Congential Heart Disease- Leah (3)

Question 1

Two diseases that present at the time of PDA closure (3-4 days old) without cyanosis?

What must be done if these conditions are suspected?

Answer 1

1. Aortic coarctation
2. Hypoplastic left heart.

Always give these kids PGE1

Question 2

Four CHDs that are not causes of cyanosis and do not directly correlate with time of PDA closure?

Answer 2

1. ASD
2. VSD
3. PDA
4. AVSD

*probably the least symptomatic of all of the congenital defects.

Question 3

Most common congenital defect?

With what is it commonly associated?

Answer 3

VSD
associated with fetal alcohol poisoning
(Yes, I agre this important!)

Question 4

Defect Assc with:

1. Turners
2. Digeorge
3. Downs
4. Heretotaxy

Answer 4

1. Turners: aortic coarctation
2. Digeorge: truncus arteriosus
3. Downs: AVSD
4. Heretotaxy: TAPVR

Question 5

Most common cyanotic lesion?

Answer 5

Tetralogy of Fallot

Question 6

How to treat cyanotic spell/ tet spell?

How does it work?

Answer 6

Knee chest position
Decreases systemic venous return; Increases LV resistance
Decreases R –> L shunt

*^ SVR–> ^ resistance in Lt ventricle–> Decreases R–>L shunting (I had to write this to visualize)

Question 7

Classic murmur Assc with PDA?

Answer 7

Constant machine like murmur

Question 8

Classic VSD murmur

Answer 8

Holosystolic murmur between T/M regions

Question 9

Murmurs Assc with ASD?

Answer 9

Basically right sided stenotic murmurs.

• Systolic murmur in pulmonic region
• Diastolic murmur in tricuspid region
• widely fixed and split s2
• this is because you see right sided hypertrophy/ L –> R shunting.

Question 10

Condition Assc with decreased femoral pulse?

Answer 10

Aortic coarctation.

Blood doesn’t flow through the aorta distal to the subclavian.

Question 11

Classic murmur Assc with tetralogy

Answer 11

HARSH pulm stenosis murmur radiating into the BACK!

Question 12

One murmur you can expect to hear in truncus arteriosus? Why?

Answer 12

Condition causes VSD.

Will cause the same murmur + cyanosis.

Question 13

What murmur does TAPVR mimic?

Answer 13

ASD; causes right sided hypertrophy

* but will also have cyanosis.

Question 14

Symptoms Assc with transposition of the great arteries?

Answer 14

CHF-like + cyanosis.

Question 15

Tricuspid atresia Presentation: (3)

Answer 15

Single S2, left PMI, cyanosis.

Question 16

Five diseases that present with CYANOSIS.

Answer 16

The five T’s.

1. Truncus Arteriosus. (1 great artery)
2. Transposition of the (2) great arteries
3. TRIcuspid atresia
4. TETralogy of fallot
5. TAPVR (5 letters)

Question 17

1. Most common cause of ASD?
2. Physical changes that occur as a result of ASD
3. Symptoms
4. Heart exam findings (3)

Answer 17

1. Usually ostium secundum defect.
2. Causes L –> R shunting; R sided hypertrophy; increased pulmonary flow.
3. Usually asymptomatic, don’t present at birth/ during PDA closure/ with cyanosis.
4. Get RV impulse, split S2, and two right sided murmurs (sounds like pulmonary + tricuspid stenosis)

Question 18

VSD

1. Most common cause
2. Physical effects
3. Symptoms
4. Heart exam findings (1)

Answer 18

1. Usually membranous defect
2. L–> R shunting; LEFT hypertrophy; increased pulmonary flow.
3. Typically asymptomatic; falure to thrive a 1 mos if severe
4. Hear holosystolic murmur between T + M

Question 19

PDA:

1. Common cause/ Assc
2. Physical effects
3. Symptoms
4. Classic murmur
5. Treatment for preemies

Answer 19

1. Assc with maternal rubella infection (first aid)
2. Causes aortic –> pulm shunting after birth, leads to left hypertrophy
3. Tachypnia/hepatomegly
4. Constant machine like murmur
5. NO indomethacin for term babies!!!!

