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2: Cardio - Cyanotic Heart Defects Flashcards

1
Q

What is cyanotic congenital heart disease

A

Congenital HD characterised by right-to-left shunt. Meaning deoxygenated blood enters oxygenated systemic. Hypoxaemia results as cyanosis

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2
Q

What are the 5 T’s of cyanotic HD

A 
TOF
TGA
Tricuspid atresia
Total anomalous pulmonary venous return 
Truncus arteriosus
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3
Q

What is tetralogy of fallot

A

Combination of four heart conditions

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4
Q

What is a way to remember 4 abnormalities in TOF

A

VORP

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5
Q

What are the four abnormalities in TOF

A

VSD
Over-riding aorta
Right ventricular hypertrophy
Pulmonary artery stenosis

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6
Q

What is the most common cyanotic congenital heart condition

A

TOF

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7
Q

What condition is TOF associated with

A

DiGeorge’s Syndrome

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8
Q

What is DiGeorge’s Syndrome

A

22q 11.2 deletion syndrome

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9
Q

How is TOF typically diagnosed

A

Antenatally

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10
Q

If not identified antenatally what age does TOF tend to present

A

1-2 months. Can be picked up to 6m afterwards

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11
Q

What are three clinical features of TOF

A
  1. Ejection systolic murmur
  2. Tet spells
  3. Squatting
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12
Q

What are tet spells

A

Intermittent, unpredictable episodes of tachypnoea, restlessness and cyanosis that lasts minutes-to-hours. Associated with physical stress.

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13
Q

Why do children with TOF squat

A

Squatting increases peripheral vascular resistance. This increases pressure in left-side of the heart reducing right-to-left shunt and subsequent hyperaemia.

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14
Q

What are three possible triggers for Tet spells

A
  • Defecation
  • Feeding
  • Crying
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15
Q

What murmur is heard in TOF

A

Harsh ejection systolic murmur

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16
Q

Where is murmur in TOF loudest

A

Erb’s point or left-upper sternal edge

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17
Q

Where is Erb’s point

A

3rd IC space. Left lower sternal edge.

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18
Q

What causes the murmur in TOF

A

Pulmonary stenosis

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19
Q

What are two other signs of TOF

A
  • Right ventricular heave

- Cyanosed w/clubbing

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20
Q

What is TOF caused by

A

Anterior misalignment of the aorticopulmonary septum

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21
Q

Explain why there is RVH in TOF

A

Pulmonary stenosis reduces outflow of blood. To try and maintain output right ventricle undergoes hypertrophy to increase pressure.

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22
Q

Why is there a right-to-left shunt in TOF

A

Pulmonary stenosis causes RVH. This increases pressure in the right-side so it is greater than the left. VSD creates a tract between right and left sides of the heart. This means blood can pass.

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23
Q

What determines degree of cyanosis in TOF and why

A

Degree of pulmonary stenosis - as this determines extent of RVH and hence pressure in right ventricle.

