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Congenital Defects & Pediatrics IA > TOF, DILV, DORV, Damus-Kaye-Stansel- Topic 14 > Flashcards

TOF, DILV, DORV, Damus-Kaye-Stansel- Topic 14 Flashcards

1
Q

What percent of all cyanotic heart defects are TOF?

A

10%

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2
Q

TOF is the most common cause of _________ (2) syndrome.

A

Blue baby

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3
Q

TOF has a very high association with what condition?

A

Down’s Syndrome

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4
Q

TOF involves 4 heart defects. ___ are congenital; ___ are acquired.

A

3, 1

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5
Q

What are the 4 TOF defects?

A

A large VSD
Pulmonary stenosis (RVOT obstruction)
An overriding aorta
RVH

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6
Q

Simply put, TOF is ……

A

an anterior-lateral displacement of the infundibular septum

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7
Q

Simply put, TOF is ……

A

an anterior-lateral displacement of the infundibular septum

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8
Q

Aorta and PA start as a single tube called what?

A

Truncus arteriosus

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9
Q

Aorta and PA start as a single tube divided by what? What direction does it grow?

A

Spiral septum; grows down and attaches to the ventricular septum which isolates the ventricles and isolates the aorta and the pulmonary artery

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10
Q

Aorta and PA start as a single tube divided by what? What direction does it grow?

A

Spiral septum; grows down and attaches to the ventricular septum which isolates the ventricles and isolates the aorta and the pulmonary artery

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11
Q

What is outflow tract septation?

A

Partitioning of the outflow tract

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12
Q

Truncus Arteriosus becomes….

A

Aorta

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13
Q

Conus Cordis becomes….

A

Pulmonary Artery

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14
Q

What creates the conus cordis?

A

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

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15
Q

What creates the conus cordis?

A

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

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16
Q

RSTS

A

right superior truncus swelling

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17
Q

LITS

A

left inferior truncus swelling

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18
Q

RDCS

A

right dorsal conus swelling

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19
Q

LVCS

A

left ventral conus swelling

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20
Q

LVCS

A

left ventral conus swelling

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21
Q

If the spiral septum is not midline, but shifted toward the right side of the heart- What would this cause?

A

Aorta opening would be large
Pulmonary opening would be small
Spiral septum would miss the septum

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22
Q

If the spiral septum is not midline, but shifted toward the left side of the heart- What would this cause?

A

Aorta opening would be small
Pulmonary opening would be too large
Spiral septum would miss the septum

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23
Q

If the spiral septum is not midline, but shifted toward the left side of the heart- What would this cause?

A

Aorta opening would be small
Pulmonary opening would be too large
Spiral septum would miss the septum

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24
Q

What are risk factors of TOF?

A

Increased risk during pregnancy:

  • Alcoholium in the mother
  • Diabetes
  • Mother > 40 y/o
  • Poor nutrition during pregnancy
  • Rubella or other viral illnesses during pregnancy
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25
Q

What are risk factors of TOF?

A

Increased risk during pregnancy:

  • Alcoholium in the mother
  • Diabetes
  • Mother > 40 y/o
  • Poor nutrition during pregnancy
  • Rubella or other viral illnesses during pregnancy
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26
Q

TOF w/ Pulmonary Atresia AKA

A

pseudotruncus arteriosus

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27
Q

Pseudotruncus Arterosus (TOF w/ PA)

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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28
Q

How does blood shunt in TOF w/ PA?

A

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 arterosus

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29
Q

Pentalogy of Fallot

A

TOF with the addition of an ASD

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30
Q

TOF results in low oxygenation of blood due to what?

A

Mixing of blood in the LV via the VSD and preferential flow of the mixed blood through the aorta (because of the flow through the pulmonary valve)

R –> L shunt

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31
Q

What is worse in TOF w/ PA?

A

R –> L shunt is significantly worse

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32
Q

What might be the body’s response to low saturations and decreased pulmonary blood flow?

A

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

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33
Q

What are symptoms of TOF?

