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

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

1
Q

TOF is what percent of all cyanotic heart defects?

A

10%

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2
Q

What causes the physiologic manifestations of TOF

A

a large VSD and some degree of pulmonary stenosis.

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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

Aorta and PA start as a single tube called what?

A

Truncus arteriosus

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7
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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8
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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9
Q

Truncus Arteriosus becomes….

A

Aorta

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10
Q

Conus Cordis becomes….

A

Pulmonary Artery

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11
Q

What creates the conus cordis?

A

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

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12
Q

What creates the conus cordis?

A

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

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13
Q

RSTS

A

right superior truncus swelling

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14
Q

LITS

A

left inferior truncus swelling

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15
Q

RDCS

A

right dorsal conus swelling

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16
Q

LVCS

A

left ventral conus swelling

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17
Q

LVCS

A

left ventral conus swelling

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18
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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19
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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20
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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21
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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22
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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23
Q

Pentalogy of Fallot

A

TOF with the addition of an ASD

24
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

25
Q

What is worse in TOF w/ PA?

A

R –> L shunt is significantly worse

26
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%

27
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

28
Q

Palliation: Shunts Used

A

B-T Shunt Central Shunt

29
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)

30
Q

Central Shunt

A

Ascending aorta to the main PA

31
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

32
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)

33
Q

What correction is preferred over palliation?

A

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

34
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

35
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
36
Q

Two ways of TOF Repair

A

Transannular vs. Non-transannular

37
Q

Dilated-Anuerysmal TOF

A

TOF w/ absent pulmonary valve

38
Q

Hypoplasia TOF

A

TOF w/ pulmonary atresia

39
Q

Rastelli Procedure

A

RV- PA conduit

40
Q

Why would pacing wires be needed in surgically correcting TOF?

A

Due to large VSD patch

41
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

42
Q

What should you be careful with during surgical repair of TOF?

A

Your RV was stressed pre-op Be careful of vasodilators post-op since low BP can worsen RV dysfunction

43
Q

DORV

A

Double outlet right ventricle

44
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)

45
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)

46
Q

What is the spectrum of variation of DORV?

A

TOF on one end to TGA on the other end

47
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

48
Q

Two basic types of repair for DORV

A

Anatomic- restores circulation w/ two ventricles Univentricular- only one ventricle is functional

49
Q

Two basic types of repair for DORV

A

Anatomic- restores circulation w/ two ventricles Univentricular- only one ventricle is functional

50
Q

Surgery: Subaortic VSD DORV

A

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

51
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)

52
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)

53
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

54
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

55
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

56
Q

General Steps to transventricular TOF repair

A
  1. Mise en place
  2. Incise RVOT and pass Hagar dilator antegrade through the pulmonary valve.
  3. Identify the anatomy of:
    1. VSD
    2. tricuspid valve
    3. subvalvar apparatus of the tricuspid valve.
  4. Resect hypertrophied septoparietal muscle bundles.
  5. VSD Repair:.
  6. De-airing
  7. Closing:

Congenital Defects & Pediatrics COPY (19 decks)

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