L5: Cyanotic CHD Flashcards

1
Q

Def of Cyanosis

A

It is bluish discoloration of skin and mucus membranes.

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2
Q

When Does Clinical Cyanosis Present?

A
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3
Q

Cyanosis is recognized at …..

A
  • Higher levels of SaO2 ⇢ in patients with polycythemia.
  • Lower levels of SaO2 ⇢ in patients with anemia
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4
Q

Etiology of Cyanosis

A
  • Central
  • Peripheral
  • Methemoglobinemia
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5
Q

Etiology of Cyanosis

  • Central
A
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6
Q

Cental Causes of Cyanosis

  • Cardiac
A
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7
Q

Cental Causes of Cyanosis

  • Pulmonary
A

◈ Obstruction of the airway
◈ Parenchymatous lung disease (e.g. pneumonia)
◈ Weakness of the respiratory muscles

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8
Q

Cental Causes of Cyanosis

  • CNS
A

Central nervous system depression

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9
Q

Causes of Cyanosis

  • Peripheral
A

“⇡⇡ extraction of oxygen by tissues.”

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10
Q

Causes of Cyanosis

  • Methemoglobinemia
A

“Hemoglobinopathies

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11
Q

INVx for Cyanosis

A
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12
Q

INVx for Cyanosis

  • Hyperoxia Test
A
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13
Q

Consequences of Cyanosis

A
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14
Q

Polycythemia in Cyanosis

  • Pathogenesis
A
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15
Q

Polycythemia in Cyanosis

  • Effects
A
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16
Q

Clubbing in Cyanosis

  • Pathogenesis
A

◈ It results from soft tissue proliferation at the base of nail beds 2ry to chronic hypoxemia.

◈ It appears when the duration of central cyanosis > 6 months.

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17
Q

Clubbing in Cyanosis

  • Site & Grades
A
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18
Q

Hypoxic “Hypercyanotic” in Cyanosis

  • Incidence
A

◈ Common in 1st 6 months of life & decrease after 1st 2 years.

◈ Common in early morning.

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19
Q

Hypoxic “Hypercyanotic” in Cyanosis

  • Provoked By
A

Feeding, crying & straining.

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20
Q

Hypoxic “Hypercyanotic” in Cyanosis

  • Mechanism
A
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21
Q

Hypoxic “Hypercyanotic” in Cyanosis

  • CP
A
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22
Q

Hypoxic “Hypercyanotic” in Cyanosis

  • TTT
A
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23
Q

Squatting in Cyanosis

  • Cause
A
  • Seen in children with right-to-left shunt (e.g., TOF) to increase arterial oxygen saturation
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24
Q

Squatting in Cyanosis

  • Effect
A
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25
Q

CNS Complications in Cyanosis

A
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26
Q

Brain Abcess in Cyanosis

  • Cause
A
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27
Q

CVT in Cyanosis

  • Site
  • Incidence
  • CP
A
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28
Q

Arterial Ischemic Stroke in Cyanosis

  • Causes
A
  • Caused by embolization of a thrombus in the cardiac chamber or in the systemic veins.
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29
Q

Bleeding Disorders in Cyanosis

  • Causes
A

◈ Thrombocytopenia and defective platelet aggregation

◈ Consumption coagulopathy with prolonged PT & APTT

◈ Decreased levels of fibrinogen and factors V and VIII

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30
Q

Bleeding Disorders in Cyanosis

  • CP
A
  • Easy bruising,
  • petechiae of skin & MM
  • epistaxis
  • gingival bleeding
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31
Q

Bleeding Disorders in Cyanosis

  • TTT
A

Red cell withdrawal and replacement with equal volume of plasma

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32
Q

Low IQ in Cyanosis

A
  • Children with cyanosis and chronic hypoxia have lower than expected intelligent quotient, poorer perceptual and gross motor function than children with acyanotic CHDs
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33
Q

Scoliosis in Cyanosis

A

Children with chronic cyanosis, particularly girls with TOF may have scoliosis

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34
Q

Incidence of TOF

A

The most common congenital cyanotic heart disease.

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35
Q

Pathology of TOF

A
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36
Q

Pathology of TOF

  • The more the infundibular septum is displaced anteriorly, ……
A
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37
Q

Associated Anomalies to TOF

38
Q

Associated Anomalies to TOF

  • Coronary Arteries
39
Q

Associated Anomalies to TOF

  • PS
A

(sub valvular / valvular).

40
Q

Associated Anomalies to TOF

  • Aortic Arch
A

aortic arch is right sided in 20% of cases with TOF.

