1
Q
Murmur of a patient with a VSD
A
Murmur: precordial – pansystolic / hyperactive
2
Q
Anatomy of a PFO
A
septum primum lies to the left of the PFO (ostium secundum) - fails to fuse with the sides.
3
Q
surgical correction of PDA in preterm infants
phrenic nerve injury
A
More common than full-term infants (4%)
opposite side injury common
(not surgical damage)
4
Q
Perimembranous
Frequency: ?
A
Perimembranous
Frequency: most common (80%)
5
Q
Repair of coarctiaion of the aorta - current mortality for isolated lesions
A
2-10%
6
Q
Neonatal Presentation of aortic coarc ?
A
Heart failure after a variable time of being well
Once PDA closure –> Heart failure; this is related to the amount of collaterals which have developed
Symptoms:
- tachypnea
- feeding difficulties
- acidosis
- bp miss match with decreased or absent femoral pulses
7
Q
HLHS - frequency of concominant coarctation ?
A
80%
8
Q
Outcomes (In hospital mortality) for type B interrupted aortic arch ?
A
Outcomes (In hospital mortality) for type B interrupted aortic arch
Type B: 11%
9
Q
The timing of the repair of an aortic coarctation?
A
- Neonate with severe failure: operation at the time of diagnosis
- If NO cardiac failure or FTT: 3-6 months
Issues with timing of operation:
a) Increased re-stenosis if operated before 3 months
b) Persistent HTN if operated on after infancy
10
Q
PDA in preterm infants
indications for surgical intervention
A
Indications for surgical correction of a PDA in a preterm infant
- Respiratory Distress
- Large PDA
- Failure of two courses of inomethacin
- NEC (cant give NSAID)
- Intracrainial hemorrhage
11
Q
Interrupted aortic arch is ?% of all CHD
A
1%
12
Q
Hybrid approach for HLHS
A
the hybrid procedure for hypoplastic left heart physiology was first developed in 1993 in response to poor outcomes following the Norwood procedure.
The hybrid procedure (avoids CPB) :
- surgical placement of bilateral branch pulmonary artery bands
- placement of a stent in the ductus arteriosus
- catheter-based atrial septostomy,
13
Q
Conal septal VSD
- AKA?
- Morphology?
- Conduction system?
A
Conal septal VSD
- AKA: conalseptal, supracristal, infundibular, subpulmonary, doubly committed
- Morphology: conjoined leaflets of the aortic and pulmonary valves form a rim
- may have AI in up to 50% of the cases
- Conduction system: remote to the defect
14
Q
Factors which promote the closure of a VSD
A
those VSD that are muscular or juxtatricuspid are most likely to close spontaneously
Adherence to the tricuspid leaflet and chordal tissue is an important mechanism for perimembranous VSD
- Can also result in a LV-RA shunting – Gerbode Defect
.
15
Q
Outcomes (In hospital mortality) for Type C interrupted aortic arch
A
Outcomes (In hospital mortality) for type C interrupted aortic arch
Type C: highly lethal
16
Q
Unrestricted VSD
A
No resistance to flow across the VSD
the LV and RV pressures become equal
the Qp/Qs become Equal
17
Q
Type B interrupted aortic arch - % of cases and anatomic description?
A
A. Classification:
- Type A: (40%) – distal to the left subclavian
- Type B: (55%) – between L common carotid and L subclavian
- Type C: (5%) – between the innominate and L common carotid
18
Q
In what type of patient is the common atrium usually found
A
Heterotaxy
19
Q
Cardiac Anomalies associated with AVSD ?
A
Associated Cardiac Anomalies
- PDA: 10%
- TET : 10%
- 1% of TET have AVSD
- DORV: 2%
- TGA – Rare
- Unroofed coronary siunus – 3%
- Downs Syndrome
- 45% of Down’s patients have CHD
- 45% of the Down’s CHD cases are AVSD
- 75% complete AVSD
- Partial VSD is rare
20
Q
Anatomic definition of the PDA
A
Connection between the upper descending aorta and the left PA
21
Q
Partial AV septal defect
A
- Formerly known as an Ostium primum defect
- Usually associated with a cleft in the anterior leaflet of the mitral valve
22
Q
PDA in preterm infants
What is the frequency of PDA in babies < 1000g?
