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Peds - Exam 4 > Topic 14: TOF DORV DILV > Flashcards

Topic 14: TOF DORV DILV Flashcards

1
Q

most common cause of blue baby syndrome ?

A

TOF

10 % of all cyanotic heart defects,

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2
Q

Tetralogy of Fallot involves four heart defects:

A 
(3 are congenital, 1 is acquired)
A large VSD
Pulmonary stenosis (RVOT obstruction)
An overriding aorta
Right ventricular hypertrophy (RVH)
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3
Q

Simply Put TOF is what?

A

anterior-lateral displacement of the infundibular septum

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4
Q

TOF Embryology – how are Aorta and PA formed?

A

Aorta and PA start as a single tube (truncus arterosis) divided by the spiral septum
The spiral septum grows down and attaches to the ventricular septum which:
◦Isolates the ventricles
◦Isolates the aorta and the pulmonary artery

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5
Q

Partitioning of the outflow tract

Truncus Arteriosus becomes?

A

–Aorta

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6
Q

Partioning the Outflow tract

Conus Cordis becomes?

A

–Pulmonary Artery

◦Created by a septum that forms in the outflow tract from these swellings

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7
Q

What if ?
The spiral septum is not midline, but shifted towards the right side of the heart.
This would cause:

A

◦The aorta opening to be large
◦pulmonary opening to be small
◦Spiral septum would miss the septum

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8
Q

What if ?
The spiral septum is not midline, but shifted towards the left side of the heart.
This would cause:

A

◦The aorta opening to be small
◦pulmonary opening to be large
◦Spiral septum would miss the septum

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9
Q

Factors that increase the risk for TOF during pregnancy include:

A
  • Alcoholism in the mother
  • Diabetes
  • Mother> 40 years old
  • Poor nutrition during pregnancy
  • Rubella or other viral illnesses during pregnancy
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10
Q

TOF w/Pulmonary Atresia aka? and what is it?

A

TOF with PA (pseudotruncus arteriosus) is a severe variant in which there is complete obstruction (atresia) of the right ventricular outflow tract (RVOT), causing an absence of the pulmonary trunk during embryonic development

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11
Q

TOF with PA - how does blood shunt? and how are the lungs perfused?

A

R → L shunt is significantly worse than norm.
In these individuals, blood shunts completely
from the right ventricle to the left where it
is pumped only through the aorta.
The lungs are perfused via extensive collaterals from the systemic arteries, and sometimes also via the ductus arteriosus

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12
Q

TOF w/PA w/an ASD is aka a ?

A

Pentology of Fallot

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13
Q

TOF w/PA w/an ASD is aka a ?

A

Pentology of Fallot

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14
Q

TOF blood shunting ?

A

results in low oxygenation of blood due to the mixing of blood in the LV via the VSD and preferential flow of the mixed blood through the aorta
significantly worse with TOF w/PA

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15
Q

What might be the bodies response to the low saturations and decreased pulmonary blood flow?

A

A: Elevate the hematocrit, it is not uncommon for these children to have hcts >50 %

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16
Q

TOF symptoms?

A

The primary symptom is low blood oxygen
saturation with or without cyanosis
If the baby is not cyanotic then it is sometimes
referred to as a “pink tet”
Periods of severe hypoxic spells i.e. “tet spells”
Clubbing of fingers (skin or bone enlargement
around the fingernails)
Difficult feeding/failure to gain weight
Passing out
Poor development
Squatting during episodes of cyanosis

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17
Q

Children having a TET spell may have several symptoms:

A 
•Rapid, deep breathing.
•Fainting/loss of consciousness.
•Increasing blueness (cyanosis) of the lips,
tongue and nailbeds
•Irritability or uncontrolled crying
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18
Q

Dealing with Tet Spells: what a child will do?

A

Squatting and the knee chest position will increase aortic wave reflection,
increasing pressure on the left side of the heart, decreasing the right to left shunt (think clamping distal to outflow)
◦thus decreasing the amount of deoxygenated
blood entering the systemic circulation

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19
Q

Palliation of TOF procedures

A

B-T Shunt
Modified BT shunt
Central Shunt

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20
Q

When are BT shunts used on infants with TOF?