41
Q

Associated Anomalies to TOF

  • Venous Abnormalities
A

(TAPVR, PAPVR)

◈ Persistent left SVC drains into coronary sinus.

◈ Intrahepatic interruption of IVC with azygous continuation,

42
Q

Associated Anomalies to TOF

  • PDA
43
Q

Phsyiology of TOF

44
Q

Phsyiology of TOF

  • The magnitude and direction of the shunt are determined by
45
Q

Phsyiology of TOF

  • Mild PS
46
Q

Phsyiology of TOF

  • Moderate PS
47
Q

Phsyiology of TOF

  • Severe PS
48
Q

Phsyiology of TOF

  • Cyanosis will increase in the following conditions:
49
Q

Phsyiology of TOF

◈ If the shunt is left to right ⇢
◈ If the shunt is balanced ⇢
◈ If the right to left ⇢

50
Q

◈ However, mild to moderate neonatal cyanosis tends to increase.

◈ By 5-8 years of age the majority of children are cyanotic and symptomatic.

51
Q

CP of TOF

52
Q

CP of TOF

  • Hx
A

◈ Cyanosis
◈ Dyspnea on exertion
◈ Squatting
◈ Hypoxic spell

53
Q

CP of TOF

  • General Ex
A
  • Central cyanosis “Mostly delayed to 2nd month”
  • Blue clubbing “After 6 months”
  • Squatting position
54
Q

CP of TOF

  • Local Ex
55
Q

CP of TOF

  • Inspection & Palpation
56
Q

CP of TOF

  • Apex
57
Q

CP of TOF

  • Auscultation
58
Q

CP of TOF

  • Heart Sounds
59
Q

CP of TOF

  • Murmur
60
Q

INVx in TOF

61
Q

INVx in TOF

  • Chest X-Ray
62
Q

INVx in TOF

  • ECG
A

◈ Right axis deviation
◈ Right ventricular hypertrophy

63
Q

INVx in TOF

  • ECHO
64
Q

Complications of TOF

65
Q

Complications of TOF

  • Hypoxic Spells
A

in infant less than 2 years

66
Q

Complications of TOF

  • Hematologic Abnormalities
67
Q

Complications of TOF

  • Infective endocarditis
A

Occurs on
- stenotic pulmonary valve
- thickened tricuspid valve

68
Q

Complications of TOF

  • CNS Complications
A
  • Brain abscess in older children.
  • Arterial ischemic strokes
  • Cerebral venous thrombosis in infants < 2 years.
  • Cerebrovascular accidents as hemorrhage
69
Q

Management of TOF

70
Q

Management of TOF

  • Medical
71
Q

Medical Management of TOF
- 1st Thing to do

72
Q

Medical Management of TOF

  • Oral Iron
A

To prevent anemia

73
Q

Medical Management of TOF

  • Oral Propranolol
74
Q

Medical Management of TOF

  • Phlebotomy & Plasma Replacemet
A

⇢ For patients with high hematocrit

⇢ Prior to surgery in case of coagulation abnormalities

75
Q

Medical Management of TOF

  • IE Prophylaxis
76
Q

Medical Management of TOF

  • TTT of Hypercyanotic Spells
A

If present
“As mentioned before”

77
Q

Surgical Management of TOF

78
Q

Incidence of TGA

A

The most common congenital cyanotic heart disease in newborns.

79
Q

Pathology in TGA

80
Q

CP of TGA

81
Q

CP of TGA

  • General Ex
82
Q

CP of TGA

  • Auscultation
83
Q

CP of TGA

  • Heart Sounds
A

S2 ⇢ single, loud

84
Q

CP of TGA

  • murmur
A

No heart murmur is heard in infants with an intact ventricular septum.

  • If associated with VSD ⇢ Pan systolic murmur
  • If associated with PS ⇢ Ejection systolic murmur
85
Q

INVx of TGA

86
Q

INVx of TGA

  • Chest X-Ray
87
Q

INVx of TGA

  • Others
A

Same as in TOF
+
ECG, ECHO, Pulse oximetry & ABG, CBC, Cardiac catheterization “Angiography”

88
Q

Management of TGA

89
Q

Management of TGA

  • Medical
A

◈ Prostaglandin El infusion to improve arterial oxygen saturation by reopening the ductus.

◈ If associated with CHF may be treated with
- ACE
- Diuretics
- Digoxin.

90
Q

Management of TGA

  • Cardiac Cathetrization
A

Balloon atrial septostomy to ensure adequate intracardiac mixing.

91
Q

Management of TGA

  • Surgical