Cardio Developmental Defects

Question 1

what kind of shunt is more common

Answer 1

L to R shunt

Question 2

when do L to R shunts cause cyanosis

Answer 2

later–> “blue kids”

Question 3

What is the most common congenital cardiac anomaly

Answer 3

VSD

Question 4

3 L to R shunts

Answer 4

1. VSD
2. ASD
3. PDA (patent ductus arteriousus)
   * this is the order of prevalence

Question 5

loud S1; wide, fixed split S2

Answer 5

ASD

Question 6

continous machine-like murmur

Answer 6

PDA

Question 7

MCC of early cyanosis

Answer 7

Tetralogy of Fallot

Question 8

5 R to L shunts (early cyanosis)

Answer 8

1. Persistant Truncus arteriosus
2. Transposition of great vessels
3. Tricuspid atresia
4. Tetralogy of Fallot
5. Total anomalous venous return

Question 9

defect in persistant truncus arteriosus

Answer 9

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

Question 10

2 physical exam findings in persistent truncus arteriosus

Answer 10

1. loud S2
2. 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

Question 11

defect in tricuspid atresia

Answer 11

absence of tricuspid valve and hypoplastic RV

*need both a VSD and ASD for viability

Question 12

why does transposition of great vessels produce profound cyanosis

Answer 12

there are two closed, independent loops

Question 13

tx for transposition of g.v.?

Answer 13

prostaglandins to keep ductus arteriosus patent–> needs a shunt to mix some of the blood

Question 14

defect in total anomalous venous return

Answer 14

pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc)

Question 15

4 defects in tetralogy of Fallot

Answer 15

1. pulmonic stenosis
2. right ventricular hypertrophy (boot-shaped heart)
3. VSD
4. Overriding aorta (overrides VSD)

*caused by anterosuperior displacement of infundibular septum

Question 16

what kind of flow causes cyanosis?

Answer 16

R to L (unoxygneated blood into systemic circulation)

Question 17

why does squatting help during cyanotic episodes (tet spells) in tetralogy

Answer 17

increases peripheral vascular resistance, increasing afterload; increased L ventricular pressure forces blood L to R across the VSD

Question 18

tx for patent ductus arteriosus (PDA)

Answer 18

indomethacin–> NSAID that decreases prostaglandins and allows it to close

Question 19

where does coarctation of aorta occur in infants

Answer 19

proximal to insertion of ductus arteriosus–> this the only place aorta is receiving blood from

Question 20

what congenital anomaly is coarctation of aorta most commonly associated with

Answer 20

bicuspid aortic valve

Question 21

High BP in upper extremities, no pulses in lower extremities; pulsatile intercostal arteries

Answer 21

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

Question 22

Truncus arteriosus, tetralogy of fallot associated w/ which genetic disorder

Answer 22

22q11 syndrome–> DiGeorge syndrome

*heart defect + hypocalcemia = Digeorge

Question 23

endocardial cushion defect (ASD, VSD, AV septal defects ass. w/

Answer 23

Down’s syndrome

• as is AML, ALL, Alzheimer’s

Question 24

heart defects expected if congenital rubella

Answer 24

1. septal defects
2. PDA
3. pulmonary artery stenosis

Question 25

coarctation of aorta associted w/ What other heart defect is commonly seen with this

Answer 25

Turner syndrome

*commonly have bicuspid aortic valve also

Question 26

Aortic insufficiency ass. w/

Answer 26

Marfans

*later, aortic dissection

Question 27

Infants of diabetic mothers have an increased risk of which heart defect

Answer 27

transposition of great vessels

Question 28

Adult coarctation of the aorta can eventually lead to heart failure symptoms because what happens

Answer 28

develop aortic regurgitation–> increased afterload due to obstructions wears the valve out over time

Question 29

what is Eisenmenger’s syndrome

Answer 29

reversal of a L to R shunt (VSD, ASD, PDA) to a R to L shunt

Question 30

pathgenesis of Eisenmengers

Answer 30

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

Question 31

4 symptoms of cyanotic heart dz

Answer 31

1. cyanosis
2. clubbing
3. SOB
4. polycythemia

• seen later in L to R shunts and in Eisenmenger’s syndrome

Question 32

what is Ebstein anomaly ( 2 main features)

Answer 32

1. Tricuspid leaflets are displaced into right ventricle–> tricuspid regurg or stenosis
2. Hypoplastic right ventricle

Question 33

What happens in Ebstein due to the shitty tricuspid valve/ right ventricle

Answer 33

right atrium dilates–> due to poor flow through the malformed tricuspid into the small right ventricle

Question 34

what do 80% of children with Ebstein anomaly have associated (heart defect)

Answer 34

Patent foramen ovale-> R to L shunt (pressures are higher in the dilated right atrium)

Question 35

2 physical exam finding in Ebstein

Answer 35

1. widely split S2

2. Tricuspid regurgitation

Question 36

What causes Ebstein malformation

Answer 36

maternal Li use

Question 37

2 arrythmias kids with Ebstein are at increased risk for

Answer 37

1. WPW

2. supraventricular Tachycardia (SVT)