A

Currently, Blalock-Thomas-Taussig shunts are not normally performed on infants with TOF except for severe variants such as TOF with pulmonary atresia (pseudotruncus arteriosus).

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21
Q

Central Shunt

A

(ascending aorta to main pulmonary artery)

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22
Q

Central Shunt - The advantages of this technique are?

A

•Applicability to small children with small
peripheral vessels
•Prevention of distortion of pulmonary arteries
•Provision of equal pulmonary blood flow to both lungs
•Lower occlusion rate (compared with the CBTS or MBTS techniques)
•Avoidance of subclavian artery steal
•Ease of closure during corrective repair

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23
Q

Palliative shunts are done mostly with TOF patients with what?

A

TOF w/PA

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24
Q

TOF surgery – when is it done?

A

Surgery to repair TOF is done when the infant is young.
When the condition warrants, palliation is done (more often on TOF w/PA).
Corrective Surgery is preferred over palliation and is performed in the first few months of life. (Less RV hypertrophy)

25
TOF - what is the preferred treatment?
Corrective Surgery
26
TOF - risk
Total repair of TOF initially carried a high mortality risk. This risk has gone down steadily over the years. Surgery is now often carried out in infants
27
The TOF open-heart surgery is designed to do 2 things mainly?
1) Relieve the RVOT stenosis by careful resection of muscle 2) Repair the VSD with a Gore-Tex patch or a homograft patch. Additional reparative or reconstructive surgery may be done on patients as required by their particular cardiac anatomy.
28
TOF w/Absent Pulmonary Valve think of it as?
(dilated-aneurysmal)
29
TOF w/ Pulmonary Atresia
(hypoplasia)
30
W/TOF what will need to be placed do the large VSD patch ?
Pacing wires will be needed
31
TOF Incision? what will is disrupt?
Intracardiac incision: will be a right atriotomy or right ventriculotomy-(depending on the severity RVOT obstruction) ◦ This will disrupt the conduction system
32
``` CPB Considerations for TOF - Incision? Arterial Cannula? Venous Cannula? Temp? CPG? ```
``` Incision: Median sternotomy Arterial: Aortic Venous: Bicaval Hypothermia: Mild to Moderate Cardioplegia: Antegrade (multiple doses due to Ao-pulmonary collateral circulation) ```
33
Special note about CPG and TOF CPB?
The heart warms quickly-be prepared for LOTS of CPG | Your RV was stressed pre-op be careful with it
34
TOF and CPB - what type of drugs may need to be given and why? and what is a possibility of needing?
May need ionotropes to keep BP ↑ CVP and RV pressures may stay high post-op for a while Due to RV dysfunction ECMO is a possibility
35
TOF - be careful of what type of drugs?
Be careful of vasodilators post-op since low BP can worsen RV dysfunction
36
TOF what to do with HCTs on CPB?
Make sure you know how you are going to deal with high hematocrits before going on CPB (pull off volume) Be careful with MUF since it will be easy to get the hct at 50%+ (most likely will add volume pulled off when your warming)
37
Double Outlet Right Ventricle what is it?
The Aorta and Pulmonary Artery both originate from the RV and blood from the LV passes across a VSD into the RV to reach the great arteries. Pulmonary circulation is very high pressure with increased blood flow (as with a large VSD) The anatomic dysmorphology of double outlet right ventricle can vary from that of TOF on one end of the spectrum to TGA on the other end
38
The majority of DORV cases occur with?
pulmonary stenosis and VSD, behaving as a severely cyanotic Tetralogy of Fallot
39
DORV are classified how?
Classified according to the VSD location One of the ways that DORV is diagnosed is by the location of the VSD
40
DORV symptoms?
* Baby tires easily, especially when feeding * Bluish skin color (the lips may also be blue) * Clubbing (thickening of the nail beds) on toes and fingers * Failure to gain weight and grow * Peripheral edema * Dyspnea