A
83%
23
Q
PDA in preterm infants
What is the frequency of a PDA in infants born at < 30 weeks?
A
75%
24
Q
HLHS - most common
A
Most common is combined aortic and mitral atresia (2/3)
25
**Perimembranous**
_Conduction system location:_ ?
**Perimembranous**
_Conduction system location:_ is on the posterior rim
26
Septum primum
Thin flap
27
Percutaneous closure of PDA in infants
Indications and contraindications
Percutaneous closure
* Contraindicated in patients \< 6kg
* best in a PDA 1.5mm or smaller
28
**Malalignment** Type type of **VSD** - 3 varities ?
**Malalignment Type**
a) _Anterior_: tetralogy of Fallot
b) _Posterior_: interrupted arch or coarct-VSD
c) _Rotational_: Taussig-Bing
29
*Problems* with the **Pott's shunt**
1. L PA _aneurysm_ formation
2. Risk of _stroke_ with shunt take down
3. *Excessive* _pulmonary flo_w
30
*Embryologic* and *anatomic* significance of the _ductus_ in in the devellopment of **coarc of the aorta ?**
1. **Ductus** --\> _highly muscular_
1. In the *normal* aorta \< 30% of normal circumference
2. 95% of pathology specimines is comprises the circumference
3. Aorta proximal to the coarc is abnormal
2. *Normal* fetus has _decreased blood flow_ across the **aortic isthmus**
1. _Increased_ blood flow across in coarctatioin
31
Rate of early, non-facial shunt closure
7%
32
Type C interrupted aortic arch - % of cases, anatomic description ?
Classification:
1. Type A: (40%) – distal to the left subclavian
2. Type B: (55%) – between L common carotid and L subclavian
3. **Type C**: (5%) – between the innominate and L common carotid
33
Most common ASD
_Most common_: ostium secundum (fossa ovalis)
34
surgical correcton of PDA in preterminfant
long term survival
1-5 year survival is ~50%
typically due to unrelated conditions
35
what % of Coarctation are Isolated lesion ?
what % of Coarctation are Isolated lesion: **82%**
36
Coarctation of the aorta - associated abnormalities
* Isolated lesion: 82%
* VSD: 11%
* other (HLHS): 8%
* distal aortic arch narrowing: 50-60%
* bicuspid aortic valve: 27-46%
37
**Downs Syndrome**
what % of Down’s patients have CHD?
what % of the Down’s CHD cases are AVSD?
_Downs Syndrome_
45% of Down’s patients have CHD
45% of the Down’s CHD cases are AVSD
38
How much of the CO is delivered through a **PDA**
55%
39
Clinical presentation of children with a VSD
Clinical Symptoms:
(1) Heart failure: poor feeding, growth failure, tachypnea
(2) If PVR \> SVR à Cyanosis
(3) Murmur: precordial – pansystolic / hyperactive
40
Medical Support for HLHS
1. **PGE-1** - to maintain ductal patency
2. Keep **HCT** _45-50%_
3. Keep **O2 sat** between _70-75%_
* FiO2 only should be 0.18 to 0.21
4. Maintain **PCO2** 40-50
5. **Do not perform** _atrial septostomy preop_
41
what % of Coarctation are associated with VSD ?
what % of Coarctation are associated with VSD : **11%**
42
?% of TET have AVSD?
