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congenital heart disease Flashcards

1
Q

syndromes with associated CHD sporadic

A

williams- supra-valvular AS, PS

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2
Q

syndromes with associated CHD:

teratogenic:

  1. rubella
  2. alcohol
  3. dilantin
  4. lithium
A
  1. rubella- PDA, PS, ASD
  2. Alcohol- VSD
  3. Dilantin- PS AS, coarctation, PDA
  4. lithium- Ebstein’s tricupsid atresia
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3
Q

syndromes with associated CHD

chromosomal

  1. tri 21
  2. tri 13
  3. tri 18
A
  1. tri 21: endocardial cushion, TOF
  2. tri 13: VSD
  3. tri 18: polyvalvular dysplasia
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4
Q

cardiac circulation is in

A

series

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5
Q

complex CHD may be in

A

parallel or mixing

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6
Q

ductus arteriosus dependent (requiring protaglandin) (3)

A
  1. pulmonary atresia
  2. transposition of the great arteries
  3. severe coarctation of the aorta
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7
Q

non- duct dependent (3)

A
  1. transposition of the great arteries with a large VSD
  2. double outlet right ventricle
  3. truncus arteriosus
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8
Q

RVOT/obstruction

A

TOF

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9
Q

parallel circulations

A

TGA

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10
Q

common mixing

A

truncus arteriosus

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11
Q

CHD with pulmonary HTN (Eisenmengers)

A

VSD, ASD and PDA

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12
Q

cyanosis generally occurs when (2)

A
  1. there is too little pulmonary blood flow

2. venous blood mixes with arterial blood

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13
Q

flow is largely influenced by

A

resistance and compliance

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14
Q

shunts cause

A

downstream chamber dilation

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15
Q

_______ equalize across a large defect/ shunt

A

pressures

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16
Q

obstruction to flow produces

A

hypoplasia

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17
Q

obstruction elevates

A

upstream proximal pressure

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18
Q

the blood flow through an organ can be quantitated if an indicator is either added to or removed from the blood during its flow through the organ

A

Fick principle

19
Q

Qp/Qs

A

AO - SVC / AO- PA

20
Q

ASD:

  1. common in
  2. women or male
  3. types
  4. volume overload on the
  5. eisenmenger’s
    magnitude of shunt relative to
A
  1. common defect in children and 2nd most common in adult
  2. women 3:1
  3. secundum, venosus and primum
  4. right sided volume overload
  5. eisenmenger’s rare- 5%
  6. magnitude of shunt relative to diastolic distensibility and vascular resistance
21
Q

a shunt in the atrium will cause an

A

increase in pulmonary blood flow

22
Q

ASD Qp to Qs ratio

23
Q

a small VSD will _______ pulmonary blood flow and to a certain extent pressure

24
Q

a large VSD will increase both

A

blood flow and pressure

25
Q

VSD Qp to Qs ratio

26
Q

ASD vs VSD

if the amount of pulm. blood flow in both pt. is the same, who will be sicker

A

ASD- volume overload

VSD- volume and pressure overload

27
Q

reversed shunt

A

eisenmenger’s syndrome

28
Q

Eisenmenger’s syndrome:

  1. what happens over time?
  2. as the pulmonary vascular resistance increases the Qp/Qs
  3. When ______ blood will shunt from the right heart to the left heart leading to the development of systemic cyanosis
  4. associated with
A
  1. increased blood flow/pressure damages the pulmonary arteriole muscular walls causing hypertrophy, leading to elevation of the PVR and a reduction in PBF
  2. decreases
  3. Qp
29
Q

overall effect in eisenmenger’s

A

resistance to flow in pulmonary circuit now higher than systemic

30
Q

PDA connects the

A

descending aorta to left pulmonary artery

31
Q

what closes the PDA

A

after birth - up to 2 weeks with the increase in O2 and PGE

32
Q

PDA becomes the

A

ligamentum arteriosus

33
Q

aorta constriction typically distal to subclavian artery

A

coarctation of the aorta

34
Q

COA:

  1. causes
  2. men or women
  3. associated with
  4. consider intervention when
A
  1. causes upper extremity HTN leading to cardiac hypertrophy from pressure overload
  2. males 2:1
  3. BAV (60-80%)
  4. consider intervention in persistent HTN and a gradient > 20-30 mmHg
35
Q

most common cyanotic CHD

36
Q

TOF:

  1. has four issues
  2. in 25% of the cases we see
  3. if it is pentalogy of fallot what do they have in addition to
A
  1. (i) RVOT obstruction (ii) RVH (iii) overriding aorta (iv) VSD
  2. right aortic arch - 25%
  3. pentalogy of fallot- ASD
37
Q

blue child

38
Q

TOF:

  1. what do we see
  2. Qp to Qs
A
  1. output from the right and left ventricles is different

2, Qp

39
Q

rashkind septostomy

A

emergency procedure that creates an inter-atrial bi-directional shunt prior to performing an arterial switch operation in which the aorta and pulmonary artery are exchanged to connect LV to aorta and RV to pulmonary artery

40
Q

mustard procedure

A

atrial switch operation

41
Q

tricuspid atresia

A

single ventricle

42
Q

Qp>Qs

A

pulmonary blood flow too much

43
Q

Qp

A

pulmonary blood flow too little

44
Q

Qp=Qs

A

pulmonary blood flow just right

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