the annular hypoplasia
- Trans annular patch
- Previous palliative operations
- Multiple VSD
- Co-existing cardiac anomalies
- Down Syndrome
- Large AP collaterals
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
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%
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)
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
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
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
25
Anatomic Type C Tetralogy of Fallot with Pulmonary Atresia
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_
26
TOF with Pulmonary Atresia - MAPCA:
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
27
**Syndromes** associated with **Pulmonary stenosis with intact ventricular septum**
Noonan syndrome
28
**Pulmonary stenosis with intact ventricular septum** - _Neonatal presentation_
*Morphology of the* _Pulmonary Valve:_
**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
29
**Pulmonary stenosis with intact ventricular septum - Neonatal presentation**
*Morphology of the* _Pulmonary Artery?_
**Pulmonary stenosis with intact ventricular septum - Neonatal presentation**
*Morphology of the* _Pulmonary Artery:_
Hypoplastic secondary to low blood flow
30
**Pulmonary stenosis with intact ventricular septum - Neonatal presentation**
*Morphology of the*_Right Ventricle?_
**Pulmonary stenosis with intact ventricular septum - Neonatal presentation**
*Morphology of the*_Right Ventricle:_
* Mild to moderate reduction in the cavity size
* Concentric hyperplasia
* Endocardial fibrosis is common
31
**Pulmonary stenosis with intact ventricular septum - Neonatal presentation**
_Morphology of the_ *Tricuspid?*
**Pulmonary stenosis with intact ventricular septum - Neonatal presentation**
*Morphology of the* _Tricuspid_:
Normal annulus in 50%
32
**Pulmonary stenosis with intact ventricular septum - Neonatal presentation**
*Morphology of the* _Right Atrium?_
**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
33
Pulmonary stenosis with intact ventricular septum - Neonatal presentation
Myocardial blood flow?
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
34
**Pulmonary stenosis with intact ventricular septum- neonatal presentation**
1. survival of those without surgical treatment ?
2. survival of those with surgical treatment ?
**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
35
**Pulmonary stenosis with intact ventricular septum- neonatal presentation**
Preferred initial therapy?
What makes this technique difficult?
Success Rates?
Failure Rate?
Restenosis Rate?
**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
36
**Pulmonary stenosis with intact ventricular septum- neonatal presentation**
Operative intervention- surgical technique ?
**Pulmonary stenosis with intact ventricular septum- neonatal presentation**
Operative intervention- surgical technique ?
Surgical valvotomy –
(a) with cardioplegic arrest
(b) valvotomy with inflow occlusion
37
_Infants/ Children/ and Adults_ Pulmonary Stenosis with intact ventricular septum
_Associated conditions:_
####
_Infants/ Children/ and Adults_ Pulmonary Stenosis with intact ventricular septum
_Associated conditions:_
a) Noonan Syndrome
b) Intrauterine Rubella
###
38
**_Infants/ Children/ and Adults_ Pulmonary Stenosis with intact ventricular septum**
Murmur:
**_Infants/ Children/ and Adults_ Pulmonary Stenosis with intact ventricular septum**
Murmur: Harsh systolic murmur with an ejection click
Right ventricular heave
###
39
**_Infants/ Children/ and Adults_ Pulmonary Stenosis with intact ventricular septum**
ECG?
**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
40
Diagnosis ?
Retro-esophageal right subclvian artery
41
_Pulmonary Atresia with intact ventricular Septum_
**Mild** RVOT obsctuction
**RV to PA gradent**
25mmHg
42
_Pulmonary Atresia with intact ventricular Septum_
*Mild* RVOT obsctuction -Peak RV Presssure?
50mmHg
43
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?
Pulmonary Atresia with intact ventricular Septum
RV to PA gradient 25, with a peak pressure of 50mmHg?
severe obstruction and normal srvival
44
what is the major determinant when figuring out Pulmonary Atresia with intact ventricular septum
the myocarial perfusion is dependent on sinusoids and coronary arteriy fisulae, which ultimately govern the clinical course
45
Initial preoperative management of pulmonary artresia with inact ventricular septum?