1% of TET have AVSD
43
**Sinus Venosus Syndrome** in general terms.
**_Sinus Venosus Syndrome:_** _Right upper_ and _middle pulmonary veins_ attach to the *low* _SVC or the SVC/RA junction_
44
Overall classification of **interrupted aortic arch**
Classification:
* Type A: (40%) – distal to the left subclavian
* Type B: (55%) – between L common carotid and L subclavian
* Type C: (5%) – between the innominate and L common carotid
45
**1st and 2nd** _most common_ types of **VSD**
**Perimembranous** – most common (80%)
**Doubly committed subarterial** (10% -second most common)
46
Frequency of **first degree AV block** after repair of **AVSD**
Up to 50% of patients will have first degree AV block after repair
47
**Perimembranous VSD**
* Frequency?
* Borders ?
* Conduction System Location?
**Perimembranous VSD**
* _Frequency_: most common (80%)
* _Borders_: the tricuspid valve
* _Conduction system:_ is on the posterior rim
48
**Interrupted Aortic Arch**
Initial _medical_ management?
**Interrupted Aortic Arch**
Initial _medical_ management?
* PGE-1 (keep duct open)
* Oxygenation
* Pressors
49
what % of Coarctation are associated with bicuspid aortic valve?
27-46%
50
Repair of Sinus Venosus Syndrome
Sinsus venosus
* Path*: Right upper pulmonary vein is shunted to the Right atrium
* Repair* : Patch shunting pulmonary vein flow to the left atrium
51
_Aortic valve morphology_ associated with **interrupted aortic arch**
Aortic valve: bicuspid in: 30-59%
52
Indictions For palliative AP shunt
Decreased pulmonary Q and cyanosis SaO2.
53
Factors which increase the risk of mortality with the repair of aortic coarctation ?
Repair of coarctation of the aorta --\> Increased mortality if
* distal arch hypoplasia
* increased mortality and recurrence if \< 3months
54
Coarctatioin of the aorta
Epidemiology - gender association ?
**Coarctatioin of the aorta**
1. Gender:
1. _Isolated_: Male / Female: **2:1**
2. _Coexisting_: Male/Female: **1:1**
55
Embryologic derrivation of the PDA
6th aortic arch
56
Classic BT shunt - technic
Direct anastomosis of the subclavian artery to the PA
57
What is the problem with a waterson shunt
uncontrolled, may lead to pulmonary overflow and pulmonary HTN
58
what % of Coarctation are associated with (HLHS) ?
what % of Coarctation are associated with HLHS: **8%**
59
Stages of PDA closure
**_First stage_**
1. _complete_ **10-15 horus**
2. _smooth muscle_ **contraction**
3. intimal cushion apposition
**_Second stage_**
1. _Complete_ at **2-3 weeks**
2. _Fiberous poliferation_ of the **intima**
3. _Necrosis_ of the inner layer of the **media**
4. _Hemorrhage_ into the **wall**
\*\*\* ocurs from *PA to aorta* --\> residual PDA on the aorta --\> **Kommerell's Diverticulum**
60
**PDA** is associated with \_\_?\_\_ **prenatal infection**?
*_Rubella_* in the **first trimester**
61
what % of AVSD have an Unroofed coronary siunus?
Unroofed coronary siunus – 3%
62
_surgical correction of **PDA** in preterm infants_
What is the in-hospital mortality?
10-20%
63
**Interrupted Aortic Arch**
typical anatomy of the ascending aorta and subsequent branches
Interrupted aortic arch
Ascending aorta: about ½ normal diameter and divides into two --\> Branches nl size
but .... the Brachiocephalic vessels: Commonly anomalous origins
64
Effect of aneurysm of the membranous septum on a VSD
Aneurysm of the membranous septum functional _reduces the size of the VSD_ but _promotes tricuspid regurgitation_ and is a _nidus for endocarditis_
65
% of AVSD have DORV?
