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Congenital Defects (Pathoma) (ToF ToF (stenosis of RV outflow tract…
Congenital Defects (Pathoma)
start w/ embryogenesis
weeks 3-8 (classic)
1% of live births
most sporadic
OFTEN resulting in L and R shunting
VSD
#1 most common CHD
assd. w/ fetal alcohol syndrome
bad septum of ventricles
hi P Left ventricular blood enters lo P Right circulation
L to R shunt (key prob)
Eisenmenger Syndrome
RV hypertrophy( hi RV pressures)
polycythemia
hypoxemia (EPO release)
clubbing (fingernail changes due to cyanosis)
incr. blood vol in pulm circ
Pulm HTN
rising R circuit pressures facilitates R to L shunt (blue blood enters systemic)
cyanosis develops
Treatment
Surgical closure (large def)
spon. closure (small def)
ASD
bad septa
#1 common Ostium Secundum
osmium primum assd. w/ Down's
results L to R shunt
Findings
split S2 on auscultation
hi P in LA crosses to RA, incr volume delays closure of pulmonic valve (hence splitting sound)
DVT emoli bypass pulmonary circulation, lodge in brain
paradoxical embolus
PDA patent ductus arteriosus
assd. w/ cong. Rubella
hi P blood enters R heart circ via PDA
R heart circ P rises until reversal of shunt: #Eisenmenger syndrome
pHTN also happens
blue blood enters
after major branches of aortic arch:
lower extremities:
cyanosis in LE later in life
Clinical sign
Asymptomatic at birth
holosystolic "machine like" murmur
Indomethacin
decr. PGE
PGE keeps DA open
ToF
stenosis of RV outflow tract
results in R to L shunt
early cyanosis
exercise induced cyanosis relieved by squatting
puts pressure on aorta (incr art. vascular resistance) to move blood back to R heart circulation (lungs)
degree of stenosis determines cyanosis
RV hypertrophy
VSD
overriding aorta
Findings
CXR: boot shaped heart
Transposition of Great Vessels
RV feeds aorta, systemic feeds RA
Likewise, LV feeds PA, PV feeds LA
only way to survive is to create atrial septal and ventricular septal openings
maintain PDA (PGE administration)
assd. maternal diabetes
early cyanosis
Truncus Arteriosus
1 trunk: fails to divide
mixing of oxy/blue bloods
early cyanosis
Tricuspid Atresia
R ventricle becomes hypoplastic
classic assd w/ ASD: allows blood to cross over
early cyanosis: blue blood in systemic circ
Coarctation of Aorta: narrowing
Infantile
assd. w/ PDA
distal to aortic arch/ Proximal to PDA
cyanosis to LE
assd. w/ Turner syndrome
Adult (discovered in adultood)
simple coarctation
HTN in upper Extremity.
hypotension in LE w/ weak pulses
assd w/ bicuspid Aortic valve
collateral circulation develops across intercostal arteries in order to pass block
engorged intercostal arteries
notched ribs
