Congenital Heart Disease Flashcards
5 Ts
Tetrology Transposition Truncus Tricuspid atresia TAPVR
Can also be Ebstein, HRHS, HLHS
Eisenmerger syndrome
Noaways late dx ASD in adult
AV canal
Coarctation
Truncus
Pulmonis stenosis
Trisomy 21
Monosomy X
DiGeroge
Noonan (12q22)
Supravalvular AS
PDA
TGA
William syndrome (chr 7, elastin)
Congenital rubella syndrome
Infant of diabetic mother
Comps of R-L shunt
Cyanotic
Dec pulmonary blood flow
Grwoth retard, polycyth, clubbing, cerebral hypoxia, paradoxic embolism
TOF
Sx depend on degree of RVOT obstruction
Mild - similar to isolate VSD
Mod to severe - R-L in 6 mo…classic
Tet spells due to RVOT spasm
TGA
Requires shunt for survival
VSD stbale
PFO unstable and need a baloon
if unrepaired, RVH develops
Arterial switch is main way to repair
COngenitally corrected TGA
Hypertrophy and late decompensation of the RV
COmplete repair requires both atrial and arterial switch
Truncus arteriosus
Single great artery with underlying VSD
Early cyanosis and inc pulmonary blood flow
DiGeorge/velocardiafcial syndrome - Retinoids
Tricuspid atresia
Large MV orifice
Associated RV hypoplasia
Need ASD/PFO (right to left) for outlet from RA
Need VSD for outflow to PA
TAPVR
All 4 pulmooanry veins fail to drain into LA
Need ASD/PFO for pulmonary venous blood to enter the LA
Cyanosis due to large R-L shunt via ASD
Secondary RA and RV hypertrophy with dilated PA and LA hypoplasia
Left to right shunt
inc pulmonary blood flow so no dec in O2 sat
Comps of L-R shunt
Eisenmerger - overciculation of lungs leads to pulm HTN…PVR>SVR so becomes R to L shunt and get cyanosis
ASD
Flow murmur
Most are secundum
Get lung trnasplant and close ASD
VSD
2 types - most common is perimembranous
AVSD
Associated with down syndrome
Failure of endocardial cushions to fuse
PDA
Continuous harsh murmuor
Indomethacin is Rx tx
Coarctation
Narrowing of aorta
In adult - tubular hypoplasia prox to PDA
Turner syndrome
With PDA - surgery as neonate
Without PDA - asx…UE HTN as adult and weak LE pulses
Congenital aortic stenosis and atresia in neonate
Neonatal critical aortic valve stenosis - small or dysplastic cusp…if severe, associated with hypoplastic left heart syndrome
Subvalvular stenosis - rung or collar
Williams sx
Supravavlular aortic stneosis
Elfin face
Thickening and constriction of aortic wall
Elastin gene of chr 7
Noonan syndrome
Short webbed neck with ear malformations
Pulm stenosis Hypertrophic CM (rasopathy), and mitral valve prolaspe