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24
Q

At what SpO2 will a child develop cyanosis

A

<80%

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25
What are three investigations in TOF
SpO2 CXR ECG
26
What will be seen on CXR in TOF
Boot-shaped heart: due to RVH
27
What will be seen on ECG
RVH
28
What 4 things are done to manage cyanotic attacks (Tet spells) in TOF
1. Oxygen 2. Knee's up position 3. B-blockers 4. IV morphine
29
Why are B-blockers given in TOF attacks
Reduce spasm of infundibulum (pulmonary stenosis)
30
How is severe TOF managed in the neonatal period
- IV prostaglandin E | - Blalock-Taussig Shunt
31
What is a blalock-taussig shunt and what is its purpose
Blalock Taussig shunt connects aorta to pulmonary.a. This means deoxygenated blood that has been ejected into the aorta can pass back to pulmonary.a to be oxygenated by the lungs
32
What is the definitive management of TOF
Surgical correction - resection infundibulum to reverse PA stenosis and VSD correction
33
When can surgery for TOF be performed
> 4 months
34
What can chronic cyanosis lead to in TOF
polycythaemia
35
What are 4 complications of TOF
- Cerebral thrombosis + ischaemia - Brain abscess - Bacterial endocarditis - Congestive HF
36
What is transposition of the great arteries
Anatomical reversal of pulmonary artery and aorta
37
What are 5 maternal risk factors for TGA
- Diabetes - >40 - Congenital rubella - Malnutrition - Alcohol
38
What is another risk factor for TGA
Genetic syndromes: Trisomies, DiGeorge
39
If there is no mixing of oxygenated and deoxygenated blood how will TGA present
Death
40
How does TGA typically present clinically
Post-natal cyanosis. Typically presents with cyanosis once the ductus arteriosus closes. Neonates will be breathless and have poor feeding
41
What is a sign of TGA on auscultation
Single loud S2
42
Explain the pathophysiology of TGA
In TGA anatomical roles aorta and PA are reversed. On right side, deoxygenated blood enters right atrium, passes to right ventricle and is then ejected via aorta. Meaning deoxygenated blood passes to systemic circulation. On left side blood enters via pulmonary vein to left atrium passes to left ventricle and is ejected via pulmonary artery.
43
Explain why babies survive in-utero with TGA
As oxygen is obtained from ductus venosus (from placenta) which enters IVC
44
Why are neonates with TGA symptomatic
As need to rely on lungs to obtain oxygen, no longer receiving oxygen via the placenta. If no mixing of circuits infant will die. If patent FO, VSD or patent DA enables mixing - will survive.
45
Why does TGA lead to heart failure
As right-side of the heart has less musculature and has not been designed to maintain systemic circulation
46
What is levo-TGA
Where ventricles and AV valves switch - not vessels meaning circulation is functional. Children asymptomatic until later life present with RVH and tricuspid regurgitation.
47
What investigations are ordered in TGA
CXR | ECHO
48
What will be seen on x-ray in TGA and why
Egg on string appearance. Large cardio-mediastinal silhouette causes globular appearance of the heart. Stress causes thymic atrophy which appears as the string.
49
What is first-line management of TGA in short term and why
IV PGEI infusion. PGs maintain patency of ductus arteriosus enabling mixing
50
What is definitive management of TGA
Surgical correction by Arterial switch procedure.
51
When is arterial switch procedure performed
First 2W
52
What is the arterial switch procedure
Switching of aorta and PA. With associated coronary vessels
53
When is ballon arterial septostomy indicated and what does it involve
If unsuitable for surgery. Involves creating a large ASD to enable mixing
54
What is mortality rate of TGA without surgery
90%
55
What is tricuspid atresia
Absent or rudimentary tricuspid valve
56
How will tricuspid atresia present clinically
Cyanosis days following birth
57
Explain pathophysiology of tricuspid atresia
Tricuspid atresia means there is no connection between right atrium and right ventricle. Venous blood is diverted from right atrium to left side via patent foramen ovale. Oxygenation depends on PDA.
58
How will tricuspid atresia present on ECG and why
- Left axis deviation = due to left ventricular hypertrophy | - P mitrale
59
How will tricuspid atresia present on CXR and why
Decreased pulmonary markings - due to decreased blood flow to the lungs
60
What 3 investigations are ordered for tricuspid atresia
- ECG - CXR - ECHO
61
What is given in short-term to manage tricuspid atresia
IV PGEI - to maintain patency of ductus arteriosus and oxygenation
62
What is 'long-term' management of tricuspid atresia
Surgical correction
63
What surgical correction of tricuspid atresia is offered in neonatal period
- Blalock trussing shunt | - or pulmonary artery banding
64
What is a blalock tausig shunt
Connection between pulmonary. a and aorta. This enables deoxygenated blood pumped out by aorta to return to pulmonary artery down a pressure gradient and undergo oxygenation
65
At 6 months of age what surgical procedure is offered for tricuspid atresia
Glenn shunt
66
What is a Glenn shunt
Shunt placed between aorta and pulmonary artery
67
What is offered to pre-school children with tricuspid atresia
Fontan procedure
68
What is a Fontan procedure
IVC is connect to pulmonary artery so blood may be oxygenated directly

Paediatrics (61 decks)

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