5 types of R to L shunts "blue babies"? (early cyanosis)
Tetralogy of Fallot
Transposition of Great Vessels
Total anomalous pulmonary venous return
What is truncus arteriosus?
Failure of truncus to divide into pulmonary artery and aorta.
Single large vessel arises from both ventricles before splitting.
S/S of truncus arteriosus?
- Early cyanosis
- Deoxygenated blood from R ventricle mixes with oxygenated blood from L ventricle.
What is tricuspid atresia?
Absence of tricuspid valve.
Often associated with ASD
S/S of tricuspid atresia?
- Early cyanosis
- R ventricle is hypoplastic
- Requires ASD & VSD for survival
What is TAPVR?
Pulmonary veins drain into R heart circulation
What are the L to R shunts "blue kids"? (late cyanosis)
What are the frequencies of the L to R shunts?
VSD > ASD > PDA
What is Eisenmerger's syndrome?
This is when a L to R shunt reverses to become R to L because of compensatory pulmonary vascular hypertrophy.
Initial L to R shunt increases blood flow to pulmonary circulation leads to pulmonary HTN forcing the pulmonary vasculature to hypertrophy. This increases pulonary resistance leading to a reversal in shunt. Later R to L shunt causes the "late cyanosis" in L to R shunt defects.
S/S of Eisenmerger's syndrome?
- RV hypertrophy (reactive to pul resistance)
- Polycythemia (reactive to hypoxia)
- Clubbing fingers (hypoxia)
4 defects of Tetralogy of Fallot?
- Pulmonary stenosis
- RV hypertrophy
- Overriding aorta (overrides VSD)
Why is Tetralogy of Fallot a R to L shunt although it has VSD?
Stenotic pulmonary valve increases RV pressure
S/S of Tetralogy of Fallot?
Boot shaped heart
Patients squat in response ot cyanotic spell
Why does squatting help Tetralogy of Fallot symptoms?
Squatting increases TPR which increases LV pressure thus decreasing R to L shunt and increases blood flow towards lung.
What is transposition of great vessels?
Aorta leaves RV while pulmonary artery leaves LV
Separation of systemic and pulmonary ciruclations.
Not compatible with life unless shunt is present to allow mexing to blood.
Associated with diabetic mother
Where is the coarctation of the aorta in the infantile type?
Distal to the aortic arch but proximal to the ductus arteriosus.
S/S of inftantile type coarctation of hte aorta
Lower extremity cyanosis in infants, often at birth.
Associated with Turner's syndrome .
Location of adult type coarctation of aorta?
Distal to aortic arch and ligamentum arteriosum
S/S of adult type coarctatoin of the aorta
HTN in upper extremities with weak pulses in lower extremities.
Usually seen with notching of ribs on X-ray
Notching from collarteral circulation that develops to go around coarctation
Associated with bicuspid aortic valve
- Notching from collarteral circulation that develops to go around coarctation
What is patent ductus arteriosus?
Ductus arteriosus does not close after fetal period to become ligamentum arteriosus
Forms L to R shunt between aorta and pulonary trunk
What body molecule maintains ductus arteriosus (PDA)?
PGE synthesis and low O2 tension
What congenital defects are associated with 22q11 syndrome?
Tetralogy of Fallot
What congenital defects are associated with down syndrome?
AV septal defect (endothelial cushion defect)
What congenital heart defects are associated with congential rubella?
Pulmonary artery stenosis
What congenital heart defects are associated with Turner syndrome?
Infantile type coarctation of the aorta.
What congenital heart defects are associated with Marfan's syndrome?
What congenital heart defects are associated with infant of diabetic mother?
Transposition of great vessels.