A 
Low blood oxygen sat w/ or w/o cyanosis
"Pink tet" - no cyanosis
Periods of severe hypxic spells ("tet spells")
Clubbing of fingers (skin or bone enlargement around the fingernails)
Difficult feeling/failure to gain weight
Passing out
Poor development
Squatting during episodes of cyanosis
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34
Q

Tet Spells

A

Rapid, deep breathing
Fainting/loss of consciousness
cyanosis of the lips tongue and nailbeds
Irritability or uncontrolled crying

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35
Q

Tet Spells

A

Rapid, deep breathing
Fainting/loss of consciousness
cyanosis of the lips tongue and nailbeds
Irritability or uncontrolled crying

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36
Q

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 amount of deoxygenated blood entering the systemic circulation

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37
Q

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 amount of deoxygenated blood entering the systemic circulation

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38
Q

Blalock Taussig Shunt

A

Formed anastomosis between the subclavian artery and the pulmonary artery

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39
Q

Palliation: Shunts Used

A

B-T Shunt

Central Shunt

40
Q

Currently, how often are Blalock Taussig shunts performed?

A

Not normally performed on infants with TOF except for severe variants such as TOF w/ pulmonary atresia (pseudotruncus arteriosus)

41
Q

Central Shunt

A

Ascending aorta to the main PA

42
Q

Advantages of Central Shunt

A

Applicability to small children with small peripheral vessels
Prevention of distortion of pulmonary arteries
Provision if 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

43
Q

When is surgery for TOF done?

A

When the infant is young; when the condition warrants, palliation is done (more often on TOF w/ PA)

44
Q

What correction is preferred over palliation?

A

Corrective surgery and is performed in the first few months of life (Less RV hypertrophy)

45
Q

Total repair of TOF Risk

A

iniitally carried high mortality risk
The risk has gone down steadily over the years
Surgey is now often carried out in infants

46
Q

The open-heart surgery for TOF is designed to:

A
  1. Relieve the RVOT stenosis by careful resection of the muscule
  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

47
Q

Two ways of TOF Repair

A

Transannular vs. Non-transannular

48
Q

Dilated-Anuerysmal TOF

A

TOF w/ absent pulmonary valve

49
Q

Hypoplasia TOF

A

TOF w/ pulmonary atresia

50
Q

Rastelli Procedure

A

RV- PA conduit

51
Q

Why would pacing wires be needed in surgically correcting TOF?

A

Due to large VSD patch

52
Q

Intracardiac incision for TOF reapir

A

will be a right atrotomy or right ventriculotomy- depending on the severity RVOT obstruction; tis will disrupt the conduction system

53
Q

CPB Considerations: incision

A

Median sternotomy

54
Q

CPB Considerations: Cannulation

A

Arterial: Aortic
Venous: Bicaval

55
Q

CPB Considerations: Hypothermia

A

Mild to moderate

56
Q

CPB Considerations: Cardioplegia

A

Antegrade (multiple doses due to Ao-pulmonary collateral circulation)

57
Q

How quickly will the heart warm during surgical repiar?

A

Quickly; be prepared for lots of cardioplegia!

58
Q

What hsould you be careful with during surgical repair?

A

Your RV was stressed pre-op

Be careful of vasodilators post-op since low BP can worsen RV dysfunction

59
Q

What will you need during surgical repair to keep the blood pressure up?

A

Ionotropes

60
Q

What pressures will stay high post-op for a while?

A

CVP and RV pressures may stay high post op for a while

61
Q

Why is ECMO a possibility after surgical repair?

A

Due to RV dysfunction

62
Q

How do you deal with high hematrocrits?

A

know this in advance

63
Q

Why should you be careful with MUF?

A

It will be easy to get the HCt at 50% + (most likely will add volume pulled off when your warming)

64
Q

Why should you be careful with MUF?

A

It will be easy to get the HCt at 50% + (most likely will add volume pulled off when your warming)

65
Q

DORV

A

Double outlet right ventricle

66
Q

What is DORV?

A

The Aorta and Pulmonary artery both originate from the RV and blood form the LV passes across a VSD into the RV ito reach the great arteries

Pulmonary circulation is very high pressure with increased blood flow (as with a large VSD)

67
Q

What is DORV?

A

The Aorta and Pulmonary artery both originate from the RV and blood form the LV passes across a VSD into the RV ito reach the great arteries

Pulmonary circulation is very high pressure with increased blood flow (as with a large VSD)

68
Q

CT

A

Conal tissue

69
Q

What is the spectrum of variation of DORV?

A

TOF on one end to TGA on the other end

70
Q

Majority of cases of DORV occur with what?

