EXAM #2: CYANOTIC HEART DISEASE Flashcards

(35 cards)

1
Q

What causes cyanosis?

A

Increased concentration of reduced Hb

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2
Q

What is acrocyanosis?

A

Cyanosis of the hands and feet in the newborn that is a NORMAL finding

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3
Q

What is isolated circumoral cyanosis?

A

Cyanosis of the lips seen often in cold, fair-skinned children

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4
Q

What is the utility of a hyperoxitest?

A

Test to determine the etiology of cyanosis

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5
Q

Outline the methology of the hyperoxitest.

A

1) Check baseline pO2
2) 100% O2 for 10 min
3) Recheck pO2

*Pulmonary= increase in pO2; Cardiac= no change

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6
Q

What is the utility of Prostaglandin E1?

A

Maintain the patency of the ductus arteriosus

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7
Q

What is the mnemonic to remember the causes of cyanotic heart disease?

A

5T’s:

1) Truncus Arteriosus
2) Transporition of Great Vessels
3) Tricuspid Atresia
4) Tetralogy of Fallot
5) Total anomalous pulmonary venous return

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8
Q

What defect is seen in TGA?

A

Transposition of the Great Arteries

  • Aorta from RV
  • Pulmonary from LV
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9
Q

What shunts can there be in TGA to maintain life?

A

1) VSD
2) PFO (patent foramen ovale)
3) PDA

*It is better to have 2x than 1x

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10
Q

What is this typical history for TGA?

A

Cyanosis from birth, especially in a male (full-term)

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11
Q

What is the typical presentation of TGA?

A
  • Cyanotic, tachypneic infant in NO distress

- S2 is a single loud sound

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12
Q

Outline the medical management for TGA.

A

1) PGE1
2) Correct acidosis
3) Balloon atrial septostomy

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13
Q

What is the name for the Balloon atrial septostomy? Outline this procedure.

A

Rashkind Procedure

1) Catheter advanced across PFO
2) Balloon inflated and ripped back into RA

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14
Q

What is the surgical procedure to treat TGA?

A

Arterial switch operation

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15
Q

What is the major complication of the Arterial switch operation?

A

Getting the coronaries right

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16
Q

What are the four components of Tetralogy of Fallot (TOF)?

A

1) Large VSD
2) Overriding aorta
3) RV outflow obstruction (pulmonary stenosis)
4) RVH

*Based on a malalignment of the infandibular septum

17
Q

What direction will blood shunt through the VSD in TOF?

A

Right-to-left b/c of resistance due to pulmonary stenosis/ overriding aorta

18
Q

What causes the murmur in TOF?

A

RV outflow tract obstruction

*Note that a quieting murmur is v. concerning–RV is failing

19
Q

What is the classic CXR in TOF?

A

Boot-shaped heart

20
Q

What is a TET spell?

A

Hypoxic spell induced by spasm of the RV that presents as:

1) Sudden onset rapid breathing
2) Irritability
3) Prolonged crying
4) Increased cyanosis
5) Decreased heart murmur

21
Q

How do you treat a TET spell?

A

1) Infant knee-chest
2) Oxygen
3) Morphine
4) Sodium bicarbonate

22
Q

What are the two surgical procedures to treat TOF?

A

1) Blalock-Taussig shunt

2) Full surgical repair

23
Q

What is the Blalock-Taussig shunt?

A

Connection between PA to Brachiocephalic a.

This is a surgical shunt similar to a PDA

24
Q

What is Tricuspid Atresia?

A

Absent tricuspid valve with hypoplastic RV

25
What is the role of the ECG in Tricuspid Atresia?
Left axis and LVH in cyanotic child is diagnostic
26
What is are the medical steps to treat Tricuspid Atresia?
1) PGE1 | 2) Balloon atrial septostomy
27
What surgical procedures are indicated for Tricuspid Atresia?
1) BT | 2) Fontan procedure
28
What is the Fontan procedure?
IVC/SVC directed straight into the PA
29
What is a Truncus Arteriosus?
Single arterial trunk leaves the heart giving rise to: - Systemic circulation - Pulmonary circulation - Coronary circulation *Large VSD is associated
30
What are the signs of Truncus arteriosus?
1) Wide pulse pressure | 2) Single S2
31
How are Truncus arteriosus cases managed medically?
Anti-CHF meds temporarily
32
How is Truncus arteriosus surgically managed?
Complete repair early in life that involves: 1) Separation of the PA and connecting it to the RV via a conduit 2) Patching the VSD
33
What is Total Anomalous Pulmonary Venous Return (TAPVR)?
Pulmonary veins DO NOT return to the LA; instead, they return to the RA *Need the ASD
34
What is the utility of the CXR in TAPVR?
V. helpful in evaluating obstruction - Obstruction= normal heart and pulmonary edema - Non= cardiomegaly
35
How do you surgically manage TAPVR?
Reconnect the pulmonary venous confluence back to the LA