Congenital Cardiac Defects

Question 1

What causes a Congenital Heart Defect?

Answer 1

Genetic Predisposition + an environmental factor:

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

Question 2

Which cardiac defects present with cyanosis?

Answer 2

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

Question 3

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

Answer 3

Feeding
Weight & height
Development
Dysmorphia

Question 4

What are the types of VSD?

Answer 4

Subaortic
Intramembranous
Muscular

Question 5

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

Answer 5

Ventricular Septal Defect (VSD)

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

Question 6

How do we spot ASDs?

Answer 6

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

Question 7

Whats the worst form of septal defect?

Answer 7

AVSD

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

Question 8

How can we treat a VSD?

Answer 8

Close it with Amplatzer Device

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

Question 9

Mild stenosis of the semilunar valves is often asymptomatic.

How would more severe pulm or aortic stenosis present?

Answer 9

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

Question 10

How do we treat semilunar valve stenosis?

Answer 10

Balloon Valvuloplasty

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

Question 11

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

Answer 11

Born pre-term

Treatment:

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

Question 12

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

Answer 12

Umbrella Device

Question 13

How can we spot a Coarctation of the Aorta?

Answer 13

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

Can do Angio & MRI but not everytime

Question 14

How do we manage Coarctation of the Aorta?

Answer 14

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

Question 15

How do we manage Transposition of the Great Vessels?

Answer 15

Serious emergency

1) Prostaglandin Infusion maintains PDA
2) Switch Procedure

Question 16

What are the parts of Tetralogy of Fallot?

Answer 16

Pulm Stenosis
Overriding Aorta
VSD
RVH

Question 17

Which Trisomies are ass with which congenital heart defects?

Answer 17

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

Question 18

Which genetic problem is ass with Coarctation of the Aorta?

Answer 18

Turner’s Syndrome aka 45,X

Question 19

What genetic problem is ass with pulm stenosis?

Answer 19

Noonan’s Syndrome

Question 20

What genetic problem is ass with Supravalvular Aortic Stenosis?

Answer 20

William’s Syndrome

Also causs LDs &
Cocktail Party Personality

Question 21

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

Answer 21

Innocent Murmurs

Question 22

What features would suggest an innocent murmur?

Answer 22

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

Also most important no other cardiac signs or symptoms

Question 23

What are the most common innocent murmurs?

Answer 23

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

Question 24

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?

Answer 24

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

Question 25

Child has a soft low grade murmur

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

What is it?

Answer 25

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

Question 26

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?

Answer 26

Sounds like a Carotid/Brachiocephalic Bruit

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

Question 27

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

Answer 27

Venous Hum

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

Question 28

Ductus arteriosus what does it do?

Answer 28

Blood from PA to AORTA

Question 29

What does foramen oval do?

Answer 29

Blood from RA to LA