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HD 2 > Congenital defects > Flashcards

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
Q

Effect of left heart outflow tract obstruction

A

Pulmonary oedema, impaired tissue perfusion, lactic acidosis

26
Q

In which 2 ways can acyanotic CHD lead to cyanosis

A

Pulmonary oedema impairs gas exchange

Pulmonary HT causes right to left shunting

27
Q

Is a ventricular septal defect cyanotic or acyanotic

A

Acyanotic

28
Q

How can left to right shunts be quantified

A

Ratio of pulmonary (Qp) to systemic (Qs) blood flow

29
Q

Is coarctation of the aorta cyanotic or acyanotic

A

Acyanotic

30
Q

What are the consequences of coarctation of the aorta?

A

Obstruction causes backflow into the left atrium, pulmonary oedema, poor blood flow to lower parts of the body

31
Q

Name 4 acyanotic forms of CHD

A

Atrial septal defect
Atrioventricular septal defect
Critical aortic stenosis
PDA

32
Q

What is hypoplastic left heart syndrome

A

Blood flows up pulmonary artery and only way to get through is patent DA

33
Q

What is the initial treatment for CHD

A

Reopening ductus with prostaglandic E or enlarging foramen ovale with balloon septostomy

34
Q

3 general treatments for CHD

A
  • Diuretic for pulmonary oedema
  • Re-open ductus arteriosus with prostaglandin E
  • Surgery and catheter procedure
35
Q

What are the limiting factors in treatment of CHD

A

Anatomical- cannot grow new ventricles

Physiological- chronically elevated pulmonary blood flow= irreversible pulmonary HT

36
Q

What is meant by Eisenmenger syndrome in ventricular septal defect

A

Secondary pulmonary HT reverses direction of the shunt leading to cyanosis

37
Q

Define the following

a) meningocoele
b) encephalocoele
c) anencephaly

A

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
Q

Is anencephaly compatable with life

A

No

39
Q

What is hydrocephalus

A

Build up of CSF in ventricles leading to enlargement of cerebral ventricles

40
Q

What are the neurological consequences of lumbar myelomeningocoele (4)

A
  • Mixed sensory, motor and autonomic problems
  • Loss of bladder control
  • Faecal incontinence
  • Paralysis and loss of sensation of legs
41
Q

What is gastroschisis

A

Full thickness small defect in abdominal wall lateral to umbilicus
Bowel free in amniotic cavity

42
Q

How long does the bowel take to function post surgical closure of gastroschisis

A

1-3 months

43
Q

What is exomphalos

A

Wide based defect in which membrane covers herniated viscera

44
Q

What is cleft lip and palate

A

Failure of fusion of maxillary and frontonasal processes

HD 2 (26 decks)

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