PGE1
Balance PA and systemic blood flow
46
PULMONARY *ATRESIA* WITH INTACT VENTRICULAR SEPTUM
_Morphology?_
_Pulmonary Arteries:_
_Right Ventricle:_
_Tricuspid Valve:_
_Right Atrium:_
* _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
PULMONARY *ATRESIA* WITH INTACT VENTRICULAR SEPTUM
_Morphology of Right Ventricle?_
* _Right Ventricle_
* Hypoplastic with myocardial hypertrophy in 90%
* Reduced cavity size
* Endocardial fibroelastosis
48
PULMONARY *ATRESIA* WITH INTACT VENTRICULAR SEPTUM
_Morphology of Pulmonary Arteries?_
* _Pulmonary Arteries:_
* Trunk is usually near normal in size
* RPA and LPA are normal or Slightly Hypoplastic
49
PULMONARY *ATRESIA* WITH INTACT VENTRICULAR SEPTUM
_Morphology of Tricuspid Valve:_
* _Tricuspid Valve_
* Dysplastic in most cases
* TR is typical
* TV size correlates directly with RV cavity size
50
PULMONARY *ATRESIA* WITH INTACT VENTRICULAR SEPTUM
_Right Atrium morphology:_
* _Right Atrium_
* Always in enlarged secondary to ASD
51
**_Pulmonary Atresia with Intact Ventricular Septum_**
Most Significant Issue in Surgical Planning
**_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
**Pulmonary Atresia with Intact Ventricular Septum**
CORONARY PEFUSION
How often are RV sinusoids seen?
## Footnote
RV sinusoids are seen in 50% of cases
53
**Pulmonary Atresia with Intact Ventricular Septum**
_CORONARY PEFUSION_
Frequency of RV sinusoids ?
Upon what is their devellopment dependent?
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
Pulmonary Atresia with Intact Ventricular Septum
Frequency of RV Dependent coronary Circulation?
Frequency: 20% of cases
55
Pulmonary atresia with intact ventricular septum
Risk factors for Repair
Small Diameter Tricuspid valve
RV dependent myocardial blood flow
56
**Pulmonary Atresia with Intact Ventricular Septum**
_Surgical management:_
Risk factors for surgery?
**Pulmonary Atresia with Intact Ventricular Septum**
_Surgical management-_*Risk factors for surgery:*
1. Small diameter TV
2. RV dependent blood flow
###
57
_Surgical management_ of **Pulmonary Atresia with Intact Ventricular Septum**
Goals of Surgery?
_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
_Surgical management_ of **Pulmonary Atresia with Intact Ventricular Septum**
Upon what is the Treatment Strategy based?
1. z score
2. right heart morphology
3. coronary circulation
59
Surgical management of Pulmonary Atresia with Intact Ventricular Septum for neonate with
Z score 0 to -2?
_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
_Surgical management_ of **Pulmonary *Atresia* with Intact Ventricular Septum** for neonate with Z score -2 to -3?
mild-mod TV hypoplasia
Relief of the RVOTO + Shunt
2 ventricle repair if adequate RV/TV
Single ventricle if inadequate growth
61
Surgical management of Pulmonary Atresia with Intact Ventricular Septum for neonate with Z score Z score ?
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
Vascular ring - general definition
_Vascular ring:_ abnormality of the aortic arch that results in either complete or partial encirclement of the trachea or the esophagus
63
Vascular Sling: general defintion
_Vascular Sling:_ Left PA originates from the Right PA and passes between the trachea and esophagus
64
Double aortic arch - which arch is dominant? how often?
* Right Arch: Larger in 50%
* Left Arch: Larger in 25%
* The arches are balanced in 25%
65
Varriants of right sided aortic arch?
which one is a complete vascular ring?
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
What conditions are associated with *right aortic arch with mirror image branching*?
1. Right Arch (Mirror Image Branching)
1. Commonly associated with TOF and truncus
67
Varities of **incomplete vascular rings**
**_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
Surgical Repair of a PA Sling
_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
General definition of Truncus ateriosus
_Failure of development_ of the **aortocopulmonary and infundibular septums**
Therefore a **single arterial vesse**l from the heart _overriding the ventricular septum_ giving rise to the:
* systemic
* coronary
* pulmonary circulation
There is always a VSD
70
side preference of the tuncus in TA?
_Truncal artery_ –
Overrides the ventricular septum in 50%
Overides the right in 40%
Left in 10%
71
_Truncal valve in truncus arteriosus_
what is the structure of the leaflets?
% deviation from tricuspid?
Truncal valve Leafelets in truncus arteriosus
* * Leaflet number is variable
* 65% are tricuspid
* 25% are quadracuspid
* balance are bicuspid or five leaflets
72
What is the _annular structure_ in the **Truncal valve** of **truncus arteriosus**?
Annular structure
Fiberous continuity between the posterior leaflets and the anterior leaflet of the mitral valve as with the normal heart.
73
_frequency_ of *physiologic dysfunction* in the **Truncal valve in truncus arteriosus**?
Function:
50% have truncal insufficiency
Severe myxomatous changes are associated with truncal valve insufficiency
74
Describe the _coronaries_ of **truncus arteriossus**
_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
Describe the **VSD** of *Truncus arteriosus* ?
