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42 terms

Congenital Cardiac Path

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22q11 syndromes
assoc w/
Truncus arteriosus and tetraology of Fallot
Down syndrome
assoc w/
1. ASD
2. VSD
3. AV septal defect (endocardial cushion effect)
Congenital rubella
1. septal defects
2. PDA
3. pulmonary artery stenosis
Turner syndrome
Coarctation of aorta (preductal)
Marfan's syndrome
Aortic insufficiency (late complication)
Infant of diabetic mother
Transposition of great vessels
pt squats to improve symptoms
Tetralogy of Fallot
caused by anterosuperior displacement of the infundibular septum
Tetralogy of Fallot
boot-shaped heart on x-ray
Tetralogy of Fallot
(caused by RVH)
pt suffers from "cyanotic spells"
Tetralogy of Fallot
long-standing cyanosis leads to:
clubbing and polycythemia
truncus arteriosus should divide into:
pulmonary trunk and aorta
a single great artery
Persistent Truncus Arteriosus
requires both ASD and VSD (or patent foramen ovale) for viability
Tricuspid artesia
pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc)
TAPVR
(total anomalous pulmonary venous return)
no pulmonary veins into left atrium
TAPVR
(total anomalous pulmonary venous return)
loud S1; wide, fixed split S2
ASD
TBX5
ASD
asymptomatic until adulthood
ASD
close this w/ infomethacin
Patent Ductus Arteriosus (PDA)
due to failure of the aorticopulmonary septum to spiral
D-transposition of great vessels
w/out surgical correct, most infants die w/in first few months of life
D-transposition of great vessels
shunt must be present to allow adequate mixing of blood (ie, VSD, PDA, patent foramen ovale)
D-transposition of great vessels
aorta leaves right ventricle
D-transposition of great vessels
pulmonary trunk leaves left ventricle (posterior)
D-transposition of great vessels
separation of systemic and pulmonary circulations
D-transposition of great vessels
can result in aortic regurgitation
Coarctation of the aorta
check femoral pulses on exam
Coarctation of the aorta
normal in utero, closes only after birth
Patent Ductus Arteriosus
if uncorrected, can eventually result in late cyanosis in the lower extremities ("differential cyanosis")
Patent Ductus Arteriosus
maintained by PGE synthesis and low O2 tension
Patent Ductus Arteriosus
continuous, "machine-like" murmur
Patent Ductus Arteriosus
"blue babies"
right-to-left shunts
(early cyanosis)
Right-to-left shunts (5):
5 T's:

1. Tetralogy of Fallot
2. Transposition of great vessels
3. Truncus arteriosus
4. Tricuspid atresia
5. TAPVR
"blue kids"
left-to-right shunts
(late cyanosis)
Left-to-right shunts (4):
1. VSD
2. ASD
3. PDA
4. AVSD
inadequate formation of tricuspid and mitral valves
AVSD
(AV septal defect)
all four chambers communicate (equal)
complete AVSD
caused by uncorrected VSD, ASD, or PDA
Eisenmenger's syndrome
Eisenmenger's syndrome
(5 steps)
1. uncorrected VSD, ASD, or PDA
2. causes compensatory vascular hypertrophy
3. results in progressive pulmonary hypertension
4. as pulm resistance increases, shunt reverses from L -> R to R -> L.
5. shunt reversal causes late cyanosis (clubbing and polycythemia)
Tetralogy of Fallot (4):
PROVe

1. Pulmonary stenosis
2. Right ventricular hypertrophy
3. Overriding aorta (overrides the VSD)
4. VSD
squating ->
(4 steps)
1. compresses femoral arteries
2. increases TPR (total peripheral resistance)
3. decreases the R -> L shunt
4. directs more blood from RV to the lungs

compression -> increased resistance -> decreased pressure