Congenital Cardiac Defects Flashcards
What causes a Congenital Heart Defect?
Genetic Predisposition + an environmental factor:
- Drugs e.g. Alc, ecstasy, lithium & phenytoin
- Infection e.g. TORCH
- Maternal illness e.g. DM/SLE
Which cardiac defects present with cyanosis?
- Transposition of the great vessels
- Pulm Atresia
- Truncus Arteriosus
- TAPVD
- Tetralogy of Fallot
When doing a paediatric history & exam what do you add to it?
Feeding
Weight & height
Development
Dysmorphia
What are the types of VSD?
Subaortic
Intramembranous
Muscular
Patient has a pansystolic murmur at the Lower L SB
The baby is also sob, tachypnic and tachycardic
What’s the defect?
Ventricular Septal Defect (VSD)
Causes a L–>R shunt hence the murmur +/- thrill
Also causes HF & Pulm HTN hence SOB, tachypnoea, tachycardia & hepatomegaly
How do we spot ASDs?
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
Whats the worst form of septal defect?
AVSD
End up with a single AV valve with both ASD/VSD
How can we treat a VSD?
Close it with Amplatzer Device
Or Patch closure if necessary (required open surgery and CP bypass)
Mild stenosis of the semilunar valves is often asymptomatic.
How would more severe pulm or aortic stenosis present?
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
How do we treat semilunar valve stenosis?
Balloon Valvuloplasty
once past puberty you can replace the valve but if you do it too early they’ll just outgrow it
What could cause a bairn to have a Patent Ductus Arteriosus? and do you treat it?
Born pre-term
- Fluid Restriction / Diuretics
- Prostaglandin Inhibitors (NSAIDs)
- Surgical Ligation
Most PDA will close spontaneously, how do we close it if needed?
Umbrella Device
How can we spot a Coarctation of the Aorta?
Femoral Pulses will be reduced or absent and may be femoral-radial delay
Can do Angio & MRI but not everytime
How do we manage Coarctation of the Aorta?
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
How do we manage Transposition of the Great Vessels?
Serious emergency
1) Prostaglandin Infusion maintains PDA
2) Switch Procedure
What are the parts of Tetralogy of Fallot?
Pulm Stenosis
Overriding Aorta
VSD
RVH
Which Trisomies are ass with which congenital heart defects?
13 (Patau’s) = VSD/ASD
18 (Edward’s) = VSD/PDA
21 (Down’s) = AVSD
Which genetic problem is ass with Coarctation of the Aorta?
Turner’s Syndrome aka 45,X
What genetic problem is ass with pulm stenosis?
Noonan’s Syndrome
What genetic problem is ass with Supravalvular Aortic Stenosis?
William’s Syndrome
Also causs LDs & Cocktail Party Personality
Most Paediatric murmurs are unrelated to any pathology what do we call them?
Innocent Murmurs
What features would suggest an innocent murmur?
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
What are the most common innocent murmurs?
1) Still’s (LV outflow) murmur
2) Pulm Outflow Murmur
3) Carotid/brachiocephalic bruit
4) Venous Hum
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?
An innocent murmur
Systolic, found at apex & L SB and worse on supine/exercise suggest’s Still’s Murmur
Child has a soft low grade murmur
- Systolic
- Upper L SB
- Worse when supine or exercising
What is it?
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
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?
Sounds like a Carotid/Brachiocephalic Bruit
- Harsh systolic murmur
- Supraclavicular, radiates to neck
- Worse on exercise
- Better on turning/extending neck
Kid has an indistinct continuous murmur in the supraclavicular region.
you note it disappears when they lie down. What is it?
Venous Hum
- Indistinct
- Continuous (exacerbated on diastolic)
- Supraclavicular
- Only when upright
