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)

(total anomalous pulmonary venous return)

no pulmonary veins into left atrium

(total anomalous pulmonary venous return)

loud S1; wide, fixed split S2


asymptomatic until adulthood


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

"blue kids"

left-to-right shunts
(late cyanosis)

Left-to-right shunts (4):

1. VSD
2. ASD
3. PDA

inadequate formation of tricuspid and mitral valves

(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):


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

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