DORV: 2%
66
Sequale of **Eisenmenger Syndrome**
in untreated patients:
increased PVR --\> high right side pressure --\> shunt reversal & Cyanosis
tends to occur in the 3rd and 4th decades of life
***_Sequale_***:
1. Fatal hemotypsis
2. Polycythemia
3. Cerebral abscess or infarction
4. RV failure
67
Most common cause of death \< 1 month of age
HLHS
68
Potts Shunt
_*Descending* aorta_ to the _Left PA_
Basically a **_P_**otts is a **_P_**FO
69
ASD /TAPVR Contraindications for Surgery
ASD /TAPVR Contraindications for Surgery
1. PVR \> 7 wu
70
Types of **Palliative Operations** - general categories
**_Palliative Operations:_**
1. Increase pulmonary blood flow (eg: AP shunt)
2. Decrease pulmonary Q (eg: PA band)
3. Improve mixing
4. Reduce ventricular work
71
% AVSD asspciated with a PDA?
PDA: 10%
72
Indication for surgical repair of a VSD
1. Large VSD
* \< 3 months - chf / Qp/Qs \> 2.1
* Elective repair at 6 months if PVR \< 8.0 wu
2. Small VSD (QP/Qs\<2.1)
* Endocarditis
* Cardiac enlargement
* Any AI
3. Subarterial VSD - any size
73
*Surgery* for **Interrupted Aortic Arch ?**
* Timing
* Surgical Technic
* Surgery* for **Interrupted Aortic Arch ?**
* _Timing_
1. If in good condition operate at the first convenient time
2. If Unsuitable for surgery – pressors, treat acidosis, and increase the CO2
* _Surgical Technic:_
1. Palliation vs 1 stage correction
74
% of AVSD associated with TET?
TET : 10%
75
Most important risk factor for death after shunting procedure
pulmonary arterial problems
76
Conduction system in AVSD
* **Coronary sinus** is displaced inferior
* **AV node** displaced inferior
* Located between the coronary ostium and the ventricular crest
* **Bundle of His** _courses along the top of the **ventricular septum**_
* Becomes the **right bundle** at the **midpoint of the crest**
* RBB common after complete repair
1. _Up to 50% of patients will have first degree AV block after repair_
77
Disadvantage of the BT shunt
sacrifices a sublcaivan artery
78
Unroofed Coronary Sinus Syndrome
4 forms:
_Unroofed Coronary Sinus Syndrome_
4 forms:
1. Completely unroofed with persistent LSVC
2. Completely unroofed without persistent LSVC
3. Partially unroofed mid portion
4. Partially unroofed terminal portion
79
Epidemiology of Ventricular septal defects
Epidemiology:
Most common congenital heart defect
1/100 Births –
25% of all CHD
80
Risks if a VSD does not spontaneously close and is left untreated:
*Infancy*:
_Small or moderate VSD:_ Endocarditis is a rare, but more common with the small or moderate
_Large_: 9% mortality at 1 year if left untreated
_PVR increases with age_:
* AI in the first decade
* Eisenmenger’s syndrome \> 20years old (typically)
* Infundibular pulmonary stenosis (5%)
81
*_Indications_* of the *classic* **BT shunt**
Increases pulmonary artery blood flow in a controlled fashion - most common:
1. TOF
2. Pulmonary atresia
3. Tricuspid atresia
82
**Type A** _interrrupted aortic arch_ - % of cases, and anatomical description
1. **Type A: (40%)** – distal to the left subclavian
2. Type B: (55%) – between L common carotid and L subclavian
3. Type C: (5%) – between the innominate and L common carotid
83
what % of all CHD have a PDA?
5-10%
of all patients with CHD have a PDA
84
Valve regurgitation and the presentation of an AVSD
A. AV Valve regurgitation – worsens the course and severity
a) Moderate – 20%
b) Severe – 15%
85
PDA epidemiology -
M:F ratio
PDA is 2x as common in females
| (think "p" aka concha- DA)
86
Coarcation of the aorta
% narrowing needed for hemodynamic significant narrowing
50%
87
Determinants of the clinical presentation of a PDA
The Magnitude of the shunt, which is determined by:
1. size of the PDA
2. PVR
88
ASD /TAPVR **optimal age** for Surgery ?