Question 20

Describe the COMPLETE AVSD defect (4).
What is the heart exam like? (2)
How do these patients present?

Answer 20

1. -ostium Primum defect
   - one AV valve annulus
   -no mitral valve
   -inlet ventricular septal defect
   (Heart = Giant, hollow hole! good)
2. Hear VSD + ASD like murmurs, very loud S2
3. Kiddos have symptoms of CHF but not cyanosis.

Question 21

Describe the PARTIAL AVSD defect (4).
What is the heart exam like (3)?
How do these patients present?

Answer 21

1. -ostium primum defect
   - TWO AV valve annuli
   - mitral valve present but cleft
   - no ventricular septal defect
2. Presents like an ASD; largely asymptomatic
3. pulmonary stenosis like murmur; RV impulse; split S2

• sounds so much like an ASD; I think the only way to differentiate in a test question is if the stem involves a kid with Downs.*

Question 22

Two conditions Assc with aortic coarctation:

Answer 22

1. Turners syndrome

2. 50% have bicuspid aortic valves = aortic stenosis later in life.

Question 23

Where is the coarctation in aortic coarctation?

Answer 23

Just distal to subclavian

Question 24

Aortic coarctation classic presentation (3)

Answer 24

• CHF symptoms 3-4 days after birth
• absent femoral pulse (upper extremity hypertension, lower extremity)
• Gallop rhythm.

rib notches on CXR late in life

Question 25

Hypoplastic left heart syndrome 1. Physical cause 2. Classic presentation (2)

Answer 25

- Aortic + mitral stenosis ---> atresia (severity varies) - Right heart is dominant; complete mixing of venous/ arterial blood in the right atrium - Shock at 3-4 days after birth

Question 26

Tetralogy of Fallot- name the four defects

Answer 26

``` IHOP Interventricular septal defect Hypertrophy of the right heart Overriding aorta (over-rides the ventricular defect) Pulmonary stenosis ```

Question 27

What kind of shunt is seen in tetralogy of Fallot?

Answer 27

Initially it is L -->R, but it progresses from R--> L as pulmonary stenosis increases.

Question 28

Classic murmur and CXR Assc with Tetralogy?

Answer 28

1. Boot shaped heart on CXR | 2. Harsh pulmonary stenosis murmur radiating into the back

Question 29

Truncus Arteriosus 1. Physical defect 2. Assc findings on heart exam (3) 3. Symptoms

Answer 29

1. Single great vessel overlying ventricular septal defect (all blood- venous and arterial- mixes in the single vessel) 2. Hear VSD murmur + systolic click and diastolic murmur at the truncal valve 3. Kiddo will be cyanotic **Note: This disorder is caused by improper neural crest cell migration, as is transposition of the great vessels.**

Question 30

Transposition of the great vessels 1. most common form 2. Symptoms (2) 3. Classic CXR findings 4. What do these babies need? What do you give them!?

Answer 30

1. D-TGA (aorta is anterior and right; receives deoxygenated blood). 2. Babies will present with CHF + cyanosis 3. "Egg on string" appearance on CXR 4. These babies need a PDA to send any oxygenated blood to the body. Must administer PGE

Question 31

Tricuspid atresia is Assc with what other physical effects? (3)

Answer 31

Most often kids also have VSD, ASD, and RV hypoplasia | *findings are kind of non-specific aside from cyanosis*

Question 32

Describe the TAPVR defect. What is necessary to maintain blood flow?

Answer 32

Pulmonary veins empty into the right atrium. In order for kids to have any oxygenated blood flow, they need a patient foramen ovale/ ductus arteriosus. Have to give PGE1 if they have PDA + TAPVR

Question 33

Symptomatic. Presentation of TAPVR (2 scenarios) | Heart exam findings?

Answer 33

Venous return OBSTRUCTED: - Severe respiratory distress at the time of birth - Heart exam may not be specific. Venous return NOT OBSTRUCTED: - Baby is asymptomatic at birth but fails to thrive - Presents with cyanosis - Heart exam findings similar to ASD.

Question 34

Defect Assc. With maternal diabetes

Answer 34

Transposition of GA