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Congenital Defects & Pediatrics COPY > Tetralogy of Fallot > Flashcards

Tetralogy of Fallot 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

What are the risk factors of TOF?

A

Alcoholism in the mother

Diabetic mother

Mother > 40 y/o

Poor nutrition during pregnancy

Rubella or other viral illnesses during pregnancy

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7
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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8
Q

Pentalogy of Fallot

A

TOF with the addition of an ASD

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9
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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10
Q

What is worse in TOF w/ PA?

A

R –> L shunt is significantly worse

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11
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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12
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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13
Q

Palliation: Shunts Used

A

B-T Shunt Central Shunt

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14
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)

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15
Q

Central Shunt

A

Ascending aorta to the main PA

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16
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

17
Q

When is surgery for TOF done?

A

Infants able to sat > 80% can wait until 6 months old.

18
Q

What correction is preferred over palliation?

A

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

19
Q

Total repair of TOF Risk

A

2%

20
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
21
Q

Two ways of TOF Repair

A

Transannular vs. Non-transannular

22
Q

Dilated-Anuerysmal TOF

A

TOF w/ absent pulmonary valve

23
Q

Hypoplasia TOF

A

TOF w/ pulmonary atresia

24
Q

Rastelli Procedure

A

RV- PA conduit

25
Q

Why would pacing wires be needed in surgically correcting TOF?

A

Due to large VSD patch

26
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

27
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

28
Q

Non-cardiac causes of cyanosis?

A

severe lung disease

hypoventilation

hemoglobin disorders (ex: methemoginemia)

29
Q

Treatment of cyanotic episode (TET spells)

A
  1. Oxygen supplementation
  2. Intravenous fluids
  3. Morphine
  4. Knees to chest
  5. IV Beta-blocker
  6. Alpha blockade
30
Q

How much hypothermia for surgical repair?

A

28 degrees Celcius

31
Q

Postoperative RV failure - how to treat?

A
  • Supportive
  • Inotropes and diuretics
  • usually will resolve in 72 hours
32
Q

Treatment of JET

A
  • Cool core to 35 deg
  • electrolyte replacemet
  • pain control
  • amioderone
  • overide pacing
33
Q

Residual

A
34
Q

Cyanotic heart diseases

A

cyanotic heart diseases

T: tetralogy of Fallot (TOF)

T: transposition of the great arteries (TGA)

T: truncus arteriosus

T: total anomalous pulmonary venous return (TAPVR)

T: tricuspid valve abnormalities and hypoplastic right heart syndrome

35
Q

how to treat TET spells ?

A
  1. Oxygen
  2. IVF
  3. Morphine, sedation
  4. Squat
  5. IV Beta blocker
  6. Alpha Agonist (phenylephrine )

Congenital Defects & Pediatrics COPY (19 decks)

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