Age – optimal at 1-2 years
89
Pulmonary trunk in patients with an interrupted aortic arch
Pulmonary trunk : HUGE
90
**Trussler's Rule**
**Trusler's rule** for the _circumference of a PA Band_
* 20mm + 1mm/kg body weight (non mix VSD)
* 22mm + 1mm/kg bw (pre-fontan)
* 24mm + 1mm/kg bw (TGA w/ VSD)
91
what % of Coarctation are associated with distal aortic arch narrowing?
50-60%
92
Timing of the repair of a coarc aorta in infant with failure?
1. **Neonate with severe failure** – operation a*t the time of diagnosis*
2. If NO cardiac failure or FTT à 3-6 months
_Issues with timing of operation:_
a) Increased re-stenosis if operated before 3 months
b) Persistent HTN if operated on after infancy
93
Anomalies associated with scimitar syndrome
* Lung hypoplasia
* Anomalous arterial supply via the inferior pulmonary ligament
* Pulmonary hypertension
* **20%** are associated with cardiac and diaphram abnormalities
94
Effect of _prostaglandins_ and _indomethacin_ on **PDA**
PGE1 / PGE2 --\> promote opening
Indomethacin ---\>encourage closure
95
**Perimembranous**
_Borders_: ?
**Perimembranous**
_Borders_: the tricuspid valve
96
PVR where VSD repair is prohibitive
\> 8
97
Outcomes (In hospital mortality) **for type A interrupted aortic arch**
1. Type A:
Outcomes (In hospital mortality) **for type A interrupted aortic arch**
Type A: 4%
98
**Interrupted Aortic Arch**
Clinical Presentation?
**Interrupted Aortic Arch**
_Presentation_ - keep in mind the distal perfusion is highly duct dependent
1. Critically ill patient in severe heart failure
2. A left to right shunt, with high afterload following closure of the ductus
3. Decreased pulses, acidosis, and anurea
99
_Symptoms_ of **Coarctaion of the Aorta** in *Childhood* (1-14yo)?
_Childhood (1-14)_
1. HTN – 90% "Hypertension in a 5 year old is never normal "
2. Cardiomegaly (33%)
3. Rib notching 15% (\>3 years)
4. LVH on EKG
100
Posterior Malalignment Type VSD
Posterior: interrupted arch or coarct-VSD
101
what % of AVSD have TGA?
TGA – Rare
102
**Interrupted Aortic Arch** - In hospital _Mortality_ for:
Type A:
Type B:
Type C:
Outcomes (mortality)
1. Type A: 4%
2. Type B: 11%
3. Type C: highly lethal
103
coarctation of the aorta
1. Epidemiology
1. % of all CHD?
1. Coarctation is 6.5% of all CHD
104
Rate of closure of a VSD
a) 80% at 1 month
b) 60% at 3 months
c) 25% at one year
105
% of PDA that are typically closed by 8 weeks
88% are closed
106
ASD /TAPVR Indications for Surgery
ASD /TAPVR Indications for Surgery
* Qp/Qs \>= 2
* Scimitar
* PAPVR
* Part of one lung \< 1.8 No
* Whole lung – yes
* Age – optimal at 1-2 years
* TVD/MVD
107
Anterior Malalignment Type VSD
Malalignment Type
a) Anterior: tetralogy of Fallot
108
Waterson shunt
Ascending aorta to right PA
109
Rotational malalignment VSD
Rotational: Taussig-Bing
110
111
Septum secundum
Thick round superior rim of in folded atrial roof
112
Salient features of the morphology of an **interrupted aortic arch**
1. Aortic valve: bicuspid in 30-59%
2. Ascending aorta: about ½ normal diameter and divides into two à Branches nl size
3. Brachiocephalic vessels: Commonly anomalous origins
4. Descending Aorta: is a continuation of the ductus
5. Pulmonary trunk : HUGE
6. Large VSD is always present