What does this mean for the ventricular structures?
**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
Abrnomalities associated with Truncus Arteriosus
**Associated Abrnomaities**
IAA (B) or coarcation 10-20%
Right aortic arch 25-30%
Persistent left superior vc 10%
Chromosome 22q11 deleton
DeGeorge Syndrome
77
truncus
what percent of CHD ?
**Natural History**
* 2.8% of CHD
*
78
Natural history of truncus arteriosus
- % die within first month
- % die within frist year
**Natural History**
* 50% die in first month
* 88% die in the first year
79
what is the most common kind of reoperation for Truncus
Reoperation for the RV to PA Conduit
80
Presentaion of child with pulmonary atreasia with IVS
* **Well developed babies**
* **Early and rapid cyanosis**
* **Absent P2**
* **CXR clear oligemic lung fields**
* **EKG normal or absence of RVH**
* **Echo diagnostic**
81
Congenital aortic stenosis
valve gradient considered severe
* * _Severe stenosis_ – Gradient \> 75mmHg
82
Presentation of neonatal aortic stenosis
* Neonate/Infant:
* Most severe valve deformity
* Severe stenosis – Gradient \> 75mmHg
* Reduced Ventricular Function
* Needs to be differentiated from HLHS
*
83
Supravalvular aortic stenosis
Associated lesions
1. Subvalvular LVOTO
2. Multiple peripheral pulmonary artery stenosis
3. Stenosis of major systemic arteries
4. Familial form
5. Williams Syndrome
84
What is williams syndrome
- Devellopmental delay, elefin facies, hypercalcemia
- Elastin gene mutation on chromosome 7q11.23
- Sudden death at a young age is common
85
Surgery for congenital supravalvular aortic stenosis
frequency of reoperation
Many patients (25-30%) require operation in 10-20 years.
86
**Sinus of Valsalva Aneurysm**
Morphology
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
**Sinus of Valsalva Aneurysm**
Associated Pathology
* 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
**Sinus of Valsalva Aneurysm**
Where do the fistulas most commonly form?
The “windsock” may rupture --\> generating a fistula most commonly into the RV
89
**Sinus of Valslava Aneurysm**
VSD: frequency associated
30-50%
90
**Sinus of Valslava Aneurysm**
VSD -
* frequency ?
* Morphology?
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
**Sinus of valsalva aneurysm**
Related aortic valve issues
Prolapse of the leaflet with with the VSD
Bicuspid valve
92
**Sinus of Valslava Aneurysm**
Nature of pulmonic stenosis
Pulmonic stenosis
Usually subvalvular related to the windsock in the RVOT
93
**Stenosis of the Aortic Valve**
Indications for surgery in infancy
* Gradient \> 75mmHg
* Depressed ventricular function
* Symptoms of ischemia or syncope
*
94
**congenital stenotic aortic valve**
factors to consider with patient presenting as an older infant or child:
* *With moderate stenosis in older infants or children*:
* Sudden death is rare if the gradient \< 75mmHg
* Valve replacement is inevitable
95
Congenital aortic valve stenosis
options for intervention:
Neonate/infant:
Child:
Adult/Elderly:
Neonate/infant: BAV/Valvotomy for survival
Child: BAV/Valvotomy for palliation
Adult/Elderly: AVR
96
varriations of subvalvular aortic stenosis
1. Localized form (Membranous- Fibrous)
2. Diffuse form (Tunnel, Muscular HOCM)
97
Key features of the *_localized_* form of **subvalvular aortic stenosis**
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
Key features of the diffuse form subvalvular aortic stenosis
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
Most severe form of diffuse subvalvular aortic stenosis
1. _Most severe form_ is the long tunnel and small aortic annulus
100
Morphology of the aortic valve in the setting of subvalvular aortic stenosis
1. Aortic valve is usually tricuspid and normal or with thickened leaflets
101
**_Tetralogy of Fallot w Pulmonary Stenosis_**
Risk factors for early post opreative death :
* Impact of age?
Increased risk if:
**_Age_**: **\< 1-3 months** or **\> 4 years**
102
**Tetralogy of Fallot w Pulmonary Stenosis**
_Risk factors for early post opreative death_ :
RV/LV pressure ratio: ?
_RV/LV pressure rati_o: \> 0.7 – in the OR or 24 hrs post op
103
**Tetralogy of Fallot w Pulmonary Stenosis**
_Risk factors for early post opreative death :_
Structural considerations (4)?
**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
**Tetralogy of Fallot w Pulmonary Stenosis**
_Risk factors for early post opreative death :_
Comorbidities and history impacting ?
**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
