Congenital defects Flashcards

1
Q

How does the heart start embryologically

A

Clusters of angiogenic cells on mesodermal cardiogenic plate

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2
Q

Briefly describe steps of heart development

A
  • R/L endocardial tubes fuse into a single cardiac tube
  • Folding into bulboventricular loop
  • Atrial, ventricular and outflow tract septation
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3
Q

Name the 3 anatomical connections found in foetal circulation

A

Foramen ovale
Ductus Arteriosus
Ductus venosus

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4
Q

Describe resistance in foetal circulation

A

High resistance pulmonary circulation

Low resistance systemic circulation

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5
Q

How is foetal able to stay healthy despite congenital defect

A

Foetal circulation may bypass abnormalities via anatomical connections
Blood bypasses obstruction via one of these

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6
Q

How does blood go from the placenta to the foetus

A

Oxygenated blood flows from umbilical vein into the ductus venosus and into right side of the heart

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7
Q

What circulatory changes occur post birth

A

Closure of ductus arteriosus, ductus venosus and foramen ovale
Drop in pulmonary vascular resistance–> drop in pulmonary pressure

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8
Q

How does blood flow in foetal circulation

A

Oxygenated blood goes through foramen ovale, into left atrium and into head and neck

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9
Q

How long do foramen ovale and ductus arteriosus stay open

A

12-72 hours post birth

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10
Q

What is PPHN

A

Persistent pulmonary hypertension
Baby still has high pulmonary artery pressure so blood shunts from right to left
Presents as blue baby

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11
Q

What is a common cause of PPHN

A

Group B streptococcal pneumonia

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12
Q

What are the 2 main categories of congenital heart disease

A

Cyanotic- patient is blue

Acyanotic- patient is pink

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13
Q

What produces the blue colour in cyanotic congenital heart disease

A

Amount of deoxygenated Hb (g/l)

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14
Q

What are the parameters for defining cyanosis

A
  • Deoxygenated Hb is >50g/l in capillaries

- Deoxygenated Hb is >34g/l in arterial blood

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15
Q

What are the hallmarks for cyanosis due to congenital heart disease

A
  • Normal alveolar gas exchange (normal CO2)
  • No dyspnoea
  • Normal pulmonary venous saturations
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16
Q

What are the hallmarks for cyanosis due to lung disease (eg pneumonia)

A
  • Impaired alveolar gas exchange (CO2 may be high)
  • Tachypnoea and recession
  • Reduced pulmonary venous saturations
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17
Q

What do the following result from

a) cyanosis due to CHD
b) cyanosis due to lung disease

A

a) shunting of deoxygenated blood from R to L

b) Oxygen diffusion problems or ventilation/ perfusion mismatch

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18
Q

Where does blood mixing occur in transposition of the great vessels?

A

Mixing from ductus arteriosus and foramen ovale

19
Q

What defects are seen in tetralogy of fallot

A

Misalignment of outflow tract
Ventricular septal defect
Right ventricle hypertrophy due to trying to pump against resistance
R–L shunt

20
Q

What is the difference between atresia and stenosis

A
Atresia= valve completely blocked
Stenosis= valve is narrowed
21
Q

What is total anomalous pulmonoary venous drainage

A

Pulmonary veins go back into RA instead of left–> blood mixing

22
Q

What is truncus arteriosus

A

Aorta and PA haven’t separated`

23
Q

What are the 2 main groups of acyanotic congenital heart defects

A
  • Left right shunts

- Left heart outflow tract obstruction

24
Q

Effect of left- right shunt

A

Increased pulmonary blood flow leading to pulmonary oedema/ hypertension

25
Effect of left heart outflow tract obstruction
Pulmonary oedema, impaired tissue perfusion, lactic acidosis
26
In which 2 ways can acyanotic CHD lead to cyanosis
Pulmonary oedema impairs gas exchange | Pulmonary HT causes right to left shunting
27
Is a ventricular septal defect cyanotic or acyanotic
Acyanotic
28
How can left to right shunts be quantified
Ratio of pulmonary (Qp) to systemic (Qs) blood flow
29
Is coarctation of the aorta cyanotic or acyanotic
Acyanotic
30
What are the consequences of coarctation of the aorta?
Obstruction causes backflow into the left atrium, pulmonary oedema, poor blood flow to lower parts of the body
31
Name 4 acyanotic forms of CHD
Atrial septal defect Atrioventricular septal defect Critical aortic stenosis PDA
32
What is hypoplastic left heart syndrome
Blood flows up pulmonary artery and only way to get through is patent DA
33
What is the initial treatment for CHD
Reopening ductus with prostaglandic E or enlarging foramen ovale with balloon septostomy
34
3 general treatments for CHD
- Diuretic for pulmonary oedema - Re-open ductus arteriosus with prostaglandin E - Surgery and catheter procedure
35
What are the limiting factors in treatment of CHD
Anatomical- cannot grow new ventricles | Physiological- chronically elevated pulmonary blood flow= irreversible pulmonary HT
36
What is meant by Eisenmenger syndrome in ventricular septal defect
Secondary pulmonary HT reverses direction of the shunt leading to cyanosis
37
# Define the following a) meningocoele b) encephalocoele c) anencephaly
a) meninges coming out at skin level b) neural tissue coming out at skin level c) failure of formantion of skull wall, brain exposed
38
Is anencephaly compatable with life
No
39
What is hydrocephalus
Build up of CSF in ventricles leading to enlargement of cerebral ventricles
40
What are the neurological consequences of lumbar myelomeningocoele (4)
- Mixed sensory, motor and autonomic problems - Loss of bladder control - Faecal incontinence - Paralysis and loss of sensation of legs
41
What is gastroschisis
Full thickness small defect in abdominal wall lateral to umbilicus Bowel free in amniotic cavity
42
How long does the bowel take to function post surgical closure of gastroschisis
1-3 months
43
What is exomphalos
Wide based defect in which membrane covers herniated viscera
44
What is cleft lip and palate
Failure of fusion of maxillary and frontonasal processes