Cardio Developmental Defects Flashcards
what kind of shunt is more common
L to R shunt
when do L to R shunts cause cyanosis
later–> “blue kids”
What is the most common congenital cardiac anomaly
VSD
3 L to R shunts
- VSD
- ASD
- PDA (patent ductus arteriousus)
* this is the order of prevalence
loud S1; wide, fixed split S2
ASD
continous machine-like murmur
PDA
MCC of early cyanosis
Tetralogy of Fallot
5 R to L shunts (early cyanosis)
- Persistant Truncus arteriosus
- Transposition of great vessels
- Tricuspid atresia
- Tetralogy of Fallot
- Total anomalous venous return
defect in persistant truncus arteriosus
failure of TA to divide into pulmonary trunk and aorta–> only one vessel of outflow for the heart and complete mixing of oxy and non-oxy blood
2 physical exam findings in persistent truncus arteriosus
- loud S2
- systolic ejection murmur at left sternal border
* also bounding arterial pulses (increased volume); biventricular hypetrophy also possible–> boot shaped heart maybe
* most pts have accompanying VSD
defect in tricuspid atresia
absence of tricuspid valve and hypoplastic RV
*need both a VSD and ASD for viability
why does transposition of great vessels produce profound cyanosis
there are two closed, independent loops
tx for transposition of g.v.?
prostaglandins to keep ductus arteriosus patent–> needs a shunt to mix some of the blood
defect in total anomalous venous return
pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc)
4 defects in tetralogy of Fallot
- pulmonic stenosis
- right ventricular hypertrophy (boot-shaped heart)
- VSD
- Overriding aorta (overrides VSD)
*caused by anterosuperior displacement of infundibular septum
what kind of flow causes cyanosis?
R to L (unoxygneated blood into systemic circulation)
why does squatting help during cyanotic episodes (tet spells) in tetralogy
increases peripheral vascular resistance, increasing afterload; increased L ventricular pressure forces blood L to R across the VSD
tx for patent ductus arteriosus (PDA)
indomethacin–> NSAID that decreases prostaglandins and allows it to close
where does coarctation of aorta occur in infants
proximal to insertion of ductus arteriosus–> this the only place aorta is receiving blood from
what congenital anomaly is coarctation of aorta most commonly associated with
bicuspid aortic valve
High BP in upper extremities, no pulses in lower extremities; pulsatile intercostal arteries
coarctation of aorta in adults–> occurs distal to ductus arteriosus (is now the ligamentum arteriosum)
*notching of ribs due to collateral flow through the intercostal arteries
Truncus arteriosus, tetralogy of fallot associated w/ which genetic disorder
22q11 syndrome–> DiGeorge syndrome
*heart defect + hypocalcemia = Digeorge
endocardial cushion defect (ASD, VSD, AV septal defects ass. w/
Down’s syndrome
- as is AML, ALL, Alzheimer’s
heart defects expected if congenital rubella
- septal defects
- PDA
- pulmonary artery stenosis
coarctation of aorta associted w/ What other heart defect is commonly seen with this
Turner syndrome
*commonly have bicuspid aortic valve also
Aortic insufficiency ass. w/
Marfans
*later, aortic dissection
Infants of diabetic mothers have an increased risk of which heart defect
transposition of great vessels
Adult coarctation of the aorta can eventually lead to heart failure symptoms because what happens
develop aortic regurgitation–> increased afterload due to obstructions wears the valve out over time
what is Eisenmenger’s syndrome
reversal of a L to R shunt (VSD, ASD, PDA) to a R to L shunt
pathgenesis of Eisenmengers
pulmonary vascular hypertension develops due to the increased left volume due to L to R shunt; eventually R side pressure exceed L side and the blood begins to flow from R to L
4 symptoms of cyanotic heart dz
- cyanosis
- clubbing
- SOB
- polycythemia
- seen later in L to R shunts and in Eisenmenger’s syndrome
what is Ebstein anomaly ( 2 main features)
- Tricuspid leaflets are displaced into right ventricle–> tricuspid regurg or stenosis
- Hypoplastic right ventricle
What happens in Ebstein due to the shitty tricuspid valve/ right ventricle
right atrium dilates–> due to poor flow through the malformed tricuspid into the small right ventricle
what do 80% of children with Ebstein anomaly have associated (heart defect)
Patent foramen ovale-> R to L shunt (pressures are higher in the dilated right atrium)
2 physical exam finding in Ebstein
- widely split S2
2. Tricuspid regurgitation
What causes Ebstein malformation
maternal Li use
2 arrythmias kids with Ebstein are at increased risk for
- WPW
2. supraventricular Tachycardia (SVT)