A

Pulmonary stenosis and VSD, behaving as a severely cyanotic TOF

Classified according to the VSD location
One of the ways that DORV is diagnosed is by the location of the VSD

71
Q

Majority of cases of DORV occur with what?

A

Pulmonary stenosis and VSD, behaving as a severely cyanotic TOF

Classified according to the VSD location
One of the ways that DORV is diagnosed is by the location of the VSD

72
Q

Symptoms of DORV

A

Baby tires easily, especially when feeding
Bluish skin color (lips may be blue)
Clubbing (thickening of the nail beds) on toes and fingers
Failure to gain weight and grow
peripheral edema
Dyspnea

73
Q

Complications of DORV

A

CHF
Pulmonary HTN
irreversible damage to the lungs due to untreated high blood pressure in the lungs

74
Q

Types of DORV

A

Sub-aortic VSD
Sub-pulmonary VSD (Tausigg- Bing)
Doubly Committed VSD
Non-commited VSD

75
Q

What is the most common type of DORV?

A

Sub-aortic VSD

76
Q

Sub-aortic VSD

A

pathophysiology depends on the degree of PS. With PS, the pulmonary blood flow is decreased with variable cyanosis (like TOF).
In the absence of PS, the pulmonary blood flow is increased, resulting in heart failure (like a VSD)

77
Q

Sub-Pulmonary VSD DORV AKA

A

Taussig -Bing

78
Q

Sub- Pulmonary VSD DORV

A

PA preferentially receives LV oxygenated blood
Desaturated blood from the RV streams to the aortta9 Like with TGA)
This Taussig-Bing anomaly is a typical example of DORV with sub-pulmonary VSD
Similar to TGA

79
Q

Doubly Committed VSD DORV

A

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

80
Q

Non Committed VSD DORV

A

The non-committed VSD is remote from the aortic and pulmonary valve
Most patients with non-committed VSD undergo single ventricular w/ palliative strategies

Univentricular appearance
Heart appearance

81
Q

Two basic types of repair for DORV

A

Anatomic- restores circulation w/ two ventricles

Univentricular- only one ventricle is functional

82
Q

Two basic types of repair for DORV

A

Anatomic- restores circulation w/ two ventricles

Univentricular- only one ventricle is functional

83
Q

Surgery: Subaortic VSD DORV

A

Intra-ventricular tunnel (LV–> VSD–> Ao)
Low risk
Age 6 months
Rastelli procedure for PS

84
Q

Intra-ventricular Tunnel: Subaortic VSD DORV

A

Channels (tunnel/patch) LV blood through the VSD to the oarta (LV–> VSD–> Aorta)
Uses of a patch (polytetrafluoroetylene [PTFE] that corresponds to the circumference of the aorta)

85
Q

Intra-ventricular Tunnel: Subaortic VSD DORV

A

Channels (tunnel/patch) LV blood through the VSD to the oarta (LV–> VSD–> Aorta)
Uses of a patch (polytetrafluoroetylene [PTFE] that corresponds to the circumference of the aorta)

86
Q

Surgery: Sub-pulmonary VSD (Taussig-Bing Heart) DORV

A

Complex intra-ventricular tunnel to Ao or PA
With infundibular resection
Close VSD to PA plus
Arterial switch procedure

87
Q

Surgery: Doubly Committed VSD DORV

A

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 oarta with an intra-ventricular tunnel

88
Q

Surgery: Non-Committed VSD DORV

A

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

89
Q

CPB Considerations DORV: Incision

A

Median Sternotomy

90
Q

CPB Considerations DORV: Cannulation

A

Arterial: Aortic
Venous: Bicaval (except with univentricular repair)

91
Q

CPB Considerations DORV: Hypothermia

A

Mild to Moderate

92
Q

CPB Considerations DORV: Cardioplegia

A

Antegrade (multiple doses due to Ao-pulmonary collateral circulation)

93
Q

CPB Considerations DORV: Cardioplegia

A

Antegrade (multiple doses due to Ao-pulmonary collateral circulation)

94
Q

CPB Case Notes: DORV

A

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

95
Q

CPB Case Notes: DORV

A

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
Make sure you know how you are going to deal with high hematocrits in severe cyanotic conditions
Be careful with MUF since it will be easy to get the HCt at 50% + (most likely will add volume pulled off when you’re warming)

Congenital Defects & Pediatrics IA (17 decks)

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