Congenital Cardiac Defects Flashcards

1
Q

What causes a Congenital Heart Defect?

A

Genetic Predisposition + an environmental factor:

  • Drugs e.g. Alc, ecstasy, lithium & phenytoin
  • Infection e.g. TORCH
  • Maternal illness e.g. DM/SLE
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2
Q

Which cardiac defects present with cyanosis?

A
  • Transposition of the great vessels
  • Pulm Atresia
  • Truncus Arteriosus
  • TAPVD
  • Tetralogy of Fallot
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3
Q

When doing a paediatric history & exam what do you add to it?

A

Feeding
Weight & height
Development
Dysmorphia

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4
Q

What are the types of VSD?

A

Subaortic
Intramembranous
Muscular

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5
Q

Patient has a pansystolic murmur at the Lower L SB
The baby is also sob, tachypnic and tachycardic
What’s the defect?

A

Ventricular Septal Defect (VSD)

Causes a L–>R shunt hence the murmur +/- thrill
Also causes HF & Pulm HTN hence SOB, tachypnoea, tachycardia & hepatomegaly

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6
Q

How do we spot ASDs?

A

Wide fixed splitting of 2nd heart sound
Pulm outflow murmur

However most don’t cause problems in childhood and so are picked up in adults with AF, HF or pulm HTN

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7
Q

Whats the worst form of septal defect?

A

AVSD

End up with a single AV valve with both ASD/VSD

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8
Q

How can we treat a VSD?

A

Close it with Amplatzer Device

Or Patch closure if necessary (required open surgery and CP bypass)

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9
Q

Mild stenosis of the semilunar valves is often asymptomatic.

How would more severe pulm or aortic stenosis present?

A

Pulm:

  • SOB & Fatigue
  • Ejection Systolic Murmur on upper L SB + radiates to back

Aortic:

  • Low exercise tolerance, exertional chest pain & syncope
  • Ejection Systolic Murmur on upper R SB + radiates to carotids
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10
Q

How do we treat semilunar valve stenosis?

A

Balloon Valvuloplasty

once past puberty you can replace the valve but if you do it too early they’ll just outgrow it

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11
Q

What could cause a bairn to have a Patent Ductus Arteriosus? and do you treat it?

A

Born pre-term

  • Fluid Restriction / Diuretics
  • Prostaglandin Inhibitors (NSAIDs)
  • Surgical Ligation
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12
Q

Most PDA will close spontaneously, how do we close it if needed?

A

Umbrella Device

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13
Q

How can we spot a Coarctation of the Aorta?

A

Femoral Pulses will be reduced or absent and may be femoral-radial delay

Can do Angio & MRI but not everytime

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14
Q

How do we manage Coarctation of the Aorta?

A

1st reopen the DA with Prostaglandin Infusion
Then Resect with End-end Anastomosis

can also do balloon aortoplasty as a short term therapy before resection
Could do Subclavian Patch Repair if not enough material to anastomose

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15
Q

How do we manage Transposition of the Great Vessels?

A

Serious emergency

1) Prostaglandin Infusion maintains PDA
2) Switch Procedure

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16
Q

What are the parts of Tetralogy of Fallot?

A

Pulm Stenosis
Overriding Aorta
VSD
RVH

17
Q

Which Trisomies are ass with which congenital heart defects?

A

13 (Patau’s) = VSD/ASD
18 (Edward’s) = VSD/PDA
21 (Down’s) = AVSD

18
Q

Which genetic problem is ass with Coarctation of the Aorta?

A

Turner’s Syndrome aka 45,X

19
Q

What genetic problem is ass with pulm stenosis?

A

Noonan’s Syndrome

20
Q

What genetic problem is ass with Supravalvular Aortic Stenosis?

A

William’s Syndrome

Also causs LDs & Cocktail Party Personality

21
Q

Most Paediatric murmurs are unrelated to any pathology what do we call them?

A

Innocent Murmurs

22
Q

What features would suggest an innocent murmur?

A

1) Soft, grade 1 or 2 murmurs
2) Vibratory
3) Localised
4) Vary on position, exertion or respiration

Also most important no other cardiac signs or symptoms

23
Q

What are the most common innocent murmurs?

A

1) Still’s (LV outflow) murmur
2) Pulm Outflow Murmur
3) Carotid/brachiocephalic bruit
4) Venous Hum

24
Q

You find a kid with a soft low grade murmur, systolic, at the Apex & Left SB
It’s worse on supine or exercise but he’s got no other symptoms
What is it?

A

An innocent murmur

Systolic, found at apex & L SB and worse on supine/exercise suggest’s Still’s Murmur

25
Q

Child has a soft low grade murmur

  • Systolic
  • Upper L SB
  • Worse when supine or exercising

What is it?

A

Pulm Outflow Murmur (an innocent murmur)

Also ass with a narrow chest, features include:

  • Systolic
  • Upper L SB
  • Worse on supine/exercise
  • Soft & low grade
26
Q

Child has a low grade, harsh, systolic murmur in supraclavicular regions. It radiates to the neck but gets better when they turn their head. What is it?

A

Sounds like a Carotid/Brachiocephalic Bruit

  • Harsh systolic murmur
  • Supraclavicular, radiates to neck
  • Worse on exercise
  • Better on turning/extending neck
27
Q

Kid has an indistinct continuous murmur in the supraclavicular region.
you note it disappears when they lie down. What is it?

A

Venous Hum

  • Indistinct
  • Continuous (exacerbated on diastolic)
  • Supraclavicular
  • Only when upright