Tetrology of Fallot
- anomalies
- surgery
- post op complications
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
TET SPELLS/Hypercyanotic spells
- pathophysio
- treatment
- 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
HLHS
- anatomy
- association
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
What kind of systolic click is heard in AV and PV stenosis? MVP?
AV and PV = early systolic click
MPV = mid-systolic click
What are some Key features seen in Marfan Syndrome
- Positive thumb sign
- Pectus excavatum
- Scoliosis
- Long arms
What is the mode of inheritance of Marfan syndrome, it’s defect and it’s cardiac abnormalities
AD
Fibrillin gene defect
Cardiac: Dilation of the ascending aorta, MVP
What kind of murmur is seen in ASD
Low pitched systolic ejection murmur at base with fixed split S2
What kind of murmur is seen in Aortic Stenosis
Systolic ejection murmur radiating to the neck
What kind of murmur is heard in PS, PDA and coarct
Systolic ejection murmur radiating to the back
What kind of murmur is seen in small VSD and mitral regurg
High pitched short systolic regurgitation murmur at the apex or LLSD
Features to suggest Neurocardiogenic Syncope
- 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
Features to suggest Cardiac Syncope
- 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:
ECG with short PR, large QRS voltages (mainly in precordial) what is diagnosis?
Pompe - GSD type 2. with cardiomyopathy
