Cardiology

Question 1

Tetrology of Fallot

• anomalies
• surgery
• post op complications

Answer 1

10% of all CHD
1. RVOT 2. RVH 3. VSD 4. overriding Ao
Sx: 
1. Palliative - central shunt or BT shunt
2. Corrective: (usually by 1 yo)
- close VSD, enlarge RVOT, preserve RV function
Complication:
- chronic Pul regurg
- RVH, RV function
- arrhythmias

Question 2

TET SPELLS/Hypercyanotic spells

• pathophysio
• treatment

Answer 2

• profound cyanosis due to inc RVOT obstruction: aggitation, exercise

Rx: valsava (inc SVR)
O2 (Pul vasodilator, inc SVR)
IV morphine (0.1mg/kg)
IV fluid bolus (inc preload)
PIV propranolol (dec RVOT, coronary met)
IF fail, BT shunt or sx

Question 3

HLHS

• anatomy
• association

Answer 3

2% of all CHD

• underdeveloped L heart with atresia, stenosis, or hypoplasia of aortic or mitral valve or both. Hypolasia of ascending aorta and arch
• Turner (XO), Jacobsen, T13, T18

Question 4

What kind of systolic click is heard in AV and PV stenosis? MVP?

Answer 4

AV and PV = early systolic click

MPV = mid-systolic click

Question 5

What are some Key features seen in Marfan Syndrome

Answer 5

1. Positive thumb sign
2. Pectus excavatum
3. Scoliosis
4. Long arms

Question 6

What is the mode of inheritance of Marfan syndrome, it’s defect and it’s cardiac abnormalities

Answer 6

AD

Fibrillin gene defect

Cardiac: Dilation of the ascending aorta, MVP

Question 7

What kind of murmur is seen in ASD

Answer 7

Low pitched systolic ejection murmur at base with fixed split S2

Question 8

What kind of murmur is seen in Aortic Stenosis

Answer 8

Systolic ejection murmur radiating to the neck

Question 9

What kind of murmur is heard in PS, PDA and coarct

Answer 9

Systolic ejection murmur radiating to the back

Question 10

What kind of murmur is seen in small VSD and mitral regurg

Answer 10

High pitched short systolic regurgitation murmur at the apex or LLSD

Question 11

Features to suggest Neurocardiogenic Syncope

Answer 11

• Prodrome- grey-out nausea
• short lived (usually 1 - 2 min)
• often associated with position change
• not during exercise (though may follow exercise)
• may be situational (see blood)
• not associated with palpitations or chest pain
• usually occurs in otherwise healthy children
• often a family hx of neurocardiogenic fainters
• normal exam

Question 12

Features to suggest Cardiac Syncope

Answer 12

• little or no prodrome
• prolonged LOC (>5 min)
• exercise-induced
• fright/startled induced
• associated chest pain or palpitations
• history of cardiac diseases - AS, pulmonary hypertension
• positive family hx for: long QT, arrhythmia, syndromes, devices, cardiomyopathy, sudden death
• may have abnormal exam:

Question 13

ECG with short PR, large QRS voltages (mainly in precordial) what is diagnosis?

Answer 13

Pompe - GSD type 2. with cardiomyopathy