41
Complications of DORV may include what?
•Congestive heart failure (CHF) •Pulmonary hypertension •Irreversible damage to the lungs due to untreated high blood pressure in the lungs
42
DORV types?
Sub-aortic VSD Sub-pulmonary VSD (Tausigg-Bing) Doubly committed VSD Non-committed VSD
43
Sub-aortic DORV
This variant is most common. Pathophysiology depends on the degree of PS. With PS, the pulmonary blood flow is decreased with variable cyanosis like a TOF. In the absence of PS, the pulmonary blood flow is increased, resulting in heart failure like a VSD
44
Most common DORV?
Sub aortic
45
Sub-pulmonary VSD (Tausigg-Bing)
The PA preferentially receives LV oxygenated blood Desaturated blood from the RV streams to the aorta like with TGA This Taussig-Bing anomaly is a typical example of DORV with sub-pulmonary VSD. Similar to TGA
46
Doubly Committed DORV
The infundibular septum is absent leaving both aortic and pulmonary valves related to the VSD. Clinical features depend on the presence or absence of pulmonary stenosis.
47
Non-committed DORV
The non-committed VSD is remote from the aortic and pulmonary valves. Most patients with non-committed VSD undergo single ventricular w/palliative strategies Note univentricular heart appearance
48
Two basic types of surgical repair for DORV?
ANATOMIC repair, which restores a circulation with two ventricles UNIVENTRICULAR repair, in which only one ventricle is functional.
49
Surgery: DORV with Sub -aortic VSD | - what? age? risk
``` Intra-ventricular tunnel (LV → VSD → Ao) Low risk Age 6 months Rastelli procedure for PS ```
50
Intra-ventricular Tunnel is what?
Channels (tunnel/patch )LV blood through the VSD to the aorta (LV → VSD → Aorta) Uses of a patch (polytetrafluoroethylene [PTFE]) that corresponds to the circumference of the aorta. FOR - DORV with Sub -aortic VSD
51
DORV with Sub-pulmonary VSD (Taussig-Bing Heart) procedure?
Complex intra-ventricular tunnel to Ao or PA With infundibular resection Close VSD to PA plus arterial switch procedure
52
DORV: Taussig-Bing?
Intra-ventricular tunnel Close VSD to PA Do Arterial Switch
53
Surgery: DORV with Doubly Committed VSD
Intra-ventricular tunnel (LV → VSD → Aorta) PS or obstruction of the RVOT due to the tunnel may necessitate the creation of a right ventricle outflow patch or even a Rastelli. The VSD, which is typically large, usually does not create difficulty in channeling left ventricular blood to the aorta with an intra-ventricular tunnel.
54
Surgery: DORV with non -committed VSD?
Appears as univentricular heart Most difficult to correct univentricular repair Complex intra-ventricular tunnel to Ao or PA patch/baffle May use of combined atrial and ventricular approaches Fontan procedure ultimately
55
``` CPB with DORV Surgical Considerations? Incision? Arterial Cann? Venous Cann? Temp? CPG? ```
Incision: Median sternotomy Cannulation: Arterial: Aortic Venous: Bicaval (except with univentricular repair) Hypothermia: Mild to Moderate Cardioplegia: Antegrade (multiple doses due to Ao-pulmonary collateralcirculation)
56
``` CPB with DORV Surgical Considerations? Incision? Arterial Cann? Venous Cann? Temp? CPG? ```
Incision: Median sternotomy Cannulation: Arterial: Aortic Venous: Bicaval (except with univentricular repair) Hypothermia: Mild to Moderate Cardioplegia: Antegrade (multiple doses due to Ao-pulmonary collateral circulation)
57
DORV CPB cases - Time? what may happen after?
These cases are amazingly variable in length, severity, and can be difficult post-op in pressure regulation. Will resemble pump runs for TOF Univentricular repairs will be of the Fontan procedure nature Depending on pre-op lung damage and pulmonary hypertension –ECMO again may be warranted
58
DORV - HCTs??
Make sure you know how you are going to deal with high hematocrits in severe cyanotic conditions (pull off volume) Be careful with MUF since it will be easy to get the hct at 50%+ (most likely will add volume pulled off when your warming)

Peds - Exam 4 (5 decks)

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