Cyanotic Heart Defects Flashcards
tetralogy of fallot - what is it?
right ventricular tract outflow obstruction/pulmonary stenosis
VSD
aortic over-ride
RVH
most common cyanotic lesion in children who survive infancy
tetralogy of fallot
ToF - risk factors
maternal rubella / viral disease
ToF pathophys
right ventricular outflow tract obstruction dictates degree of shunting
- minimal obstruction = CHF
- mild obstruction = equal L/V pressures = pink Tet (not a lot of shunting)
- severe obstruction = cyanosis = no pulm blood flow
what is a tet spell?
- context: occur in the AM when SVR is low
- precipitating factors: hot baths, fever, drugs that lower SVR
what happens during a tet spell?
cyanosis (due to R to L shunting)
syncope/seizures
cardiac arrest
tetralogy of fallot X-ray
boot-shaped, low pulm markings, right aortic arch
treatment for tetralogy of fallot
- complete repair surgically:
- VSD
- relief of RTOT
- ligation of shunts
- ASD/PFO closure
mortality w/o repair of ToF
50% by 3
90% by 20
95% by 30
most common cyanotic lesion of the newborn period
transposition of the great vessels
what is transposition of the great vessels
pulmonary arteries + aorta switched
no oxygenation of blood
how do these babies survive?
- ASD, VSD, or PDA - to survive
- PDA alone not sufficient to survive
transposition of the great vessels presentation
CHF in 1st week of life
transposition of the great vessels CXR
cardiomegaly
egg-shaped heart + narrow mediastinum
signs/symptoms of transposition
no murmur
single S2
early cyanosis
treatment of transposition
prostaglandin - keep PDA open
balloon atrial septostomy
arterial switch procedure
truncus arteriosus
bottom line: mixing o2 blood + non-O2 blood
single arterial trunk emerging from the ventricles - pulmonary + systemic circulation (co-existing VSD)
what is truncus arteriosus associated w/
DiGeorge’s syndrome
signs/symptoms of truncus arteriosus
CHF / cyanosis w/in 1st wk
L–> R shunt - dyspnea, respiratory infxn, failure to thrive
heart sounds of truncus arteriosus
S2 louder + prominent (only 1 valve closing)
chest x-ray truncus arteriosus
cardiomegaly + incr. lung markings
treatment
closure of the VSD + pulm artery reconnect
hypoplastic left heart syndrome
aortic valve stenosis or atresia +/- mitral valve stenosis/atresia
which heart conditions present w/ cyanosis in the 1st wk of life?
hypoplastic left heart syndrome
truncus arteriosus
transposition of the great vessels
